TY - JOUR
T1 - Disabling pansclerotic morphea
T2 - Clinical presentation in two adults
AU - Maragh, Sherry Henderson
AU - Davis, Mark D.P.
AU - Bruce, Alison J.
AU - Nelson, Audrey M.
N1 - Funding Information:
Supported by Stiefel Laboratories.
Copyright:
Copyright 2008 Elsevier B.V., All rights reserved.
PY - 2005/8
Y1 - 2005/8
N2 - 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.
AB - 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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U2 - 10.1016/j.jaad.2004.10.881
DO - 10.1016/j.jaad.2004.10.881
M3 - Article
C2 - 16021158
AN - SCOPUS:21844467204
SN - 0190-9622
VL - 53
SP - S115-S119
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
IS - 2 SUPPL.
ER -