Childhood sclerodermatomyositis with generalized morphea

Abstract

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.

A cross-sectional electromyography assessment in linear scleroderma patients

“…Myositis overlap at different degrees was also reported in localized scleroderma or associated connective tissue disease overlap features [29,37-39], but in these cases muscle weakness was present. None of our cases presented localized or generalized muscle weakness at any time of disease course.…”

Importance of cutaneous findings in childhood scleromyositis in Indian scenario

“…[2] A clear recognition of the cutaneous findings is essential for prompt and accurate diagnosis of DM, especially in cases in which cutaneous manifestations are the initial or only findings. Salient features of DM on skin biopsy include vacuolar alteration of the basal cell layer of the epidermis, necrotic keratinocytes and a sparse, superficial, perivascular lymphocytic infiltrate, as seen in our case.…”