Porphyria cutanea tarda mimicking progressive systemic sclerosis. Case report

  • Freddy Liñán Ponce Victor Lazarte Echegaray Hospital, Trujillo, Peru
  • Juan Leiva Goicochea Victor Lazarte Echegaray Hospital, Trujillo, Peru
  • Fernando Gross Melo Victor Lazarte Echegaray Hospital, Trujillo, Peru
  • David Sevilla Rodríguez Victor Lazarte Echegaray Hospital, Trujillo, Peru
  • Claudia Otoya Guzmán Victor Lazarte Echegaray Hospital, Trujillo, Peru
  • Daniel Corro García Victor Lazarte Echegaray Hospital, Trujillo, Peru
  • Juan Rodríguez Baldeón Victor Lazarte Echegaray Hospital, Trujillo, Peru
DOI: https://doi.org/10.47196/rar.v34i2.751
Keywords: porphyria cutanea tarda, progressive systemic sclerosis, sclerodactyly, uroporphyrin

Abstract

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.

Author Biographies

Freddy Liñán Ponce, Victor Lazarte Echegaray Hospital, Trujillo, Peru
Rheumatology Assistant Physician
Juan Leiva Goicochea, Victor Lazarte Echegaray Hospital, Trujillo, Peru
Rheumatology Assistant Physician
Fernando Gross Melo, Victor Lazarte Echegaray Hospital, Trujillo, Peru
Internal Medicine Physician Assistant
David Sevilla Rodríguez, Victor Lazarte Echegaray Hospital, Trujillo, Peru
Internal Medicine Physician Assistant
Claudia Otoya Guzmán, Victor Lazarte Echegaray Hospital, Trujillo, Peru
Rheumatology Resident Physician
Daniel Corro García, Victor Lazarte Echegaray Hospital, Trujillo, Peru
Rheumatology Resident Physician
Juan Rodríguez Baldeón, Victor Lazarte Echegaray Hospital, Trujillo, Peru
Rheumatology Resident Physician

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Published
2023-06-30
How to Cite
1.
Liñán Ponce F, Leiva Goicochea J, Gross Melo F, Sevilla Rodríguez D, Otoya Guzmán C, Corro García D, Rodríguez Baldeón J. Porphyria cutanea tarda mimicking progressive systemic sclerosis. Case report. Rev. Argent. Reumatol. [Internet]. 2023Jun.30 [cited 2025May11];34(2):69 -72. Available from: https://ojs.reumatologia.org.ar/index.php/revistaSAR/article/view/751
Issue
Vol. 34 No. 2 (2023): April-June 2023
Section
Clinical Case

Copyright (c) 2023 on behalf of the authors. Reproduction rights: Argentine Society of Rheumatology

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