open access

Vol 4, No 1 (2018)
Review paper
Published online: 2018-03-02
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Amicrobial pustulosis of the folds associated with thyroperoxidase antibodies

Monika Siedlecka1, Joanna Czuwara1, Marlena Majkut-Sobechowicz1, Katarzyna Pisarz1, Roman Nitskovich1, Lidia Rudnicka1
Forum Dermatologicum 2018;4(1):5-9.
Affiliations
  1. Klinika Dermatologiczna, Warszawski Uniwersytet Medyczny, Warszawa, Poland

open access

Vol 4, No 1 (2018)
REVIEW ARTICLES
Published online: 2018-03-02

Abstract

Amicrobial pustulosis of the folds (APF) is a rare neutrophilic dermatosis, co-existing with autoimmune disorders, especially systemic lupus erythematosus. The disease is characterized by the presence of sterile pustules localized in the skin folds, intraepidermal pustules with mainly neutrophilic infiltrate on histology, negative microbial cultures from an unopened pustule and the presence of circulating autoantibodies or autoinflammatory diseases. Due to its rare incidence, data on effective therapeutic options are limited to individual clinical cases. We present a 69-year-old man with disseminated pustular lesions in the main skin folds, focusing on the clinical, histopathological and immunological characteristics of the disease. Based on the clinical presentation and laboratory investigation, amicrobial pustulosis of the folds was diagnosed with an accompanying increased concentration of anti-thyreoperoxidase antibodies with compensated thyroid hormones. After 2 weeks of treatment with local glucocorticosteroids, skin lesions disappeared, leaving post-inflammatory discoloration. The patient remains without recurrence of any skin lesions during the five-month observation. In conclusion, in the diagnosis of this rare disease, the clinicopathological correlation plays a crucial role as well as the investigation for co-existing autoimmune disorders.

Abstract

Amicrobial pustulosis of the folds (APF) is a rare neutrophilic dermatosis, co-existing with autoimmune disorders, especially systemic lupus erythematosus. The disease is characterized by the presence of sterile pustules localized in the skin folds, intraepidermal pustules with mainly neutrophilic infiltrate on histology, negative microbial cultures from an unopened pustule and the presence of circulating autoantibodies or autoinflammatory diseases. Due to its rare incidence, data on effective therapeutic options are limited to individual clinical cases. We present a 69-year-old man with disseminated pustular lesions in the main skin folds, focusing on the clinical, histopathological and immunological characteristics of the disease. Based on the clinical presentation and laboratory investigation, amicrobial pustulosis of the folds was diagnosed with an accompanying increased concentration of anti-thyreoperoxidase antibodies with compensated thyroid hormones. After 2 weeks of treatment with local glucocorticosteroids, skin lesions disappeared, leaving post-inflammatory discoloration. The patient remains without recurrence of any skin lesions during the five-month observation. In conclusion, in the diagnosis of this rare disease, the clinicopathological correlation plays a crucial role as well as the investigation for co-existing autoimmune disorders.
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Keywords

amicrobial pustulosis of the folds, aseptic pustulosis, neutrophilic dermatosis, pustular dermatosis

About this article
Title

Amicrobial pustulosis of the folds associated with thyroperoxidase antibodies

Journal

Forum Dermatologicum

Issue

Vol 4, No 1 (2018)

Article type

Review paper

Pages

5-9

Published online

2018-03-02

Page views

488

Article views/downloads

941

Bibliographic record

Forum Dermatologicum 2018;4(1):5-9.

Keywords

amicrobial pustulosis of the folds
aseptic pustulosis
neutrophilic dermatosis
pustular dermatosis

Authors

Monika Siedlecka
Joanna Czuwara
Marlena Majkut-Sobechowicz
Katarzyna Pisarz
Roman Nitskovich
Lidia Rudnicka

References (12)
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  10. Marzano AV, Tavecchio S, Berti E, et al. Cytokine and chemokine profile in amicrobial pustulosis of the folds: evidence for Autoinflammation. Medicine (Baltimore). 2015; 94: e2301.
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  12. Amazan E, Ezzedine K, Mossalayi MD, et al. Expression of interleukin-1 alpha in amicrobial pustulosis of the skin folds with complete response to anakinra. J Am Acad Dermatol. 2014; 71(2): e53–e56.

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