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Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome in Childhood; A Rare Clinical Entity.

Sayılı A, Tosun O, Cobanoglu N, Bahceciler Onder N, Baba F, Kavukcu S - Iran J Pediatr (2014)

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics.

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Dear Editor, Referring to synovitis, acne, pustulosis, hyperostosis and osteitis; SAPHO syndrome is defined as a chronic, relapsing rheumatologic disease of uncertain etiology characterized by distinct osteoarticular and cutaneous manifestations... Serological tests for Salmonella and Brucella were negative... Having a BCG vaccination scar, PPD was negative and no abnormalities were found in chest radiography of the patient... Bone marrow aspiration and bone scintigraphy revealed no signs of malignancy... She stopped having this treatment on her own accord, and then applied to our hospital with vesiculopustular skin lesions on the palmar, plantar and retroauricular surfaces... Histopathologic findings revealed subcorneal intraepidermal vesicles as well as intracorneal plasma insudation... Inflammation manifests in the form of sterile osteomyelitis and hyperostosis in the bone, acne or pustulosis in the skin and synovitis in the joints... In cases with involvement of atypical sites or single skeletal lesions that lack signs of hyperostosis corresponding with radiologic findings and skin manifestations, the diagnosis becomes much more challenging for SAPHO... In CRMO, like SAPHO syndrome, skin manifestations just as described in our patient, are rare findings... Since the skin findings defined pathologically in our patient are characteristic for SAPHO syndrome together with the absence of neutrophil infiltration, we strongly believe that our case has the diagnosis of SAPHO syndrome rather than CRMO... The disease course in children just like in adults, is characterized by periods of exacerbations and remissions with an increasing number of lesions over time and considered to be a relatively benign and self-limiting disease without major sequelae... Recognition of SAPHO syndrome in our case despite the atypical presentation both in terms of age and skeletal involvement seems notable, since early diagnosis is important to avoid unnecessary invasive procedures and prolonged antibiotic treatment of osteoarticular lesions... It should be considered in the differential diagnosis of hip and lower back pain.

No MeSH data available.


Related in: MedlinePlus

6 months later: signal intensity of edemain. The bilateral acetabulum has decreased, however, there are new edema signals noted in the sacrum
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Figure 3: 6 months later: signal intensity of edemain. The bilateral acetabulum has decreased, however, there are new edema signals noted in the sacrum

Mentions: Naproxen sodium treatment was continued for almost next 4 months. She stopped having this treatment on her own accord, and then applied to our hospital with vesiculopustular skin lesions on the palmar, plantar and retroauricular surfaces. Histopathologic findings revealed subcorneal intraepidermal vesicles as well as intracorneal plasma insudation. Lymphocyte exocytosis, infiltrating vesicles and epidermal cells were also visualized. Patient received oral analgesic, non-steroidal anti-inflammatory drug (Ibuprofen) and topical steroid treatment. Remarkable increase in the intensity of edema signals was notable in the evaluation of serial radiological findings (Fig 3). Naproxen sodium administration was continued and after one month there was a clinical regression in the skeletal system findings and skin lesions. Being in the 18th month, she is still under follow up with no problems occurring to date.


Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome in Childhood; A Rare Clinical Entity.

Sayılı A, Tosun O, Cobanoglu N, Bahceciler Onder N, Baba F, Kavukcu S - Iran J Pediatr (2014)

6 months later: signal intensity of edemain. The bilateral acetabulum has decreased, however, there are new edema signals noted in the sacrum
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4359425&req=5

Figure 3: 6 months later: signal intensity of edemain. The bilateral acetabulum has decreased, however, there are new edema signals noted in the sacrum
Mentions: Naproxen sodium treatment was continued for almost next 4 months. She stopped having this treatment on her own accord, and then applied to our hospital with vesiculopustular skin lesions on the palmar, plantar and retroauricular surfaces. Histopathologic findings revealed subcorneal intraepidermal vesicles as well as intracorneal plasma insudation. Lymphocyte exocytosis, infiltrating vesicles and epidermal cells were also visualized. Patient received oral analgesic, non-steroidal anti-inflammatory drug (Ibuprofen) and topical steroid treatment. Remarkable increase in the intensity of edema signals was notable in the evaluation of serial radiological findings (Fig 3). Naproxen sodium administration was continued and after one month there was a clinical regression in the skeletal system findings and skin lesions. Being in the 18th month, she is still under follow up with no problems occurring to date.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Dear Editor, Referring to synovitis, acne, pustulosis, hyperostosis and osteitis; SAPHO syndrome is defined as a chronic, relapsing rheumatologic disease of uncertain etiology characterized by distinct osteoarticular and cutaneous manifestations... Serological tests for Salmonella and Brucella were negative... Having a BCG vaccination scar, PPD was negative and no abnormalities were found in chest radiography of the patient... Bone marrow aspiration and bone scintigraphy revealed no signs of malignancy... She stopped having this treatment on her own accord, and then applied to our hospital with vesiculopustular skin lesions on the palmar, plantar and retroauricular surfaces... Histopathologic findings revealed subcorneal intraepidermal vesicles as well as intracorneal plasma insudation... Inflammation manifests in the form of sterile osteomyelitis and hyperostosis in the bone, acne or pustulosis in the skin and synovitis in the joints... In cases with involvement of atypical sites or single skeletal lesions that lack signs of hyperostosis corresponding with radiologic findings and skin manifestations, the diagnosis becomes much more challenging for SAPHO... In CRMO, like SAPHO syndrome, skin manifestations just as described in our patient, are rare findings... Since the skin findings defined pathologically in our patient are characteristic for SAPHO syndrome together with the absence of neutrophil infiltration, we strongly believe that our case has the diagnosis of SAPHO syndrome rather than CRMO... The disease course in children just like in adults, is characterized by periods of exacerbations and remissions with an increasing number of lesions over time and considered to be a relatively benign and self-limiting disease without major sequelae... Recognition of SAPHO syndrome in our case despite the atypical presentation both in terms of age and skeletal involvement seems notable, since early diagnosis is important to avoid unnecessary invasive procedures and prolonged antibiotic treatment of osteoarticular lesions... It should be considered in the differential diagnosis of hip and lower back pain.

No MeSH data available.


Related in: MedlinePlus