Follicular Dowling-Degos Disease with Hidradenitis Suppurativa: A Case Report and Review of the Literature

Dowling-Degos disease (DDD) is an autosomal dominant disorder with variable phenotypic expression. Classically, DDD is characterized by progressive reticulate hyperpigmentation on flexures with perioral pitted scars and comedone-like hyperkeratotic papules. Follicular DDD is a rare variant which was...

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Autores principales: Ya-Nin Nokdhes, Thanachat Rutnumnoi, Poramin Patthamalai, Charussri Leeyaphan
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Lenguaje:EN
Publicado: Karger Publishers 2021
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spelling oai:doaj.org-article:395f546c54fb4cfd96e13077fe5925682021-12-02T12:40:22ZFollicular Dowling-Degos Disease with Hidradenitis Suppurativa: A Case Report and Review of the Literature1662-656710.1159/000520541https://doaj.org/article/395f546c54fb4cfd96e13077fe5925682021-11-01T00:00:00Zhttps://www.karger.com/Article/FullText/520541https://doaj.org/toc/1662-6567Dowling-Degos disease (DDD) is an autosomal dominant disorder with variable phenotypic expression. Classically, DDD is characterized by progressive reticulate hyperpigmentation on flexures with perioral pitted scars and comedone-like hyperkeratotic papules. Follicular DDD is a rare variant which was introduced by Singh et al. [Indian J Dermatol Venereol Leprol. 2013 Nov–Dec;79(6):802–4]. Follicular DDD differs from other variants because of its notable comedone-like hyperkeratotic hyperpigmented papules and a distinct histopathology which demonstrates pigmented filiform and branching rete pegs originating at the follicular infundibulum with many epidermal horn cysts while the interfollicular epidermis is essentially normal. Hereby, we present a case of follicular DDD with hidradenitis suppurativa (HS). A 37-year-old Thai man presented with slowly progressive hyperpigmented comedone-like papules on the face, neck, axillae, upper trunk, and buttocks with perioral pitted scars. Punch biopsy from a comedonal lesion on his back was consistent with follicular DDD. He also had recurrent painful nodules and abscess on the back, groin, and buttock which matched the clinical criteria for the diagnosis of HS. To date, a paucity of concurrent DDD with HS has been reported. Recent genetic studies speculate a shared pathophysiologic mechanism of DDD and HS.Ya-Nin NokdhesThanachat RutnumnoiPoramin PatthamalaiCharussri LeeyaphanKarger Publishersarticlefollicular dowling-degos diseasehidradenitis suppurativaacneDermatologyRL1-803ENCase Reports in Dermatology, Vol 13, Iss 3, Pp 530-536 (2021)
institution DOAJ
collection DOAJ
language EN
topic follicular dowling-degos disease
hidradenitis suppurativa
acne
Dermatology
RL1-803
spellingShingle follicular dowling-degos disease
hidradenitis suppurativa
acne
Dermatology
RL1-803
Ya-Nin Nokdhes
Thanachat Rutnumnoi
Poramin Patthamalai
Charussri Leeyaphan
Follicular Dowling-Degos Disease with Hidradenitis Suppurativa: A Case Report and Review of the Literature
description Dowling-Degos disease (DDD) is an autosomal dominant disorder with variable phenotypic expression. Classically, DDD is characterized by progressive reticulate hyperpigmentation on flexures with perioral pitted scars and comedone-like hyperkeratotic papules. Follicular DDD is a rare variant which was introduced by Singh et al. [Indian J Dermatol Venereol Leprol. 2013 Nov–Dec;79(6):802–4]. Follicular DDD differs from other variants because of its notable comedone-like hyperkeratotic hyperpigmented papules and a distinct histopathology which demonstrates pigmented filiform and branching rete pegs originating at the follicular infundibulum with many epidermal horn cysts while the interfollicular epidermis is essentially normal. Hereby, we present a case of follicular DDD with hidradenitis suppurativa (HS). A 37-year-old Thai man presented with slowly progressive hyperpigmented comedone-like papules on the face, neck, axillae, upper trunk, and buttocks with perioral pitted scars. Punch biopsy from a comedonal lesion on his back was consistent with follicular DDD. He also had recurrent painful nodules and abscess on the back, groin, and buttock which matched the clinical criteria for the diagnosis of HS. To date, a paucity of concurrent DDD with HS has been reported. Recent genetic studies speculate a shared pathophysiologic mechanism of DDD and HS.
format article
author Ya-Nin Nokdhes
Thanachat Rutnumnoi
Poramin Patthamalai
Charussri Leeyaphan
author_facet Ya-Nin Nokdhes
Thanachat Rutnumnoi
Poramin Patthamalai
Charussri Leeyaphan
author_sort Ya-Nin Nokdhes
title Follicular Dowling-Degos Disease with Hidradenitis Suppurativa: A Case Report and Review of the Literature
title_short Follicular Dowling-Degos Disease with Hidradenitis Suppurativa: A Case Report and Review of the Literature
title_full Follicular Dowling-Degos Disease with Hidradenitis Suppurativa: A Case Report and Review of the Literature
title_fullStr Follicular Dowling-Degos Disease with Hidradenitis Suppurativa: A Case Report and Review of the Literature
title_full_unstemmed Follicular Dowling-Degos Disease with Hidradenitis Suppurativa: A Case Report and Review of the Literature
title_sort follicular dowling-degos disease with hidradenitis suppurativa: a case report and review of the literature
publisher Karger Publishers
publishDate 2021
url https://doaj.org/article/395f546c54fb4cfd96e13077fe592568
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AT poraminpatthamalai folliculardowlingdegosdiseasewithhidradenitissuppurativaacasereportandreviewoftheliterature
AT charussrileeyaphan folliculardowlingdegosdiseasewithhidradenitissuppurativaacasereportandreviewoftheliterature
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