What is it about?

This article describes a case of a fulminant adult-onset pansclerotic morphea (PSM) associated with blistering and vitiligo. PSM is a rare, aggressive and mutilating variant of morphea that involves the dermis, subcutaneous tissue, muscle and even bone. Most reported cases of the disease have a childhood-onset, and recalcitrance to treatment is a major feature of this disease.

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Why is it important?

Involvement of the extremities and fingers of this magnitude has not yet been previously reported on adult PSM, such as the association between PSM and bullous morphea. In fact, most evidence points towards the sparing of face, hands and feet as a hallmark of PSM. In our case, this involvement was so important to the point of resembling epidermolysis bullosa.

Perspectives

Early diagnosis may provide the opportunity to intervene with novel antifibrotic agents, such as JAK inhibitors.

Nathalia Abreu
Hospital Eduardo de Menezes

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This page is a summary of: A rare and challenging presentation of adult‐onset pansclerotic morphea, Journal of the European Academy of Dermatology and Venereology, August 2022, Wiley, DOI: 10.1111/jdv.18522.
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