The SAPHO
syndrome./ El sindrome de SAPHO. Data-Medicos
Dermagic/Express No. (2)-79
20 October 1.999 20 October 1.999.
~ El Sindrome de SAPHO ~
~ The SAPHO Syndrome ~
EDITORIAL ESPANOL
=================
Hola amigos DERMAGICOS, la edicion de hoy se fue ayer "pegada" en el correo del cumpleaņos Ese tema del SINDROME DE SAPHO es bastante educativo, pues DETRAS DE UN ACNE vulgar, pueden estar otros problemas acompaņandolo tales como sinovitis, pustulosis, hiperostosis, osteitis y otras, de modo que debemos estar pendiente en todo caso de ACNE otras manifestaciones clinicas que puedan presentarse pues podriamos estar en presencia de este sindrome sin darnos cuenta. En estas 49 referencias los hechos.
COMENTARIOS:
El correo de hoy lleva el numero (2) 79, significa segundo aņo de DERMAGIC entrega 79.
Saludos a todos !!!
Dr. Jose Lapenta R.,,,
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EDITORIAL ENGLISH
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Hello DERMAGICS friends , today's edition left yesterday "scape" in the mail of the birthday That topic of the SAPHO SYNDROME is quite educational, because BEHIND OF A vulgar ACNE, they can be other problems accompanying him such as synovitis, pustulosis, hyperostosis,osteitis and others, so that we should be pending in any case of ACNE other clinical manifestations that can be presented because we could be in presence of this syndrome without know it. In these 49 refrerences the facts.
COMMENTS:
Today's mail takes the I number (2) 79, it means second year of DERMAGIC
edition 79.
Greetings to ALL, !!
Dr. Jose Lapenta R.,,,
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REFERENCIAS BIBLIOGRAFICAS / BIBLIOGRAPHICAL REFERENCES
===================================================================
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1.) Arthropathy associated with cystic acne, hidradenitis suppurativa, and perifolliculitis capitis abscedens et suffodiens: treatment with isotretinoin.
2.) Psoriatic arthritis and the spectrum of syndromes related to the SAPHO(synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome.
3.) SAPHO syndrome or psoriatic arthritis? A familial case study.
4.) SAPHO syndrome of the temporomandibular joint associated with sudden deafness.
5.) The SAPHO syndrome in children: a rare cause of hyperostosis and osteitis.
6.) Cervical spinal cord injury in sapho syndrome.
7.) Thrombosis of the subclavian vein in SAPHO syndrome. A case-report.
8.)Vertebral involvement in SAPHO syndrome: MRI findings.
9.) SAPHO syndrome: 20-year follow-up.
10.) Stress-induced SAPHO syndrome.
11.) Recurrent subcutaneous abscess of the sternal region in ulcerative colitis.
12.) [Vertebral destruction with sever pain in the SAPHO syndrome].
13.) [The SAPHO syndrome: a study of 16 cases].
14.) [Spondarthritis hyperostotica pustulo-psoriatica: nosologic study with clinical and radiologic presentation in relation to the SAPHO syndrome].
15.) [The synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome, a rare extra-digestive manifestation of Crohn's disease. Presentation of 1 case and review of the literature].
16.) [Inflammatory spondylodiscitis as a unique radiological manifestation of the SAPHO syndrome].
17.) The SAPHO syndrome: defining the radiologic spectrum ofdiseases comprising the syndrome.
18.) Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome: a brief review of a rare condition.
19.) Two cases of discitis due to Propionibacterium acnes.
20.) Is SAPHO syndrome a spondylarthropathy? A vasculopathy? Report of a case.
21.) Chronic recurrent multifocal osteomyelitis associated with Crohn's disease.
22.) Osteitis--an under-recognised association with seronegative spondyloarthropathy?
23.) [Garre sclerosing osteomyelitis].
24.) Anterior chest wall arthritis and osteitis associated with Sneddon- Wilkinson disease.
25.) Chronic diffuse sclerosing osteomyelitis of the mandible or mandibular
location of S.A.P.H.O. syndrome.
26.)[SAPHO syndrome: common denominator for various bone and skin diseases]
27.)[SAPHO syndrome. Case description of 3 patients with acne conglobata and
osteoarticular symptoms]
28.)Case report. synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome.
29.) [Chronic recurrent multifocal osteomyelitis in children]
30.) Acquired hyperostosis syndrome--AHYS--(sternocostoclavicular hyperostosis, pustulotic arthro-osteitis, SAPHO-syndrome): bone scintigraphy of the anterior chest wall.
31.) The course of the SAPHO syndrome during pregnancy.
32.)[Lyme disease and SAPHO syndrome]
33.) Spondylodiscitis in SAPHO syndrome. A series of eight cases.
34.) [Lumbo-sacroiliac hyperostosis with retroperitoneal fibrosis in
spondarthritis hyperostotical pustulo-psoriatica--with sternocostoclavicular hyperostosisand superior and inferior venous obstruction syndrome: pathogenetic hypothesisof fibro-osteopathia psoriatica]
35.) Sapho syndrome associated with acne fulminans and prominent acromioclavicular joint involvement.
36.) An aseptic inflammation of the clavicle in a patient with Crohn's disease. A potential manifestation of the SAPHO syndrome.
37.) SAPHO syndrome: a followup study of 19 cases with special emphasis on enthesis involvement.
38.) SAPHO syndrome.
39.) Psoriatic arthritis and synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome.
40.) The SAPHO syndrome.
41.) SAPHO syndrome [see comments]
42.) SAPHO syndrome.
43.) Is diffuse sclerosing osteomyelitis of the mandible part of the synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome? Analysis of seven cases
44.) Sternoclavicular pustulotic osteitis (SAPHO). 20-year intervalbetween skin and bone lesions.
45.) Recent developments in psoriatic arthritis.
46.) Miscellaneous neurologic, cardiac, pulmonary, and metabolic disorders
with rheumatic manifestations.
47.) [Lymphoplasmacellular osteomyelitis]
48.) [Primary chronic osteomyelitis]
49.) Aseptic spondylitis as the initial manifestation of the SAPHO syndrome.
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1.) Arthropathy associated with cystic acne, hidradenitis suppurativa, and perifolliculitis capitis abscedens et suffodiens: treatment with isotretinoin.
============================================================
Cutis 1999 Aug;64(2):87-90
Libow LF, Friar DA
Armed Forces Institute of Pathology, Dermatopathology Division, Washington,
DC 20306-6000, USA.
A patient with arthropathy associated with cystic acne, hidradenitis
suppurativa, and perifolliculitis capitis abscedens et suffodiens who
showed a dramatic response to isotretinoin is described. This, to our
knowledge, is the first report documenting effective treatment of this
condition, whose nosologic position with respect to other
spondyloarthropathies associated with cutaneous disease is considered.
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2.) Psoriatic arthritis and the spectrum of syndromes related to the SAPHO(synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome.
============================================================
Curr Opin Rheumatol 1999 Jul;11(4):251-6
Winchester R
Division of Autoimmune and Molecular Diseases, Columbia University College
of Physicians and Surgeons, New York, New York 10032, USA.
During the past year, the increasing use of nuclear magnetic resonance
imaging techniques, with their ability to delineate cartilage and
ligamentous structures and to identify edema, are providing a radical
improvement in ascertainment of musculoskeletal abnormalities, although
their significance remains incompletely delineated. A second theme has come
from the study of spondyloarthropathies in different ethnic groups and
societal environments, revealing that the Northern European and North
American form of the disease, with its powerful association with HLA-B27,
is little evident in the rest of the world's population and that different
susceptibility genes and environmental factors operate in other regions and
peoples. Related to this theme is the compelling evidence of the marked
influence of HIV infection on the development of spondyloarthropathies in
Africa. Two areas of immune recognition are discussed as examples of
emerging fields that may provide useful paradigms for the experimental
approach to mechanisms in psoriatic arthritis. One of these is the
three-cell model of CD8 T-cell interaction, in which a dendritic cell
presents a peptide from an immunogenic protein to both a CD4 and CD8 T-cell
clone, providing a cognitive interaction that disrupts tolerance and
results in the expansion of the cytotoxic T-cell clone. In this respect,
the combination of an activated dendritic cell, together with enhanced
availability of arthritogenic microbial antigens caused by microbial
persistence, are interesting candidates to explore as the basis of the
HIV-associated rheumatic diseases. The second area of immune recognition is
the growing understanding of the outline of the solution to the problem of
the association of a spondyloarthropathy with several
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3.) SAPHO syndrome or psoriatic arthritis? A familial case study.
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Rheumatology (Oxford) 1999 May;38(5):463-7
Dumolard A, Gaudin P, Juvin R, Bost M, Peoc'h M, Phelip X
Department of Rheumatology, CHU, Grenoble, France.
OBJECTIVE: To discuss the relationships between SAPHO (synovitis, acne,
pustulosis, hyperostosis and osteitis) syndrome and the group of
spondylarthropathies. METHODS: Few reports of familial SAPHO have been
published. We describe three children, two sisters and one brother, whose
clinical and radiological presentation was in accordance with SAPHO
syndrome. RESULTS: Two children developed psoriasis, and one child
palmoplantar pustulosis. Both sacroiliac and sternoclavicular joints were
involved in these three cases. Some features in our observations are also
common to psoriatic arthritis. No association was found with HLA antigens,
but a history of trauma preceding the onset of symptoms was present in all
three children. CONCLUSIONS: We can consider that SAPHO is nosologically
related to spondylarthropathies. Psoriatic arthritis could be the missing
link between SAPHO and spondylarthropathies. It is likely that both genetic
and environmental factors are involved.
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4.) SAPHO syndrome of the temporomandibular joint associated with sudden deafness.
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AJNR Am J Neuroradiol 1999 May;20(5):902-5
Marsot-Dupuch K, Doyen JE, Grauer WO, de Givry SC
Department of Radiology, University Hospital St Antoine, Paris, France.
We report a case of arthritis of the temporomandibular joint (TMJ)
associated with sclerosing osteomyelitis of the mandible and temporal bone,
causing deafness. The presence of a palmoplantar pustulosis established the
diagnosis of SAPHO syndrome. SAPHO (an acronym referring to synovitis,
acne, palmoplantar pustulosis, hyperostosis, and osteitis) syndrome is
defined by the association of characteristic osteoarticular and
dermatologic manifestations, with diffuse sclerosing osteomyelitis of the
mandible being a part of this entity. We review the literature of SAPHO
syndrome with mandibular manifestations and discuss the mechanisms of
inflammatory spread from the TMJ to the cochlea. To our knowledge, this is
the first description of skull base involvement in a patient with SAPHO
syndrome leading to sudden deafness.
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5.) The SAPHO syndrome in children: a rare cause of hyperostosis and osteitis.
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J Pediatr Orthop 1999 May-Jun;19(3):297-300
Letts M, Davidson D, Birdi N, Joseph M
Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa,
Canada.
The SAPHO syndrome is a rare constellation of signs and symptoms
characterized by synovitis, acne, pustulosis, hyperostosis, and osteitis.
The most common musculoskeletal complaints are hyperostosis, causing pain,
tenderness, and swelling of the anterior chest wall, although any part of
the axial and appendicular skeleton may be affected. There is a great
degree of variability in the dermatologic involvement of this syndrome. A
combination of clinical, radiographic, and pathological investigation is
required to establish the correct diagnosis. No single treatment has been
found to be effective, although nonsteroidal antiinflammatory drugs have
been the most frequently used. Because there is no mention of SAPHO
syndrome in the English orthopaedic literature, and pediatric orthopaedic
surgeons may be the first caregivers to treat these children, we thought it
appropriate to share our experience with a 5-year-old boy with SAPHO
syndrome recently under our care.
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6.) Cervical spinal cord injury in sapho syndrome.
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Spinal Cord 1999 Apr;37(4):301-4
Deltombe T, Nisolle JF, Boutsen Y, Gustin T, Gilliard C, Hanson P
Department of Physical Medicine and Rehabilitation, Mont-Godinne University
Hospital UCL, Yvoir, Belgium.
Cervical spinal fracture and pseudarthrosis are previously described causes
of spinal cord injury (SCI) in patients with spondylarthropathy. SAPHO
(Synovitis Acne Pustulosis Hyperostosis Osteitis) syndrome is a recently
recognized rheumatic condition characterized by hyperostosis and
arthro-osteitis of the upper anterior chest wall, spinal involvement
similar to spondylarthropathies and skin manifestations including
palmoplantar pustulosis and pustular psoriasis. We report the first case of
SAPHO syndrome disclosed by SCI related to cervical spine ankylosis.
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7.) Thrombosis of the subclavian vein in SAPHO syndrome. A case-report.
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Rev Rhum Engl Ed 1999 Mar;66(3):173-6
Lazzarin P, Punzi L, Cesaro G, Sfriso P, De Sandre P, Padovani G, Todesco S
Division of Rheumatology, University of Padova, Italy.
The hallmark of SAPHO Syndrome (Synovitis, Acne, Pustulosis, Hyperostosis,
Osteitis) is osteitis of the anterior chest wall, which can be so
pronounced as to cause thoracic outlet syndrome, thrombosis of the
subclavian vein, and compression of the superior vena cava. Suggestive skin
manifestations, namely palmoplantar pustulosis and severe acne, generally
antedate the bone and joint lesions. We report a case of SAPHO syndrome
that went unrecognized for many years and eventually caused compression and
thrombosis of the right subclavian vein requiring cleidectomy. SAPHO
syndrome should be considered in every patients with anterior chest wall
inflammation, particularly when skin lesions are also present.
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8.)Vertebral involvement in SAPHO syndrome: MRI findings.
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Skeletal Radiol 1999 Mar;28(3):163-8
Nachtigal A, Cardinal E, Bureau NJ, Sainte-Marie LG, Milette F
Department of Radiology, Hopital Saint-Luc, Centre Hospitalier de
l'Universite de Montreal, QC, Canada.
We report on the MRI findings in the vertebrae and surrounding soft tissues
in two patients with the SAPHO syndrome (Synovitis, Acne, Pustulosis,
Hyperostosis, Osteitis). The MRI findings include abnormal bone marrow
signal, either focal or diffuse, of the vertebral bodies and posterior
elements; hyperintense paravertebral soft tissue swelling and abnormal
signal of the intervertebral discs. These changes are consistent with
discitis and osteitis.
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9.) SAPHO syndrome: 20-year follow-up.
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Skeletal Radiol 1999 Mar;28(3):159-62
Davies AM, Marino AJ, Evans N, Grimer RJ, Deshmukh N, Mangham DC
Department of Radiology, Royal Orthopaedic Hospital, Birmingham, UK.
Considerable attention has been paid in the past 10 years to the
radiological spectrum of disease entities belonging to the SAPHO syndrome.
We report an unusual case presenting with an extra-axial (femoral) lesion,
prior to description of this syndrome, which was radiologically and
histologically mistaken for a parosteal osteosarcoma. Nineteen years later,
a further lesion developed in the scapula together with the typical
sternoclavicular manifestations, at which stage the correct diagnosis of
SAPHO syndrome was established.
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10.) Stress-induced SAPHO syndrome.
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J Eur Acad Dermatol Venereol 1999 Jan;12(1):43-6
Orion E, Brenner S
Department of Dermatology, Tel Aviv Sourasky Medical Center, Sackler
Faculty of Medicine, Tel Aviv University, Israel.
We describe the case of a woman with the classic combination of features of
synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome, including
pustulosis palmo-plantaris and anterior chest wall involvement. The varying
symptomology, etiology and pathogenesis of this syndrome and the
contribution of stress are discussed. The authors ascribe the dearth of
reported cases to lack of awareness and recognition of SAPHO, and not to
the real incidence of the syndrome.
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11.) Recurrent subcutaneous abscess of the sternal region in ulcerative colitis.
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Am J Gastroenterol 1999 Mar;94(3):844-5
Murata I, Satoh K, Yoshikawa I, Masumoto A, Sasaki E, Otsuki M
Third Department of Internal Medicine, University of Occupational and
Environmental Health, and The Kyoritsu Hospital, Kitakyushu, Japan.
An 18-yr-old female patient with extensive ulcerative colitis suffered from
several episodes of recurrent aseptic subcutaneous abscesses of the sternal
region with a course paralleling that of her colitis. The abscess seemed to
occur secondarily to osteomyelitis of the sternum, which is a manifestation
of the synovitis, acne, pustulosis, hyperostosis, and osteomyelitis (SAPHO)
syndrome.
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12.) [Vertebral destruction with sever pain in the SAPHO syndrome].
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Dtsch Med Wochenschr 1999 Feb 5;124(5):114-8
Hagemann D, Pfaffenbach B, Schmid G, Adamek RJ
Medizinische Klinik I, Ruhr-Universitat, St. Josef-Hospital, Bochum.
[email protected]
HISTORY AND ADMISSION FINDINGS: A 57-year-old man had for the past 18
months complained of recurrent, recently worsening, belt-like backache
radiating ventrally. On admission a skin rash consisting of blister and
pustules was noted on the palms of both hands. He had pain on pressure over
the right upper abdomen, an enlarged prostate and definite pain on
percussing the vertebral column with restricted movement of the thoracic
vertebral column, but no other physical signs. INVESTIGATIONS: Radiology
revealed clearly increased sclerosis of several thoracic vertebrae with
osteolytic destruction and a paravertebral soft tissue tumor. Search for a
primary tumor was unsuccessful. Bone scintigraphy demonstrated nuclide
enrichment of the thoracic vertebrae and of the sternoclavicular joints
without increase in the LeukoScan. These findings indicated the diagnosis
of SAPHO syndrome (synovitis-acne-pustulosis-hyperostosis-osteomyelitis).
TREATMENT AND COURSE: Rapid subjective and objective improvement followed
the administration of clindamycin and ibuprofen. CONCLUSION: In case of
bone pain of uncertain aetiology, especially when associated with skin
rash, the rare SAPHO syndrome should be considered in the differential
diagnosis, avoiding lengthy diagnostic steps and allowing early treatment.
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13.) [The SAPHO syndrome: a study of 16 cases].
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Med Clin (Barc) 1999 Jan 23;112(2):61-3
Olive A, Perez-Andres R, Rivas A, Holgado S, Casado E, Guma M, Tena X
Seccion de Reumatologia, Hospital Universitari Germans Trias i Pujol,
Badalona, Barcelona.
The SAPHO syndrome is characterized by synovitis, acne, palmo-plantar
pustulosis, hyperostosis and osteitis. SAPHO syndrome has been occasionally
described in Spain. We present our experience of 16 cases with the SAPHO
syndrome diagnosed between 1984-1995. The predominant clinical symptoms
were: anterior thoracic pain (14 cases), sacroiliac pain (7 cases) and
peripheral arthritis (2 cases). Cutaneous involvement was characterized by
palmo-plantar pustulosis (8 cases) acne (3 cases) and psoriasis (2 cases).
The histocompatibility antigen B27 was negative in all cases. A
computerized tomographic study revealed involvement of
sterno-costo-clavicular and manubriosternal joints (14 cases) and
sacroiliitis (8 cases).
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14.) [Spondarthritis hyperostotica pustulo-psoriatica: nosologic study with
clinical and radiologic presentation in relation to the SAPHO syndrome].
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Rofo Fortschr Geb Rontgenstr Neuen Bildgeb Verfahr 1998 Dec;169(6):576-84
Schilling F, Kessler S
Radiologische Universitatsklinik Mainz.
Spondarthritis hyperostotica pustulo-psoriatica (Spond.hyp.pp-Schilling),
corresponding nosologically to pustulotic arthroosteitis, is a
dermato-skeletal "double system" disease of adults. It consists of the
triad (a) palmo-plantar pustulosis (Ppp) or, alternatively,
Konigsbeck-Barber-type psoriasis, (b) sternocostoclavicular hyperostosis
(SCCH), and (c) truncal-skeletal changes with syndesmophite-like,
hyperostotic and/or parasyndesmophite-like ossifications of layers of the
anterior vertebral ligament taken together in the sense of a desmophytal
hyperostosis. There is also a potential for sclerosing inflammatory
arthritis of the sacro-iliac joints and "dry" inflammatory arthritis of
peripheral joints. Thus, the pustulo-psoriatic terrain seems to have a
decisive influence on osseous pathology. A total of 38 cases from a study
during the years 1982 to 1992 is analysed with regard to morphological
characteristics. Rare cases with diaphyseal and pelvic hyperostotic lesions
subsequent to bland sclerosing osteomyelitis constitute an overlapping
region to chronic recurrent multifocal osteomyelitis (CRMO) and illustrate
the relationship between hyperostotic spondarthritis and CRMO. The
syndromes of "acquired hyperostosis" and "SAPHO", the former more
radiologically oriented and the latter more clinically oriented, together
with mainly CRMO and hyperostotic spondarthritis and its forms, constitute
the "Spond.hyp.pp.". Although hyperostosis is a guidepost for the
radiologist and SAPHO symptoms are one for the clinician, the syndrome does
not represent a diagnosis by itself and requires further differentiation.
In this report the entity "Spond.hyp. pp." is considered and required
contributions from rheumatologically and osteologically oriented radiologist.
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15.) [The synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome, a rare extra-digestive manifestation of Crohn's disease. Presentation of 1
case and review of the literature].
============================================================
Gastroenterol Clin Biol 1998 Feb;22(2):240-3
Dharancy S, Talbodec N, Asselah T, Hachulla E, Delaporte E, Cortot A,
Colombel JF
Service des Maladies d l'Appareil Digestif et de la Nutrition, Hopital
Claude-Huriez, CHRU, Lille.
We report the case of a 33-year-old man with a severe Crohn's disease since
the age of sixteen. He presented with acne and palmoplantar pustulosis
associated with a right knee synovitis. Investigations revealed a major
axial bone condensation. The association
synovitis-acne-pustulosis-hyperostosis-osteitis leaded to the diagnosis of
SAPHO syndrome associated with Crohn's disease.
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16.) [Inflammatory spondylodiscitis as a unique radiological manifestation of the SAPHO syndrome].
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J Radiol 1998 Apr;79(4):337-40
Cabay JE, Marcelis S, Dondelinger RF
Departement d'Imagerie Medicale, Domaine Universitaire du Sart-Tilman B 35,
Liege, Belgique.
We describe two cases of SAPHO with an exclusive spinal involvement.
Diagnosis was established by sterno-clavicular arthralgias, palmar and
plantar pustulosis and by radiological signs of inflammatory
spondylodiscitis and vertebral osteitis. Spondylodiscitis and medullary
edema resolved as shown by MRI after administration of steroids in one case
and methotrexate in the other.
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17.) The SAPHO syndrome: defining the radiologic spectrum of diseases comprising
the syndrome.
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Eur Radiol 1998;8(5):800-6
Sugimoto H, Tamura K, Fujii T
Department of Radiology, Jichi Medical School, 3311 Minamikawachi-machi,
Kawachi-gun, Tochigi-ken, 329-04, Japan.
The objective of our study was to clarify the radiologic spectrum of
disease entities belonging to the SAPHO syndrome (SAPHO being an acronym
for synovitis, acne, pustulosis, hyperostosis, and osteitis). A
retrospective analysis of radiologic data was undertaken to determine the
relationship of the osteoarthritic changes seen in palmoplantar pustulosis
(PPP, n = 179), acne (n = 3), psoriasis vulgaris (PsV, n = 355),
generalized pustular psoriasis (GPP, n = 25), and chronic recurrent
multifocal osteomyelitis (CRMO, n = 4). Osseous changes of PPP, acne, and
CRMO overlap each other; 7 PPP, 2 acne, and 3 CRMO patients manifested
stenocostoclavicular hyperostosis as well as hyperostosis of the spine,
pelvis, and/or femur. These findings were not seen in either PsV or GPP
patients. Thirteen PsV and 4 GPP patients had peripheral arthritis and/or
symmetrical sacroiliitis, which were not observed in the PPP, acne, and
CRMO patients. The PPP, acne, and CRMO patients may be grouped as belonging
to the single disease entity, namely SAPHO syndrome. Our findings do not
support the inclusion of PsV and GPP in the spectrum of this syndrome.
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18.) Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome: a
brief review of a rare condition.
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Ann Acad Med Singapore 1998 Jan;27(1):122-4
Koh ET
Department of Rheumatology and Immunology, Tan Tock Seng Hospital, Singapore.
Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis (SAPHO) syndrome is
an osteoarticular-skin syndrome characterised by sterile inflammatory
arthro-osteitis of the anterior chest wall. It is associated with various
skin conditions including palmoplantar pustulosis, severe acnes and
pustular psoriasis. A link between SAPHO syndrome and seronegative
spondyloarthropathies has been suggested over the last 10 years. SAPHO
syndrome mainly affects young and middle-aged adults. Treatment of SAPHO
syndrome remains empirical as the underlying aetiopathogenesis is unclear.
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19.) Two cases of discitis due to Propionibacterium acnes.
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Rev Rhum Engl Ed 1998 Jan;65(1):68-71
Crouzet J, Claudepierre P, Aribi EH, Malbrunot C, Beraneck L, Juan LH,
Larget-Piet B
Rheumatology Department, Corbeil Hospital, Corbeil, France.
We report two cases of discitis due to Propionibacterium acnes and review
previously published cases of bone and joint infections in which this
organism was recovered as a pure culture. P. acnes is an anaerobic organism
usually considered a normal inhabitant of the skin but capable of producing
a variety of infections including discitis, osteitis, arthritis, and chest
wall osteitis. Most patients were immunocompetent. A few infections
occurred spontaneously, whereas others were secondary to a break in the
skin or to implantation of foreign material into the body for instance
during internal fixation of a fracture or arthroplasty. Cases of P. acnes
chest wall infection have been reported in patients with palmoplantar
pustulosis or chronic or multifocal osteitis, supporting a role for P.
acnes in SAPHO syndrome.
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20.) Is SAPHO syndrome a spondylarthropathy? A vasculopathy? Report of a case.
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Rev Rhum Engl Ed 1997 Jun;64(6):424-7
Boonen A, Verwilghen J, Dequeker J, Van der Linden S, Westhovens R
Department of Rheumatology, Maastricht University Hospital, The Netherlands.
We describe the case of a patient who had SAPHO syndrome with mediastinal
and retroperitoneal fibrosis. After several years, he developed erosive
polyarthritis and systemic vasculopathy. The possible implications of these
associations are discussed.
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21.) Chronic recurrent multifocal osteomyelitis associated with Crohn's disease.
============================================================
Am J Med Sci 1998 Feb;315(2):133-5
Bognar M, Blake W, Agudelo C
Internal Medicine Department, VA Medical Center, Emory University Hospital,
Atlanta, Georgia, USA.
Chronic recurrent multifocal osteomyelitis (CRMO) was first described by
Giedion in 1972 as a self-limited relapsing noninfectious inflammatory
condition usually affecting children and adolescents. The association of
CRMO with psoriasis, palmoplantar pustulosis, and SAPHO syndrome
(synovitis, acne, pustulosis, hyperostosis, and osteitis) has been
described. In this case report, we present a 49-year-old man with CRMO
coexisting with Crohn's disease. To our knowledge, this is the first report
of CRMO with Crohn's disease without the presence of pustular skin lesions
or other features of SAPHO syndrome.
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22.) Osteitis--an under-recognised association with seronegative
spondyloarthropathy?
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N Z Med J 1997 Dec 12;110(1057):455-9
Stebbings S, Highton J, Doyle TC, Jeffery AK
Department of Medicine, Dunedin School of Medicine.
AIM: To emphasise osteitis as a feature of the spondyloarthritides. METHOD:
We describe four cases spanning a spectrum of the spondyloarthritides in
which osteitis was a feature. RESULTS: One patient had psoriatic arthritis
with palmar-plantar pustular psoriasis and extensive osteitis involving the
tibia and fibula. This case provides a link with two cases with SAPHO
syndrome (synovitis, acne, pustulosis hyperostosis, osteitis) who had
palmar-plantar pustulosis and osteitis. Many now argue that this syndrome
is a form of spondyloarthritis. The fourth case, which was of particular
interest to us, had enteric reactive arthritis and scintigraphic changes
strongly suggesting the presence of osteitis of individual bones in the
wrist. CONCLUSION: We propose that these four cases demonstrate that
osteitis may be another feature common to the spondyloarthritides and
SAPHO. Awareness of this may facilitate better documentation of this
feature of the disease.
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23.) [Garre sclerosing osteomyelitis].
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Orthopade 1997 Oct;26(10):902-7
Vienne P, Exner GU
Orthopadische Universitatsklinik Zurich.
Osteomyelitis sclerosans Garre is still not completely understood. In spite
of the fact that it is caused by bacterial infection, in most cases no
bacterial growth can be discovered in culture. Furthermore, it remains open
whether the chronic process is maintained by low-grade persistent infection
or is maintained by itself after sterilization of the infection. Newer
studies that may prove the presence of bacteria, e.g., by PCR, may
elucidate the causes in the future. Sclerosing bone disease, sometimes
multifocal, which present the same clinical, radiographic and histological
features as chronic sclerosing osteomyelitis may be associated with
diseases such as palmoplantar pustulosis, colitis ulcerosa, Crohn's
disease, etc. and are reported as SAPHO (synovitis acne pustulosis
hyperostosis osteitis) syndrome. In these diseases bacterial growth is
almost always negative. However, temporary improvement under antibiotic
therapy may be observed. A relationship between typical isolated chronic
sclerosing osteomyelitis and SAPHO syndrome may exist, but the diseases
should at present be differentiated. Neoplasms such as osteoid osteoma,
Ewing sarcoma, or eosinophilic granuloma may simulate primary sclerosing
osteomyelitis, and pathological examination of peripherally taken specimens
may appear to be compatible with the diagnosis of sclerosing osteomyelitis.
It should be appreciated that malignant tumors--even though this is the
exception--can present a chronic, mild picture. Treatment of osteomyelitis
sclerosans Garre needs be determined on an individual basis. A conservative
approach with antibiotics usually leads only to temporary pain relief. The
biopsy with opening of the medullary canal is sometimes sufficient for
healing, while in extreme situations only segmental or even complete
resection of the diseased bone case result in a permanent cure.
============================================================
24.) Anterior chest wall arthritis and osteitis associated with
Sneddon-Wilkinson disease.
============================================================
Clin Exp Rheumatol 1997 Nov-Dec;15(6):667-9
Belfiore N, Caporali R, Borroni G, Montecucco C
Dipartimento di Medicina Interna e Nefrologia, IRCCS Policlinico S. Matteo,
University of Pavia, Italy.
We describe a 46-year-old man in whom anterior chest wall arthritis and
clavicular osteomyelitis occurred together with sub-corneal pustular
dermatosis (Sneddon-Wilkinson disease). This observation extends the list
of neutrophilic skin lesions that may be involved in the so-called SAPHO
syndrome.
============================================================
25.) Chronic diffuse sclerosing osteomyelitis of the mandible or mandibular
location of S.A.P.H.O. syndrome.
============================================================
Garcia-Marin F; Iriarte-Ortabe JI; Reychler H
Section of Oral and Maxillofacial Surgery, Hospital Son Dureta, Palma de
Mallorca,
Spain.
Acta Stomatol Belg (BELGIUM) Jun 1996 93 (2) p65-71 ISSN: 0001-7000
Language: ENGLISH
Document Type: JOURNAL ARTICLE; REVIEW; REVIEW, TUTORIAL
Journal Announcement: 9711
Subfile: DENTAL
The chronic diffuse sclerosing osteomyelitis (CDSO) of the mandible has
been
described as an inflammatory disease characterized by recurrent episodes of
intense
pain in the mandible, often accompanied by trismus, paresthesia and
progressive
mandibular deformity. The etiopathogeny of this entity is not fully known.
The
differential diagnosis must be carried out very carefully, and the
treatment results
are very disappointing. Recently, evidence that suggests that CDSO may be
the
mandibular location of a more diffuse condition, the synovitis, acne,
pustulosis,
hyperostosis, osteitis (SAPHO) syndrome, has been offered. We describe two
clinical
cases of CDSO of typical evolution which fulfill the criteria for SAPHO
syndrome,
offering us an occasion for a review of the current literature. (31
References)
============================================================
26.)[SAPHO syndrome: common denominator for various bone and skin diseases]
SAPHO-syndroom: gemeenschappelijke noemer voor enkele bot- en huidziekten.
============================================================
Jahangier ZN; Jacobs JW; Bijlsma JW
Academisch Ziekenhuis, afd. Reumatologie en Klinische Immunologie, Utrecht.
Ned Tijdschr Geneeskd (NETHERLANDS) Apr 19 1997 141 (16) p764-9 ISSN:
0028-2162
Language: DUTCH Summary Language: ENGLISH
Document Type:
JOURNAL ARTICLE; REVIEW; REVIEW, TUTORIAL English Abstract
Journal Announcement: 9709
Subfile: INDEX MEDICUS
The acronym 'SAPHO' stands for synovitis, acne, pustulosis, hyperostosis
and
osteitis. Three subtypes of SAPHO can be distinguished which have the
following
features in common: sternoclavicular hyperostosis and sterile inflammatory
lesions in
both bone and skin. These subtypes are: sternocostoclavicular
hyperostosis, chronic
recurring multifocal osteomyelitis, and pustular arthro-osteitis.
Hyperostosis and
osteitic lesions may be similar to those seen in malignant bone tumours.
Synovitis
generally does not lead to bone erosions and one-third of the patients
develop
sacroiliitis. The SAPHO syndrome and seronegative spondyloarthropathy
share some
common features (a higher prevalence of the HLA-B27 antigen, occurrence of
sacroiliitis and a higher incidence of chronic inflammatory bowel disease and
psoriasis). Aetiology and pathogenesis of SAPHO are unknown; prognosis is
good. The
SAPHO syndrome often runs a protracted course, with intermittent relapses and
remissions without resulting serious disability. Treatment is aimed only at
symptomatic relief and mainly consists of analgetics and nonsteroidal
antiinflammatory drugs. (37 References)
============================================================
27.)[SAPHO syndrome. Case description of 3 patients with acne conglobata and
osteoarticular symptoms]
============================================================
Das SAPHO-Syndrom. Fallbeschreibung von drei Patienten mit Akne
conglobata und
osteoartikularen Symptomen.
Gutzmer R; Herbst RA; Kapp A; Weiss J
Dermatologische Klinik und Poliklinik, Medizinische Hochschule Hannover.
Hautarzt (GERMANY) Mar 1997 48 (3) p186-90 ISSN: 0017-8470
Language: GERMAN Summary Language: ENGLISH
Document Type:
JOURNAL ARTICLE English Abstract
Journal Announcement: 9708
Subfile: INDEX MEDICUS
We report three new cases of the SAPHO syndrome. This acronyme consists of
synovitis, acne, pustulosis, hyperostosis and osteitis. Symptoms of this
syndrome,
which may not all be present, are pustulotic skin diseases (pustulosis
palmoplantaris
or severe acne) associated with osteoarticular lesions (mainly
sternoclavicular
hyperostosis, spondylarthropathies or chronic recurrent multifocal
osteomyelitis).
The dermatological aspects of this syndrome are discussed in detail.
============================================================
28.)Case report. synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome.
============================================================
Suei Y; Taguchi A; Tanimoto K; Yamada T; Otani K; Fukuda T
Department of Oral and Maxillofacial Radiology, Hiroshima University,
School of
Dentistry, Japan.
Dentomaxillofac Radiol (ENGLAND) Nov 1996 25 (5) p287-91 ISSN: 0250-832X
Language: ENGLISH
Document Type: JOURNAL ARTICLE
Journal Announcement: 9708
Subfile: ; DENTAL
A case of the SAPHO (synovitis, acne, pustulosis, hyperostosis and
osteitis)
syndrome in a 35-year-old woman is presented. Ignorance of this entity on
the part
of the physicians treating the patient may have contributed to her having
repeated
diagnostic procedures and treatment, some of which may have been
unnecessary.
Dentists are encouraged to suspect the SAPHO syndrome when they encounter a
patient
with mandibular osteomyelitis together with symptoms involving other bones
and skin
lesions such as pustulosis or psoriasis.
============================================================
29.) [Chronic recurrent multifocal osteomyelitis in children]
Osteite chronique multifocale recurrente de l'enfant.
============================================================
Quelquejay C; Job-Deslandre C; Hamidou A; Benosman A; Adamsbaum C
Service de Radiologie, Hopital Saint-Vincent-de-Paul, Paris.
J Radiol (FRANCE) Feb 1997 78 (2) p115-21 ISSN: 0221-0363
Language: FRENCH Summary Language: ENGLISH
Document Type:
JOURNAL ARTICLE; REVIEW; REVIEW, TUTORIAL English Abstract
Journal Announcement: 9707
Subfile: INDEX MEDICUS
We have studied retrospectively a series of 10 children presenting with
chronic
multifocal osteomyelitis (8 girls, 2 boys, 7 to 16 years). All patients
had plain
films, bone scintigraphies and histological studies. Three had CT scan
and/or MRI.
compared with literature data, we observed only one case of palmoplantar
pustulosis
and only 2 cases of lysis of the medial extremity of the clavicle; in
addition, we
report one case of lateral extremity of the clavicle and 2 vertebral
locations. The
radiological pattern was typical: at the beginning of the disease, plain
films showed
lytic areas which became progressively osteosclerotic with enlargement of
the bone.
In all the cases, bone scintigraphy revealed high uptake areas which were
often
infraclinical. The diagnosis was delayed from 3 months to 3 years. This
emphasizes
the difficulty of the diagnosis which relies on the association of clinical,
biological and radiological elements. Biopsies are required to rule out an
infectious bacterial osteomyelitis or a tumoral process. The pathogenesis
of OCMR
remains unknown, but the relation with the SAPHO (synovitis, acne,
pustulosis,
hyperostosis, osteitis) syndrome is general accepted because of the similar
features
of the osteitis. The long term follow up appears to be uncertain 6 of our
patients
are still symptomatic after five years despite anti inflammatory treatment.
(22 References)
============================================================
20.) Acquired hyperostosis syndrome--AHYS--(sternocostoclavicular hyperostosis,
pustulotic arthro-osteitis, SAPHO-syndrome): bone scintigraphy of the
anterior chest wall.
============================================================
Dihlmann W; Dihlmann SW; Hering L
Roentgen Institute, General Hospital Barmbek, Hamburg, Germany.
Clin Rheumatol (BELGIUM) Jan 1997 16 (1) p13-24 ISSN: 0770-3198
Language: ENGLISH
Document Type: JOURNAL ARTICLE
Journal Announcement: 9707
Subfile: INDEX MEDICUS
The objective of this report is to provide a description of diagnostically
significant scintigraphically recognizable sites and patterns of acquired
hyperostosis syndrome (AHYS) on the anterior chest wall (ACW), which is
involved in
82% of AHYS patients. In 49/90 of our own AHYS patients, planar bone scans
of the
ACW were performed with the gamma camera, applying an average of 650 MBq of
99mTc-
phosphate complexes. In addition, 53 atraumatic patients with
extrathoracic cancer
were available for routine whole-body scintigraphy. None of these patients
had
increased uptake identifiable as metastasis clinically or by imaging
modalities in
either the ACW or the rest of the skeleton. The scintigraphic involvement
of the
various morphological ACW structures is described in AHYS. Moreover,
attention is
called to the diagnostic significance of focal hyperactivities at the
anterior end of
the 2nd-8th rib of adults, which are in the 5th place with respect to their
frequency
in AHYS. The diagnostic significance of sternocostal-joint involvement in
AHYS can
likewise be recognized by bone-scan scintigraphy and will be discussed. Bone
scintigraphy is more sensitive than radiomorphological imaging in AHYS.
This,
however, only applies under three conditions. 1. The increased radiotracer
uptake in
the upper sternocostoclavicular region must be assessed on both the
anterior and the
posterior view of the ACW scan. 2. In addition to the anterior view of the
routine
scintiscan, further anterior scans with reduced scan time of the gamma
camera are
usually necessary. This ensures better visibility of the involvement of
certain
morphological structures that are important for AHYS diagnosis. Moreover, a
statement can be made about the inflammatory ossifying activity/inactivity
of the
AHYS on ACW.3. Increased radionuclide uptake in the manubrium sterni and
corpus
sterni on the anterior scan should be verified by additional lateral or
oblique scans
of the thorax (sternum).
============================================================
21.) The course of the SAPHO syndrome during pregnancy.
============================================================
Pons JC; Nekhlyudov L; Delfraissy JF
Service de Gynecologie-Obstetrique I, Hopital Cochin-Port-Royal, Paris,
France.
Eur J Obstet Gynecol Reprod Biol (IRELAND) Dec 27 1996 70 (2) p215-6
ISSN: 0301-
2115
Language: ENGLISH
Document Type: JOURNAL ARTICLE
Journal Announcement: 9706
Subfile: INDEX MEDICUS
In this report, we describe a case of a pregnancy in a 29 year old patient
suffering from a new syndrome, the SAPHO syndrome (synovitis, acne,
pustulosis,
hyperostosis, osteitis). The SAPHO syndrome improved considerably during
the course
of this pregnancy and treatment with indomethacin ceased during gestation.
============================================================
22.)[Lyme disease and SAPHO syndrome]
Enfermedad de Lyme y sindrome SAPHO.
============================================================
de Vega Santos T; Haro Herrero M; Montolio Donate S; Zamarron Moreno A;
Pascual de
Pablo E; Lopez Lopez C
Servicio de Medicina Interna, Hospital Comarcal Santiago Apostol, Miranda
de Ebro,
Burgos.
An Med Interna (SPAIN) Jan 1997 14 (1) p28-30 ISSN: 0212-7199
Language: SPANISH Summary Language: ENGLISH
Document Type:
JOURNAL ARTICLE English Abstract
Journal Announcement: 9706
Subfile: INDEX MEDICUS
Joint manifestation in Lyme disease hare shown similarities to those in
reactive
arthritis. SAPHO syndrome can also be included into the group of
seronegative
spondyloarthrophaties. It is presented a patient with joint, ocular and
serological
features proper of Lyme disease and after ones suggestive of SAPHO
syndrome, who
responds properly to an antibiotic treatment. It is considered the
possibility that
some patients suffering from SAPHO syndrome can also be suffering from Lyme
disease.
============================================================
23.) Spondylodiscitis in SAPHO syndrome. A series of eight cases.
============================================================
Toussirot E; Dupond JL; Wendling D
Department of Rheumatology, University hospital J Minioz, Besancon, France.
Ann Rheum Dis (ENGLAND) Jan 1997 56 (1) p52-8 ISSN: 0003-4967
Language: ENGLISH
Document Type: JOURNAL ARTICLE
Journal Announcement: 9705
Subfile: INDEX MEDICUS
OBJECTIVE: To determine the frequency, clinical features, and
radiological and bone
scintigraphic changes of spondylodiscitis in patients with SAPHO
(synovitis, acne,
pustulosis, hyperostosis, and osteitis). METHODS: The study was
retrospective. Data
from patients with the diagnostic criteria of SAPHO syndrome were analysed
for
clinical features, biological data (HLA B antigen), and pelvic and spine x
rays.
Spine computed tomography (CT) or magnetic resonance imaging (MRI) were
also examined
in some cases. RESULTS: 25 patients with a diagnosis of SAPHO were seen
since 1985.
Eight had spondylodiscitis. These included five with palmoplantar
pustulosis, one
with pustular psoriasis, one with psoriasis vulgaris, and one with chronic
recurrent
multifocal osteomyelitis without skin disease. The skin lesion occurred
before the
spondylodiscitis in four cases. Chest wall involvement was observed in
four cases
and sacroiliac joint lesions only in the case with osteomyelitis. HLA B27
was always
negative and HLA B8 was found in four cases. Radiological findings
consisted of
erosive or sclerosing remodelling of endplates with a narrowed disc space;
a reduced
height of the vertebral body was also observed in some cases. These spinal
lesions
occurred in the three vertebral segments. Multiple sites of
spondylodiscitis in the
same patient were common. Bone scan showed mildly increased uptake and CT
and MRI
were useful for detecting signs of infection such as abscess. Enhanced
signals on T2
weighted sequence or after injection of gadolinium were often observed.
Follow up
study of most of these patients suggests that the prognosis of
spondylodiscitis in
the SAPHO syndrome is favourable. CONCLUSIONS: Despite few description in
the
literature, spondylodiscitis in the SAPHO syndrome is common (32% in this
series).
These radiological findings are similar to the discovertebral changes of
spondylodiscitis in ankylosing spondylitis, thus giving support to the
relations
between SAPHO syndrome and spondylarthropathies. However, this does not
mean that
the pathogenic mechanisms are the same in these two conditions.
============================================================
24.) [Lumbo-sacroiliac hyperostosis with retroperitoneal fibrosis in
spondarthritis hyperostotical pustulo-psoriatica--with sternocostoclavicular hyperostosisand superior and inferior venous obstruction syndrome: pathogenetic hypothesisof fibro-osteopathia psoriatica]
============================================================
Die lumbo-sakro-iliakale Hyperostose mit retroperitonealer Fibrose bei
Spondarthritis hyperostotica pustulo-psoriatica--Mit
sterno-kosto-klavikularer
Hyperostose und mit oberem und unterem venosen Obstruktionssyndrom:
Pathogenetische
Hypothese einer Fibro-Osteopathia psoriatica.
Schilling F; Schweden F
Freier wissenschaftlicher Mitarbeiter in der Klinik fur Radiologie,
Johannes-
Gutenberg-Universitat Mainz.
Z Rheumatol (GERMANY) Sep-Oct 1996 55 (5) p331-47 ISSN: 0340-1855
Language: GERMAN Summary Language: ENGLISH
Document Type:
JOURNAL ARTICLE English Abstract
Journal Announcement: 9705
Subfile: INDEX MEDICUS
We report two cases of retroperitoneal fibrosis which were found in 38
patients
with "spondarthritis hyperostotica pustulo-psoriatica" (spond.hyp.p.-p.).
Spond.hyp.p.-p. is associated with sternocosto-clavicular hyperostosis and
resembles
pustulotic arthroosteitis and SAPHO syndrome. However, as dermatologically
and osteo-
histopathologically documented, it is an enthesiopathic and hyperostotic
form of
spondarthritis psoriatica. Both cases show a lumbosacroiliac hyperostosis
(LSIH)
which is a rare analogue to sternocosto-clavicular hyperostosis (SCCH).
Facultative
paraosseous connective-tissue proliferation results in a new form of
retroperitoneal
fibrosis with sacroiliac origin. Mediastinal and pelvic masses cause
superior and
inferior vena caval obstruction syndromes. Because of proven psoriasis in
pelvic
bone with para-periostal
============================================================
25.) Sapho syndrome associated with acne fulminans and prominent
acromioclavicular joint involvement.
============================================================
AU: Bolukbas-N; Bolukbas-S; Erel-A; Tali-ET
AD: Department of Physical Medicine and Rehabilitation, Gazi University
Medical School, Ankara, Turkey.
SO: Scand-J-Rheumatol. 1996; 25(3): 180-2
ISSN: 0300-9742
PY: 1996
LA: ENGLISH
CP: NORWAY
AB: The clinical and radiological findings of a 33 year-old male patient
with acne fulminans and associated SAPHO syndrome are described. The
patient presented with prominent acromioclavicular joint involvement,
unilateral sacroiliitis and subclavian vein compression which are
relatively uncommon features of this syndrome.
============================================================
26.) An aseptic inflammation of the clavicle in a patient with Crohn's
disease. A potential manifestation of the SAPHO syndrome.
============================================================
AU: Kotilainen-PM; Laxen-FO; Manner-IK; Gullichsen-RE; Saario-RM
AD: Department of Medicine, University of Turku, Finland.
SO: Scand-J-Rheumatol. 1996; 25(2): 112-4
ISSN: 0300-9742
PY: 1996
LA: ENGLISH
CP: NORWAY
AB: A female patient with Crohn's disease developed an septic
osteoarticular involvement of the left clavicle and sterno- and
acromioclavicular regions. Repeated surgical revisions combined with a
broad-spectrum antimicrobial treatment could not prevent the progress of
the disease. However, the patient started to improve after the diagnosis of
the SAPHO (synovitis, acne, pustulosis, hyperostosis and osteomyelitis)
syndrome was made and the dose of her immunosuppressive therapy increased.
This patient reminds of the existence of extraintestinal aseptic infections
in association with inflammatory bowel disease (IBD). Moreover, it may
provide further evidence on the significant association between SAPHO and
============================================================
27.) SAPHO syndrome: a followup study of 19 cases with special emphasis on
enthesis involvement.
============================================================
AU: Maugars-Y; Berthelot-JM; Ducloux-JM; Prost-A
AD: Rheumatology Department, Nantes University Hospital CHU, France.
SO: J-Rheumatol. 1995 Nov; 22(11): 2135-41
ISSN: 0315-162X
PY: 1995
LA: ENGLISH
CP: CANADA
AB: OBJECTIVE: To study the natural history, prognosis, and nosology of
synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome.
METHODS: We performed clinical, radiological, and biological reassessment
of 19 cases of SAPHO syndrome (13 with pustulosis palmaris et plantaris, 3
with acne, 3 with no dermatological manifestations) after a mean disease
course of 12.3 yrs (1-35). RESULTS: Similar involvement was noted in all
patient groups. Despite persistent remittent attacks in 11/19 cases and new
localizations in 10/19, the prognosis is promising and 18/19 patients
considered that improvement has occurred. Enthesis involvement,
occasionally associated with osteolysis, was in most cases the first event
leading to hyperostosis, but tended to disappear in the spine and
peripheral joints once synostosis was achieved. The behavior of
osteochondral cartilage was similar to that of the enthesis, whereas the
ribs below T6 were spared. We report the first descriptions of hyperostosis
of the first costotransversal joint, interspinal ossification, erosion of
wrist bones, followed by synostosis, and formation of bony bridges between
ribs. CONCLUSION: SAPHO syndrome appears to be a relevant disease process
with good prognosis. Although aseptic osteitis is regarded as the hallmark
of this entity, our followup study strongly suggests that the initial
disease targets are enthesis and related tissues. Forty-three percent of
our patients met criteria of the European Spondyloarthropathy Study Group
for spondyloarthropathy, but only one had the HLA-B27 phenotype. Thus, it
may be considered that SAPHO syndrome is nosologically related to
spondyloarthropathy, especially those types associated with psoriasis, but
probably differs in pathogenesis.
============================================================
28.) SAPHO syndrome.
============================================================
AU: Cotten-A; Flipo-RM; Mentre-A; Delaporte-E; Duquesnoy-B; Chastanet-P
AD: Department of Radiology, Hospital B, Centre Hospitalier Regional
Universitaire de Lille, France.
SO: Radiographics. 1995 Sep; 15(5): 1147-54
ISSN: 0271-5333
PY: 1995
LA: ENGLISH
CP: UNITED-STATES
AB: Palmoplantar pustulosis and severe acne are sometimes associated with
peculiar aseptic skeletal conditions, but such skeletal lesions can be
found without skin lesions. The term SAPHO syndrome has been coined for
this cluster of manifestations. (The acronym SAPHO refers to synovitis,
acne, palmoplantar pustulosis, hyperostosis, and osteitis.) The most common
site of the disease is the upper anterior chest wall, characterized by
predominantly osteosclerotic lesions, hyperostosis, and arthritis of the
adjacent joints. Osteosclerosis of the vertebral bodies, hyperostosis, and
erosions of the vertebral plates can be encountered. Unilateral
sacroiliitis is frequently observed. Long bone involvement consists of
osteosclerosis or osteolysis with periosteal new bone formation. Peripheral
arthritis can be present but is rarely associated with joint destruction.
The pathogenesis of this syndrome remains unknown, but a link with
seronegative spondyloarthropathies is probable. Radiologists should be
aware of this unusual syndrome to avoid misdiagnosis (eg, tumor,
infection), unnecessary surgery, and antibiotic therapy.
============================================================
29.) Psoriatic arthritis and synovitis, acne, pustulosis, hyperostosis,
and osteitis syndrome.
============================================================
SO - Curr Opin Rheumatol 1993 Jul;5(4):428-35
AU - Kahn MF
PT - JOURNAL ARTICLE; REVIEW (72 references); REVIEW, TUTORIAL
AB - Psoriatic arthritis is an inflammatory arthropathy associated with
psoriasis, and its clinical presentation varies from case to case. Distal
interphalangeal involvement is characteristic but not seen in all patients.
Enthesopathy, including that of the spine, is common and contributes to the
classification of psoriatic arthritis as a seronegative
spondyloarthropathy. The etiopathogenesis of psoriatic arthritis is not
well understood, and evolution as measured by follow-up is variable.
Treatment includes nonsteroidal anti-inflammatory drugs and some of the
drugs used in the treatment of rheumatoid arthritis. Sulfasalazine and, in
the more severe cases, cyclosporine, are being studied for efficacy and
tolerance. Some cases of psoriatic arthritis are associated with an
inflammatory bone disease, frequently seen on the anterior chest wall,
which is part of the newly described SAPHO (synovitis, acne, pustulosis,
hyperostosis, and osteitis) syndrome. SAPHO syndrome is characterized by
this bone involvement, which can affect the spine and peripheral bones.
Various skin conditions are associated with this syndrome, but they do not
necessarily occur in all cases. Chronic recurrent multifocal osteomyelitis,
which is seen mostly in children, may be a presentation of SAPHO syndrome.
Associations with sacroiliitis, bowel disease, and psoriasis link SAPHO
syndrome with the spondyloarthropathies.
============================================================
30.) The SAPHO syndrome.
============================================================
SO - Acta Derm Venereol 1993 Jun;73(3):208-9
AU - Vohradnikova O; Zaruba F; Polaskova S; Hoza J; Leiska A
PT - JOURNAL ARTICLE
AB - We report two cases of the SAPHO syndrome (synovitis, acne
conglobata, pustulosis palmoplantaris, hyperostosis and osteitis). This
syndrome has been published in the pediatric and rheumatological literature
in recent years.
============================================================
31.) SAPHO syndrome [see comments]
============================================================
CM - Comment in: Dermatology 1993; 186(3):161-2
SO - Dermatology 1993;186(3):176-80
AU - Brandsen RE; Dekel S; Yaron M; Caspi D; Ophir J; Brenner S
PT - JOURNAL ARTICLE
AB - Four women with pustulosis palmoplantaris (PPP) and associated
sternocostoclavicular hyperostosis are described. The connection between
the two diseases is not coincidental: it is encountered in 9.4% of PPP
patients, and was reported in the French literature as part of the SAPHO
(synovitis acne pustulosis hyperostosis osteomyelitis) syndrome. This
syndrome is linked to the spondyloarthropathies, having an increased
prevalence of HLA B27 and occurrence of sacroiliitis. None of our patients
had HLA B27, but HLA A26 was found in the 3 patients examined. No
conclusion can be drawn from this finding, however, since this antigen is
found in 21.7% of Ashkenazi Jews. Recognition of this syndrome by
dermatologists will improve diagnosis and treatment.
============================================================
32.) SAPHO syndrome.
============================================================
SO - Rheum Dis Clin North Am 1992 Feb;18(1):225-46
AU - Kahn MF; Chamot AM
PT - JOURNAL ARTICLE; REVIEW (148 references); REVIEW, TUTORIAL
AB - SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome
put together osteoarticular lesions described separately under numerous
denominations, such as multifocal osteomyelitis, pustulotic arthroosteitis,
acne rheumatism. The association of sterile inflammatory bone lesions and
neutrophilic skin eruptions is the cornerstone of this new syndrome, which
also has links with spondyloarthropathies and plain psoriasis.
============================================================
33.) Is diffuse sclerosing osteomyelitis of the mandible part of the
synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome?
Analysis of seven cases.
============================================================
SO - Oral Surg Oral Med Oral Pathol 1994 Nov;78(5):594-8
AU - Kahn MF; Hayem F; Hayem G; Grossin M
PT - JOURNAL ARTICLE
AB - Diffuse sclerosing osteomyelitis of the mandible has been described
as a localized disease of unknown origin. We offer evidence that mandibular
involvement in the recently described synovitis acne pustulosis
hyperostosis osteitis syndrome exactly fits the accepted description for
diffuse sclerosing osteomyelitis of the mandible. The clinical, radiologic,
and pathologic findings in seven such cases are presented and discussed.
============================================================
34.) Sternoclavicular pustulotic osteitis (SAPHO). 20-year interval
between skin and bone lesions.
============================================================
SO - J Rheumatol 1991 Jul;18(7):1104-8
AU - Kahn MF; Bouvier M; Palazzo E; Tebib JG; Colson F
PT - JOURNAL ARTICLE
AB - In 2 cases of palmoplantar pustulosis, a 20-year interval was
observed between the skin lesion and the bone involvement. In one case, the
bone lesion came first. In the other a skin lesion was seen for 3 weeks
without subsequent relapse. The 2 cases underline the multifaceted
presentation of the condition for which we have coined the name synovitis
acne pustulosis hyperostosis osteitis (SAPHO) syndrome.
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35.) Recent developments in psoriatic arthritis.
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SO - Curr Opin Rheumatol 1994 Jul;6(4):378-84
AU - Cuellar ML; Silveira LH; Espinoza LR
PT - JOURNAL ARTICLE; REVIEW (45 references); REVIEW, TUTORIAL
AB - Psoriatic arthritis affects 5% to 7% of patients with psoriasis.
Genetic, immunologic, and environmental factors play a role in its
pathogenesis. The role of inflammatory cytokines has been better defined,
and recent immunohistochemical studies of the synovial membranes have shown
important differences and similarities between psoriatic arthritis and
rheumatoid arthritis. The association of psoriatic arthritis with
infection, particularly HIV, remains an interesting observation. The most
common clinical presentation appears to be peripheral polyarticular, and
extra-articular manifestations including the SAPHO (synovitis, acne,
pustulosis, hypertosis, and osteitis) syndrome are not common. Methotrexate
and sulfasalazine therapy are effective in patients who do not respond to
nonsteroidal anti-inflammatory drugs.
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36.) Miscellaneous neurologic, cardiac, pulmonary, and metabolic disorders
with rheumatic manifestations.
============================================================
SO - Curr Opin Rheumatol 1993 Jan;5(1):104-10
AU - Larkin JG
PT - JOURNAL ARTICLE; REVIEW (54 references); REVIEW, TUTORIAL
AB - Problems both old and new are featured in this year's selection of
rheumatologic aspects of miscellaneous diseases. Paralysis of one or more
limbs can lead to many musculoskeletal complications, and the approach of
Auguste Dejerine-Klumpke in 1918 can be compared with that of the
present-day physician. The reappearance of rheumatic fever continues to
excite interest. The specificity of the modified Jones criteria has been
questioned, as have the benefits of long-term antibiotic prophylaxis
following an attack of the disease. Meanwhile, metabolic disorders may be
the first diseases to come under novel scrutiny using the techniques of
genetic engineering, with outstanding possibilities for advancing both
understanding and treatment. Dermatologic diseases other than psoriasis may
be associated with arthropathy. Many of these symptom complexes may be
variants of the recently described SAPHO (synovitis, acne, pustulosis,
hyperostosis, and osteitis) syndrome.
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37.) [Lymphoplasmacellular osteomyelitis]
TO: Lymphoplasmazellulare Osteomyelitis.
============================================================
AU: Krauspe-R; Girschick-H; Huppertz-HI
AD: Orthopadische Klinik, Konig-Ludwig-Haus, Julius-Maximilians-Universitat
Wurzburg.
SO: Orthopade. 1997 Oct; 26(10): 894-901
ISSN: 0085-4350
PY: 1997
LA: GERMAN; NON-ENGLISH
CP: GERMANY
AB: Chronic lymphoplasmacellular osteomyelitis may occur in children,
adolescents and adults, but has not been found in newborns or babies either
in our series or in the literature. Symptoms suggesting an acute disease
like fever are uncommon, but a primary chronic course with symptomatic and
asymptomatic periods is typical. Pain and swelling are the main symptoms;
painless masses are rare. In children and adolescents the clavicle and
metaphyseal regions of long bones are typical sites of chronic abacterial
osteomyelitis. In adults the clavicles or the first two ribs are mainly
affected with synovitis of the adjacent joints, but the long bones are
rarely involved. Laboratory findings are non-specific but important for the
differential diagnosis. The sedimentation rate and c-reactive protein might
be elevated. The X-ray examination shows osteolytic, sclerotic or mixed
bony changes and, in case of a diaphyseal involvement, onionskinlike
periosteal bone formation may be present, suggesting a malignant process.
In late stages sclerotic bone formations may be seen as a rest. Uni- or
multifocal lesions can be detected by bonescan, as can asymptomatic
lesions. Magnetic resonance imaging shows gross signal intensity
differences both in the bone and perifocal soft tissue and involvement of
the synovium with gadolinium DPTA enhancement in T1-weighted images. In
early stages of the disease granulocytes, microabscesses and new bone
formations might suggest bacterial osteomyelitis that cannot be
differentiated by histology. In intermediate phases lymphocytic and
plasma-cellular infiltrates are found, whereas in late phases sclerotic
bone formations and fibrosis of the bone marrow are seen histologically. In
chronic lymphoplasmacellular osteomyelitis, all clinical, radiological and
histological findings, as well as negative bacteriological cultures, are
mandatory and will allow a definitive diagnosis to be made. The disease may
be uni- or multifocal, and new bone lesions may occur over time, as well as
skin manifestations, which can be found years before or after bone
involvement. The association with dermatological diseases and/or synovitis
led to the acronym SAPHO syndrome (synovitis, acne, pustulosis,
hyperostosis, osteomyelitis). For the treatment nonsteroidal
anti-inflammatory drugs are effective for pain relief, reduction of
swelling and dysfunction. Antibiotics have been used in several series and
are not effective. Major surgery is not recommended even for recurrences,
and the prognosis for growth and function is excellent in the long term
despite recurrences over several years.
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38.) [Primary chronic osteomyelitis]
TO: Primare chronische Osteomyelitis.
============================================================
AU: Jundt-G; Jani-L
AD: Knochentumor-Referenzzentrum, Instiut fur Pathologie,
Kantonsspital/Universitatskliniken Basel.
SO: Orthopade. 1997 Oct; 26(10): 889-93
ISSN: 0085-4350
PY: 1997
LA: GERMAN; NON-ENGLISH
CP: GERMANY
AB: The term primary chronic osteomyelitis covers a heterogeneous spectrum
of clinical features that should be separated for therapeutic reasons.
Unifocal manifestations should be distinguished from multifocal ones. While
bacteria are often found in the first group, bacteriological investigations
are usually negative in the second group. Additionally, multifocal forms
often recur (chronic multifocal recurrent osteomyelitis), possess a long
clinical course and may be combined with dermatological disorders (SAPHO
syndrome). A biopsy is often necessary for tumor exclusion and obtaining
material for bacteriology. Histology cannot differentiate between the
respective forms of chronic osteomyelitis.
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39.) Aseptic spondylitis as the initial manifestation of the SAPHO syndrome.
============================================================
AU: Kotilainen-P; Gullichsen-RE; Saario-R; Manner-I; Kotilainen-E
AD: Department of Medicine, Turku University Central Hospital, Finland.
SO: Eur-Spine-J. 1997; 6(5): 327-9
ISSN: 0940-6719
PY: 1997
LA: ENGLISH
CP: GERMANY
AB: We describe the case of a 61-year-old female patient who presented with
spondylitis of the lumbar spine. Although the microbiological cultures of
the bone biopsy specimens obtained during laminotomy remained negative, the
patient was treated with broad-spectrum antimicrobials for 2 months. Eight
months later she started to suffer from pain and tenderness in her sternum
and the medial portion of her left clavicle. The findings of computed
tomography and gallium-labelled isotope scan were indicative of
sternoclavicular arthritis. Again, all surgically obtained biopsy specimens
yielded negative results in microbiological studies. The diagnosis of the
SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteomyelitis)
syndrome was then made based on the clinical presentation with recurrent
sterile osteitis in two characteristic locations, and the patient was
started on immunosuppressive therapy. This case is a reminder that SAPHO
may sometimes occur without any skin manifestations. Since this type of
patient may be admitted to an orthopedic ward, it is important that
orthopedic surgeons are familiar with the syndrome.
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DATA-MEDICOS/DERMAGIC-EXPRESS No (78) 19/10/99 DR. JOSE LAPENTA R.
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