Format

Send to

Choose Destination
Indian J Dermatol. 2013 Mar;58(2):159. doi: 10.4103/0019-5154.108079.

Disabling pansclerotic morphea of childhood with extracutaneous manifestations.

Author information

1
Department of Dermatology, Venereology and Leprosy, Grant Medical College, Mumbai, India.

Abstract

Disabling pansclerotic morphea (DPM) of childhood is a rare generalized type of localized scleroderma (LS) that is known to follow an aggressive course with pansclerotic lesions leading to severe joint contractures and consequent immobility. Mortality is due to complications of the disease such as bronchopneumonia, sepsis, or gangrene. There is no specific laboratory finding. Treatment protocols are still evolving for this severe recalcitrant disorder. Extracutaneous manifestations are rarely reported in DPM. We present the case of a 7-year-old girl with DPM with severe extracutaneous manifestations in the form of gastrointestinal and vascular disease, whose disease progressed rapidly. In spite of treatment with methotrexate, corticosteroids, and PUVA therapy, she ultimately succumbed to her illness due to sepsis.

KEYWORDS:

Disabling pansclerotic morphea of childhood; extracutaneous manifestations; gangrene; juvenile localized scleroderma

Supplemental Content

Full text links

Icon for Medknow Publications and Media Pvt Ltd Icon for PubMed Central
Loading ...
Support Center