Disabling pansclerotic morphea: Clinical presentation in two adults

Sherry Henderson Maragh, Mark D.P. Davis, Alison J. Bruce, Audrey M. Nelson

Research output: Contribution to journalArticlepeer-review

38 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)S115-S119
JournalJournal of the American Academy of Dermatology
Volume53
Issue number2 SUPPL.
DOIs
StatePublished - Aug 1 2005

ASJC Scopus subject areas

  • Dermatology

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