Elastosis perforans serpiginosa related to vascular Ehlers-Danlos syndrome

Pallesen, Kristine Appel Uldall; Lindahl, Kim Hein; Bygum, Anette

doi:10.5070/d3253043337

Cited by 3 publications

(2 citation statements)

References 5 publications

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“…Based on the clinical and histological results, EPS was diagnosed. Histopathological analysis of EPS revealed localized or perforated epidermal hyperplasia, eosinophilic deteriorated elastic fibers, and a thin vertical transepidermal channel that included nuclear debris, granular basophilic debris, scant mononuclear inflammatory infiltrate, and bumble brush appearance of elastic fibers [3,[6][7][8][9][10][12][13][14][15]. Histological evaluation of EPS specimens is usually performed with Verhoeff-Van Gieson staining, Gomori methenamine silver staining, and periodic acid-Schiff with diastase [1].…”

Section: Discussionmentioning

confidence: 99%

A Systematic Review of Case Reports of a Rare Dermatological Condition: Elastosis Perforans Serpiginosa

Besekar¹,

Jogdand²,

Naqvi³

2023

Cureus

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A systematic review was carried out on a rare dermatological condition affecting papillary dermal tissue fibers of the skin known as elastosis perforans serpiginosa (EPS). The aim of this review was to highlight this skin disease, its association with other medical conditions, and its management. The search was conducted by using the keywords "elastosis perforans serpiginosa" and "case reports" in the databases. A total of 10 case reports were analyzed and presented by the parameters like age, gender, chief complaints, and medical history. The most common causes of EPS were drug-induced and occurred along with the Wilson disease. The study concluded that as EPS is an uncommon disease with few instances, there is a need for further research to analyze randomized controlled trials that have been conducted in relation to the condition.

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Section: Discussionmentioning

confidence: 99%

A Systematic Review of Case Reports of a Rare Dermatological Condition: Elastosis Perforans Serpiginosa

Besekar¹,

Jogdand²,

Naqvi³

2023

Cureus

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“…Elastosis perforans serpiginosa is characterized by the transepidermal elimination of abnormal elastic fibres through the skin. 20 Typically, erythematous keratotic papules develop in serpiginous or arcuate configurations leaving slightly atrophic centres (Figure 2a ). Whilst not specific to EDS it is seen more frequently in vEDS and other connective tissue disorders.…”

Section: Vascular Ehlers Danlos Syndromementioning

confidence: 99%

The role of cutaneous manifestations in the diagnosis of the Ehlers‐Danlos syndromes

Stembridge

Doolan

Lavallee

et al. 2022

Skin Health and Disease

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The Ehlers-Danlos syndromes (EDS) comprise a group of inherited connective tissue disorders presenting with features of skin hyperextensibility, joint hypermobility, abnormal scarring and fragility of skin, blood vessels and some organs. The disease is generally diagnosed through the cluster of clinical features, though the addition of genetic analysis is the gold standard for diagnosis of most subtypes. All subtypes display skin manifestations, which are essential to the accurate clinical diagnosis of the condition. Furthermore, cutaneous features can be the first and/or only presenting feature in some cases of EDS and thus understanding these signs is vital for diagnosis. This review focuses on particular cutaneous features of each EDS subtype and their clinical importance. Provision of a specific diagnosis is important for management, prognosis and genetic counselling, often for family members beyond the individual.Natasha Stembridge and Brent J. Doolan are Co-first author (Equal).

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