Sclerodermiform porphyria cutanea tarda in a 64-year-old woman

Introduction. Porphyria cutanea tarda (PCT) is the most common type of porphyria caused by decreased uroporphyrinogen decarboxylase activity, which results in its elevated level. Porphyria cutanea tarda is a skin disorder, present with either bullous skin fragility or nonbullous acute photosensitivity, as well as mechanical fragility, hypertrichosis, and hyper- or hypopigmentation. Sclerotic skin changes have also been described, rarely misdiagnosed as morphea or systemic sclerosis, and therefore may be challenging in differential diagnosis.

Objective. Presentation of a case of a 64-year-old patient with PCT and sclerotic skin changes on the neck and neckline, mimicking morphea.

Case report. A 64-year-old woman was admitted to the outpatient clinic due to sclerotic skin changes on the neckline, hypertrichosis on the temples and milia and erosions on the hands. Porphyria cutanea tarda was diagnosed based on clinical symptoms and laboratory tests. After treatment with chloroquine and phlebotomy, improvement was achieved.

Conclusions. Porphyria cutanea tarda is a chronic disease with a wide range of signs and symptoms.