Synovitis-acne-pustulosis-hyperostosis-osteitis syndrome: A dermatologist's diagnostic dilemma

SAPHO syndrome with TMJ involvement: review of the literature and case presentation

“…Skin lesions may include forms of PPP, pustular psoriasis, acne conglobata, acne fulminans, and hidradenitis suppurativa 36 . Overall the dermatologic manifestations are difficult to eradicate and show a protracted course of remissions and exacerbations 3,11,32 . Skin treatments include topical corticosteroids, tetracycline and other antimicrobials, retinoids, psoralenultraviolet A therapy, intravenous isotre- tinoin, and many others 16 .…”

“…The absence of skin lesions does not exclude SAPHO as a diagnosis; skin lesions may be absent in 25% of cases 4,11,12,16,32,34 .…”

“…Most laboratory tests return in the normal range with the occasional exception of an elevated erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) 3,4,16,21,27,34,44 . The diagnosis is challenging because not all symptoms ( Table 2) are present in each patient and when they are present, some may be subtle 11,28,32 .…”

Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome complicated by seven pulmonary emboli in a 15-year old patient

“…These skin findings can be severe, mild, or even non-existent, and may not occur simultaneously with bone disease, potentially creating a diagnostic challenge for practitioners. 2 The hallmark of SAPHO syndrome is involvement of the anterior chest wall with a noninfectious inflammatory osteitis. Despite being described as noninfectious, deep bone biopsies of the sternum and clavicle have been able to isolate Propionibacterium acnes, supporting the hypothesis that P acnes may be involved in pathogenesis.…”

Response to infliximab in SAPHO syndrome

“…Skin lesions include pustulosis, usually observed as pustular psoriasis or palmoplantar pustulosis (PPP), and severe acne 1. Far less frequently, hidradenitis suppurativa and other findings of the follicular occlusion syndrome (that is, acne conglobata, dissecting cellulitis of the scalp), and neutrophilic dermatosis, such as ulcerative pyoderma gangrenosum (PG), Sweet’s syndrome, and Sneddon Wilkinson disease have been reported in association with the syndrome 2. Furthermore, an enteropathic variant with coexistence of inflammatory bowel disease has been recorded 3…”