SAPHO SYNDROME: A CASE DESCRIPTION

The SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a complex group of disorders characterized by peculiar bone lesions, most commonly involving the anterior chest wall, and sometimes accompanied by dermatological manifestations. There is a great degree of variability in the dermatological involvement of this syndrome. A combination of clinical, radiographic, and pathological investigation is required to establish the correct diagnosis. The incidence in children and young adults is not well established and probably underestimated. We describe the case of a 13 year old girl with a final diagnosis of SAPHO syndrome. She presented with a pustulosis of the palms and soles, characterized by sterile pustules admixed with yellow-brown macules. Scattered pustulosis on arms, legs, and face were also present. The patient was hospitalized in a pediatric unit for the occurrence of a painful tumescence of the right sternoclavicular joint accompanied with fever and stiff painful cervical spine. Other referred musculoskeletal symptoms were migrant sacroiliac joints swelling impairing deambulation. Histological examination of a skin biopsy from the plantar lesion showed sterile subcorneal pustules without psoriasic hyperplasia. The scintigraphy confirmed an enhancement of the sternoclavicular joint captation, with hyperostosis. Treatment with ASA at the dosage of 1500 mg/day allowed a progressive healing of the bone lesions. Beside, a month later lung involvement with a pulmonary nodule non responsive to antibiotics treatment occurred. Based on clinical appearance course, radiology and histology, a diagnosis of SAPHO syndrome has been established.