Prurigo Pigmentosa-Report of 3 Cases From Brazil and Literature Review

Prurigo pigmentosa (PP) is a rare inflammatory disease of the skin of uncertain etiology first reported in Japan. It is typified by recurrent eruptions of itching urticarial macules, papules, vesicopapules, and plaques with a reticular arrangement that quickly resolve leaving a net-like pigmentation. The disease presents specific histopathological features. Herein, 3 cases of PP in Brazilians with no Japanese ancestry are reported and a revision of all previous English-language case reports indexed on PubMed is provided. Two articles with original case reports not listed on PubMed were also included. Our patients are 2 women and 1 man at the ages of 39, 33, and 22 years, respectively. All 3 presented findings in consonance with previous cases of PP and were diagnosed based on clinicopathological correlation. They were successfully treated with oral minocycline or doxycycline. In our literature review, a total of 210 previously reported cases were included. Although PP seemed to be restricted to Japanese patients in the first years after its recognition, the geographic boundaries of the disease are continuously expanding. Korea responded for 83 previous cases and Japan for 53. The mean age was 24.4 years, with 84.3% of the cases occurring between 11 and 30 years of age. The female/male rate was 2.6 and the most affected anatomical sites were back, chest, and neck. We do believe that the rarity of case reports in western countries may represent lack of awareness about the disease by dermatologists and dermatopathologists in these regions.