Early stage prurigo pigmentosa : a case report

Abstract

Prurigo pigmentosa is a rare inflammatory dermatosis that primarily affects adolescents and young adults. Even though it is most commonly seen in Japanese women, other countries have reported cases with increasing frequency. It is characterized by erythematous papules and macules on the trunk, neck and chest that resolve leaving a reticulate hyperpigmentation. Some endogenous factors related with ketosis like fasting, diet, diabetes, pregnancy and exogenous agents like chrome, nickel, para-amino compounds have been accused of playing a role in etiology. Here we would like to present a case of a 16-year-old female patient who was referred to our clinic with pruritic lesions on the trunk and neck, consistent with the initial phase of prurigo pigmentosa, after a period of strict diet. Prurigo pigmentosa is a disease with distinctive histologic and clinical features. Due to its rare occurrence, an accurate diagnosis may be particularly challenging. Clinicopathological correlation is therefore crucial in the diagnosis of the disease in its early phase.

Prurigo Pigmentosa: A Case Report With Unusual Presentation

“…The etiology of PP is not fully understood. However, there are some endogenous factors and exogenous factors that have been implicated in the pathogenesis of the disease [2]. Here we present an unusual case of PP that presented as small lesions in the midline in the sacral area in the lower back.…”

Symmetrical pruritic lesions on the trunk in a young woman – a rare case of non‐associated Prurigo pigmentosa in Germany

“…2,3 In recent years, PP has been increasingly diagnosed outside Asia and in other ethnic groups. [4][5][6][7] The cause and pathogenesis have yet to be determined. Among patients with PP are pregnant women, patients with atopic diathesis, diabetes or malnutrition and other diseases.…”

“…1,5 The increasing number of reported cases of PP outside Japan and Asia underlines a possibly much higher incidence. 2,[4][5][6][7] In cases of young women suffering from intractable, highly pruritic skin eruptions with a symmetrical distribution on the upper back and/or in the inframammary region, especially in cases without response to glucocorticoids, dermatologists should consider PP even in countries outside Asia.…”

“…2,[8][9][10] In most cases, PP occurs in the 2nd or 3rd decade of life, with women being affected about 2-3 times more often than men. PP often starts in spring or summer 6 and shows an acute onset with intensely pruritic, erythematous urticarial papules, papulo-vesicles or vesicles with a symmetrical reticular pattern of distribution. The upper part of the back, the neck and the chest are mostly affected.…”

“…5 Recently, nemolizumab, an anti-IL 31 receptor A has been evaluated in in phase I-II studies showing a good safety profile and significant decreasing of pruritus. 6 Nowadays, chronic pruritus mechanisms are better understandable, dupilumab has allowed an important reduction of this symptom, but studies on larger cohorts of patients are necessary. In conclusion, our real-life experience can be summarized in the following points: from the first month the itching improves with a consequent improvement of the DLQI.…”