Open AccessChildhood sclerodermatomyositis with generalized morphea
Author(s) -
Girishkumar R Ambade,
Rachita Dhurat,
Nitin Lade,
Hemangi Jerajani
Publication year - 2008
Publication title -
indian journal of dermatology, venereology, and leprology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.514
H-Index - 45
eISSN - 0973-3922
pISSN - 0378-6323
DOI - 10.4103/0378-6323.39702
Subject(s) - juvenile dermatomyositis , medicine , morphea , mixed connective tissue disease , dermatomyositis , anti nuclear antibody , scleroderma (fungus) , antisynthetase syndrome , rheumatology , polymyositis , dermatology , overlap syndrome , pathology , serology , connective tissue disease , autoantibody , antibody , disease , immunology , autoimmune disease , biopsy , inoculation
Systemic sclerosis (SS) and dermatomyositis (DM) are both multisystem disorders and share some common clinical features. We report here an 11 year-old girl whose disease showed a changing clinical pattern from juvenile systemic sclerosis (JSS) to slowly progressing juvenile dermatomyositis (JDM) and had associated generalized morphea. Serological studies revealed antinuclear antibodies (ANA) with a speckled pattern. Topoisomerase-I (Scl-70), U1 RNP (ribonucleoprotein), anti-Ro, anti-La and anti Jo-1 antibody tests were negative. Electromyography (EMG) was suggestive of primary muscle disease and histopathological findings indicated scleroderma. The patient fulfilled the American College Rheumatology (ACR) diagnostic criteria for JSS as well as Bohan and Peter criteria for JDM separately and hence, was diagnosed to have sclerodermatomyositis (SDM). Mixed connective tissue disease (MCTD) and antisynthetase antibody syndrome (ASS) which share same clinical features with SS and DM were excluded by immunological studies.