Three cases of IL36RN-associated pustulosis: An evolution of acrodermatitis continua of Hallopeau to generalized pustular psoriasis.

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Date: Sept-Oct 2020
Publisher: Medknow Publications and Media Pvt. Ltd.
Document Type: Article
Length: 1,101 words
Lexile Measure: 1410L

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Byline: Yun-Liu. Chen, Zhao-Yang. Wang, Lin. Ma, Zi-Gang. Xu


Generalized pustular psoriasis is characterized by generalized erythema and pustules accompanied by severe systemic symptoms. Acrodermatitis continua of Hallopeau is a rare localized pustulosis involing the digits and nails. It remains controversial whether acrodermatitis continua of Hallopeau is an independent disease or a localized form of generalized pustular psoriasis. Co-occurrence of both in one person has been reported.[1] In addition, the same IL36RN mutation loci were identified in a boy with acrodermatitis continua of Hallopeau and in his sister with generalized pustular psoriasis.[2] Thus, we speculated that the two might belong to a disease spectrum that shares the same pathogenesis. Herein, we report three children who experienced both acrodermatitis continua of Hallopeau and generalized pustular psoriasis. The IL36RN gene mutation was identified in all patients.

Patient 1 was an 8-year-old girl. She presented with generalized erythema, pustules and high fever when she was 5 years old and was diagnosed with generalized pustular psoriasis. After 2 months of treatment with acitretin (0.5 mg/kg/day), the pustules disappeared. After 2 years, erythema and pustules emerged on her distal fingertips and on the tips of her toes, which was accompanied by damage to her nails. A diagnosis of acrodermatitis continua of Hallopeau was made and oral acitretin (0.5 mg/kg/day) was given again. However, she experienced a repeated relapse-remission process during the 14-month treatment.

Patient 2 was a 9-year-old girl. When she was 1 year old, generalized pustules emerged in large areas of erythema. A diagnosis of generalized pustular psoriasis was made. The lesions disappeared on treating with topical corticosteroids. She experienced at least one relapse every 2 years thereafter,...

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Gale Document Number: GALE|A633604991