Indian Pediatrics - Editorial

Images in Clinical Practices

Indian Pediatrics 2002; 39:203

Scleroderma - CREST Syndrome

An 11-year-old girl presented with deformity of hands and ulcers on the finger tips. On physical examination, she had expression-less face with pinched nose and small mouth. Her skin over the face and distal extremities was bound down and atrophic. She had shiny hands with tapering fingers and claw hand deformity (Fig. 1). Tiny pitting scars on finger tips and telangiectasias over the face were other prominent findings. She also had a swelling in the posterior aspect of left thigh which was discharging chalky white material. The X-ray of left thigh showed extensive subcutaneous calcification (Fig. 2).

Scleroderma is a rare disease in childhood. It is a multisystem disorder of unknown cause characterized by fibrosis of skin, blood vessels and visceral organs. The CREST syndrome refers to the manifestations of calcinosis, Raynaud’s phenomenon, esophageal involve-ment, sclerodactyly and telangiectasias. Severe pulmonary hypertension may develop in some patients with CREST syndrome. There is no specific treatment for scleroderma. Immunosuppressive agents including metho-trexate and corticosteroids may be helpful if used in the early stages of the disease.

A.M. Vijayalakshmi,
Associate Professor of Pediatrics,
P.S.G. Institute of Medical Sciences and
Research, Coimbatore 641 004, India.


Fig. 1. Photograph showing expressionless face, pinched nose, small mouth and clawhand deformity.	Fig. 2. X-ray of the pelvis and left thigh showing extensive subcutaneous calcification.