Abstract

LINAN PONCE, Freddy et al. Porphyria cutanea tarda mimicking progressive systemic sclerosis. Rev. argent. reumatolg. [online]. 2023, vol.34, n.2, pp.69-72. ISSN 0327-4411.  http://dx.doi.org/10.47196/rar.v34i2.751.

Sclerodermiform syndromes usually mimic progressive systemic sclerosis very well, with the presence of skin blisters in photo-exposed areas with hyperpigmentation being the differential data for diagnosing porphyria. We present the case of a 48-year old man with photosensitivity, capillary fragility, skin blisters, and hyperpigmentation associated with sclerodactyly with distal scar tissue loss on the fingers, which perfectly simulated progressive systemic sclerosis. The analysis showed negativity for antinuclear, antitopoisomerase and anticentromere antibodies, with high levels of uroporphyrins in urine. Phlebotomy and hydroxycloquine treatment improved photosensitivity and skin fragility.

Keywords : porphyria cutanea tarda; progressive systemic sclerosis; sclerodactyly; uroporphyrin.

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