Disabling pansclerotic morphea: clinical presentation in two adults.
Disabling pansclerotic morphea involves all layers of the skin, extending through the dermis and subcutaneous tissues to involve muscle, tendon, and bone. It is distinguished from generalized scleroderma by its lack of systemic involvement. Onset usually occurs before the age of 14 years. We describe adult-onset disabling pansclerotic morphea in two previously healthy young men. In both cases, the onset of disease was explosive, with rapid progression, widespread cutaneous involvement, and severe disablement caused by mutilating contracture deformities. Increased susceptibility of sclerodermatous tissue to recalcitrant ulceration and malignant transformation with development of nonmelanoma skin cancers was also observed. Treatment of this disease continues to present a therapeutic dilemma with only sporadic remission despite multimodality therapy.
Maragh
Sherry Henderson
SH
Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Davis
Mark D P
MD
Bruce
Alison J
AJ
Nelson
Audrey M
AM
D000328:Adult; D017668:Age of Onset; D003286:Contracture / Q000209:etiology; D006233:Persons with Disabilities; D018450:Disease Progression; D006227:Hand Deformities, Acquired / Q000209:etiology; D006801:Humans; D008297:Male; D012594:Scleroderma, Localized / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D012883:Skin Ulcer / Q000209:etiology