Disabling pansclerotic morphea: Clinical presentation in two adults - 21/08/11
Rochester, Minnesota
Abstract |
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.
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Supported by Stiefel Laboratories. Funding sources: None. Conflicts of interest: None identified. Reprints not available from the authors. |
Vol 53 - N° 2S
P. S115-S119 - août 2005 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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