Adult-onset Gunther’s disease (congenital erythropoietic porphyria) in a 37-year-old man: a case report

Authors

  • Saswati Halder
  • Sumit Sen
  • Atin Halder
  • Subhas Ch. Nag
  • Dr. Rajesh

Keywords:

Congenital erythropoietic porphyria, adult, photosensitivity

Abstract

A 37-year-old man presented with multiple blisters and ulcerations over the sun exposed skin of 2 years duration. His clinical, radiological, hematological features, fluorescent microscopy of urine and histopathology of subepidermal blister with PAS-positive material deposited in the upper dermis in a perivascular and periadnexal distribution were suggestive of congenital erythropoietic porphyria, was treated with beta carotene and advised to avoid sun exposure by using sun protective clothing. We repot this case because of its rarity. 

References

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Konton AP, Ozog D, Bichakijan C, Lim HW. Congenital erythropoietic porphyria associated with myelodysplasia presenting in a 72-year-old man: report of a case and review of literature. Br J Dermatol 2003;148:160-4.

Fritish C, Bolsen K, Ruzicka T et al. Congenital erythropoietic porphyria. J Am Acad Dermatol 1997;36:594-610.

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Published

2017-01-04

How to Cite

1.
Halder S, Sen S, Halder A, Nag SC, Rajesh D. Adult-onset Gunther’s disease (congenital erythropoietic porphyria) in a 37-year-old man: a case report. J Pak Assoc Dermatol [Internet]. 2017Jan.4 [cited 2024Dec.7];22(1):63-5. Available from: http://www.jpad.com.pk/index.php/jpad/article/view/904

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Case Reports

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