Scleredema of Buschke: A report of associated acute bowel obstruction and review of systemic complications

Abstract

Scleredema adultorum (Buschke) is a rare connective tissue disorder classically presenting in association with one of the following scenarios. Subtype 1: Post-Infective (often streptococcal) Subtype 2: Blood dyscrasias (commonly multiple myeloma) Subtype 3: Diabetic (usually type 2 and poorly controlled) Patients develop taut “unpinchable” thickened skin typically on the posterior neck, upper back and shoulders. Treatment options are limited but UVA1 Phototherapy has recently found favour. Systemic involvement and complications are infrequent. We present a 53-year-old diabetic male who while undergoing UVA1 Phototherapy for the condition, developed acute bowel obstruction necessitating laparotomy, as a result of retroperitoneal involvement of this scleredematous process. We further review the literature regarding reported significant complications, monitoring and management of this unusual condition.<br />