Generalized morphea in a child

BK Brar, RR Gupta, A Pall, SS Sahni

 Department of Dermatology and Venereology, Leprology, Government Medical College & Hospital, Faridkot-151 203 (Pb.), India

Correspondence Address:
B K Brar
C/o Brar Eye Hospital, 637, Street No. 4, Lajpat Nagar, Kotkapura-151 204 (Pb.)
India

Introduction

Generalized Morphea is a rare condition in which idiopathic sclerosis of the skin occurs in a wide spread manner[1] frequently associated with atrophy and pigmentation. The disease is extremely uncommon in children.[2] This case is being presented because of its extreme rarity.

Case Report

A 9-year-old female child presented with gradually increasing tautness of skin and joint pains. Six months back patient had measles with fever ranging from 100°F-103°F. Following the fever, patient noticed generalized progressive tautness of skin along with severe pain in joints of hands, elbows, ankles and knees which was accompanied by limitations in movements of joints. The child experienced difficulty in opening of mouth. There was history of measles in other members of family (brother and sister) at the same time. The patient was vaccinated against BCG, DPT and Polio but not against measles. Child was in good health before the onset of this disease.

On examination, scalp showed sparse hair. Face was expressionless, statue like, the skin being shiny and stretched [Figure - 1]. Mouth opening was restricted. Patient had ulceration of hard palate. There was induration of skin of lower part of chest. Skin over both upper and lower extremities and back was elbows, knees, ankles, wrists and all interphalangeal joints were tender and swollen with painful restricted movements. Spindling and semiflexion was seen in fingers of hands and the skin was adherent to underlying tissues [Figure - 1]. Systemic examination showed no abnormality.

All the routine investigations were within the normal limit except eosinophilia (8%). ESR was 32mm in the first hour. LE cell phenomenon, RA factor, ANA and CPK were negative. Barium meal examination of the oesophagus and stomach and radiological examination of joints were normal. The histological findings were consistent with clinical diagnosis of generalized morphea.

Discussion

The exact etiology of generalized morphea is unknown.[1] The most frequent age of onset is between 30-40 years, and 80% cases start between the ages of 11-15 years.[2] The disease is common among females, male to female ratio being 1:3 Apparantly many factors appear to be responsible for the precipitation of the disease. Various predisposing factors incriminated are acute infections, trauma, exposure to cold, sunrays and emotional upsets.3 Localized morphea has also been reported after measles and varicella.1 Patient was put of 30mg of prednisolone and supportive therapy. Good response was observed but subsequently patient died of respiratory complications. Unique features to be observed in the case were ulceration of hard palate which is not usually seen in generalized morphea and mild to moderate grade fever.

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