Chronic Recurrent Multifocal Osteomyelitis (CRMO) and Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome with Associated Neutrophilic Dermatoses: A Report of Seven Cases and Review of the Literature

Abstract

A growing body of literature has identified the association between neutrophilic dermatoses and multifocal, aseptic bone lesions in children, termed chronic recurrent multifocal osteomyelitis (CRMO). Classically, patients present with swelling, pain, and impaired mobility of the affected area, with skin lesions developing concurrently or in the future. Bone biopsy reveals inflammatory changes consistent with infectious osteomyelitis, but cultures and histologic staining invariably fail to identify an infectious source. Patients are refractory to antibiotic therapy, but dramatically respond to systemic steroids and may need to be maintained on low-dose steroids to prevent relapse. Numerous authors have suggested that CRMO and synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome lie along the same clinical spectrum. In fact some believe that CRMO is the pediatric presentation of SAPHO. The two syndromes share numerous characteristics, including osteitis, a unifocal or multifocal presentation, hyperostosis, and pustulosis, which all occur in a generally healthy individual. Our seven patients, five of whom were diagnosed with CRMO, and two of whom were diagnosed with SAPHO syndrome further strengthen the idea that CRMO and SAPHO syndrome do indeed lie along the same clinical spectrum. In addition, we include two rare cases of pediatric Sweet's syndrome with evidence of pathergy.

Chronic recurrent multifocal osteomyelitis with an atypical presentation in an adult man

“…In one other case of CRMO in an adult man, he subsequently developed adult-onset Still's disease and was successfully treated with anakinra [16]. CRMO and SAPHO share numerous characteristics such as osteitis, and unifocal or multifocal presentation, and pustulosis in a generally healthy individual [17]. However, CRMO usually affects the extremities, whereas SAPHO presents in the axial skeleton, classically the anterior chest wall.…”

Dermatoses neutrofílicas: parte II

“…135 CRMO is a disease almost exclusive to pediatric patients. The presentation of the disease is insidious, with periods of remission and exacerbation of pain and edema in the affected bones.…”

Chronic recurrent multifocal osteomyelitis exhibiting predominance of periosteal reaction

“…Typically, the levels of complement components (C3 and C4), IgA and IgM do not change. 1,5,7,8,10 HLA-27 is positive in less than 30% of the patients. 1,9,11 Performing a biopsy is important to rule out other diagnostic hypotheses.…”

“…1 Positive personal or family history of psoriasis, palmoplantar pustules, chronic inflammatory bowel disease or other autoimmune diseases are also common (up to 50% of cases). [7][8][9] In active disease, laboratory tests show a mild to moderate increase in ESR and CRP, with normal blood count or, more rarely, mild leukocytosis and anemia. Typically, the levels of complement components (C3 and C4), IgA and IgM do not change.…”

“…Histological findings are nonspecific and often similar to those found in septic osteomyelitis, but with no evidence of infection by any microorganism. 1,[2][3][4][5][6][7][8][9][10] Imaging tests such as radiography, CT, magnetic resonance imaging (MRI) and scintigraphy are some of the diagnostic tools. The latter two are most commonly used to rule out other diseases and to detect asymptomatic sites employing whole body screening techniques.…”