DISABLING PANSCLEROTIC MORPHEA OF CHILDHOOD – A CASE REPORT

Dr. Pooja Agarwal, Dr. Snehal Chaudhari, Dr. Amit Mistry

Abstract


INTRODUCTION: Disabling pansclerotic morphea (DPM) of childhood is a rare generalized type of localized scleroderma (LS) known to follow an aggressive course with pansclerotic lesions leading to severe joint contractures and consequent immobility. It is a chronic disease of unknown etiology, seen mostly in children less than fourteen years, involving sclerosis of all the layers of skin extending rapidly through the dermis and subcutaneous tissue to involve muscle, tendon and bone. It is distinguished from generalized scleroderma by its lack of systemic involvement. 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.

CASE REPORT: 14 years old boy presented with chief complains of tightness of the skin involving all the four limbs and trunk since 1 year. There was no history of Raynaud’s phenomenon, or exposure to chemicals and dyspnea or dysphagia. On examination multiple sclerotic plaques with atrophy present on the trunk and all four limbs and a single non- healing, painful ulcer on the left foot and distal left leg.  Skin biopsy showed thickening & homogenization of collagen bundles in dermis and subcutaneous issue. He was treated with oral steroids & methotrexate without any significant benefit.

CONCLUSION: Disabling pansclerotic morphea of childhood is exceedingly rare and severe form of Morphea. It can be very disabling and sometimes fatal. Treatment continues to present a therapeutic dilemma with only sporadic remission despite multimodality therapy. Early diagnosis is essential for initiation of proper therapy. An interdisciplinary approach is indispensable for proper management.


Keywords


Disabling pansclerotic morphea of childhood, localized scleroderma, Dexamethasone pulse therapy, Methotrexate

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