Adult-onset unilateral disabling pansclerotic morphea

Abstract

Disabling pansclerotic morphea (DPM) is a rare, severe, and mutilating form of morphea, involving the dermis, subcutaneous tissue, fat, muscle, and even bone. It is usually seen before the age of 14 years, with the patient complaining of arthralgia and stiffness at the time of onset. We report a case of unilateral adult-onset DPM with sparing of the face. Within a few months of onset, painful contracture deformities of the hand, elbow, and shoulder joints developed, impairing the patient's normal daily activities. We are reporting this case because of the unusual presentation: DPM in an adult, with history of trauma in childhood and rapid onset of complications, is rare.

A rare and challenging presentation of adult‐onset pansclerotic morphea

“…Most evidence points towards the sparing of face, hands and feet as a hallmark of PSM. [2][3][4][5][6] In our case, this involvement was so important to the point of resembling epidermolysis bullosa.…”