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Acrokeratosis verruciformis

Field	Value
name	Acrokeratosis verruciformis
synonyms	Acrokeratosis verruciformis of Hopf
image	Autosomal dominant - en.svg
caption	Acrokeratosis verruciformis has an autosomal dominant pattern of inheritance

Acrokeratosis verruciformis is a rare autosomal dominant disorder appearing at birth or in early childhood, characterized by skin lesions that are small, verrucous, flat papules resembling warts along with palmoplantar punctate keratoses and pits. However sporadic forms, whose less than 10 cases have been reported, presents at a later age, usually after the first decade and generally lack palmoplantar keratoses. Whether acrokeratosis verruciformis and Darier disease are related or distinct entities has been controversial, like Darier's disease, it is associated with defects in the ATP2A2 gene. however the specific mutations found in the ATP2A2 gene in acrokeratosis verruciformis have never been found in Darier's disease.

Signs and symptoms

Clinical signs of acrokeratosis include verrucous plaques and flat-topped, polygonal papules. The lesions range in hue from brown to skin tone, and their friction might cause vesicles. The backs of the hands and feet's proximal and distal interphalangeal joints are typically where the lesions are seen. Lesions are less common and only occasionally seen on other body parts. The frontal scalp, flexures, and oral mucosa are not affected by the condition. On the palms and soles, punctate keratosis is another symptom of the illness. Changes to the nails, such as thickening of the nail plate, leukonychia, longitudinal ridges, and nicks in the free edges, are often noted.

Causes

Acrokeratosis verruciformis is caused by mutations in the ATP2A2 gene and is inherited in an autosomal dominant fashion.

Diagnosis

The diagnosis is established by histological characteristics such as acanthosis, hyperkeratosis, hypergranulosis without parakeratosis, and papillomatosis, which are limited epidermal elevations referred to as "church spires."

Treatment

Superficial ablation is currently the only effective treatment available.

References

(2007). "Dermatology: 2-Volume Set". Mosby.
(2003). "Fitzpatrick's Dermatology in General Medicine". McGraw-Hill, Medical Pub. Division.
(2006). "Andrews' Diseases of the Skin : Clinical Dermatology.". Saunders Elsevier.
(February 2003). "Acrokeratosis verruciformis of Hopf is caused by mutation in ATP2A2: evidence that it is allelic to Darier's disease". The Journal of Investigative Dermatology.
(April 2016). "Whole-exome sequencing solves diagnostic dilemma in a rare case of sporadic acrokeratosis verruciformis". Journal of the European Academy of Dermatology and Venereology.
(2016). "Acrokeratosis verruciformis of Hopf — Case report". FapUNIFESP (SciELO).
(2007-01-01). "Acitretin treatment in acrokeratosis verruciformis of Hopf". Informa UK Limited.
(2011). "Non-familial Acrokeratosis Verruciformis of Hopf". Korean Dermatological Association and The Korean Society for Investigative Dermatology.
Nair, PragyaA. (2013). "Acrokeratosis verruciformis of hopf along lines of blaschko". Medknow.
(1998). "Acroceratose verruciforme de Hopf: relato de caso". An. Bras. Dermatol.
(2006). "Genetic heterogeneity in acrokeratosis verruciformis of Hopf". Oxford University Press (OUP).

Info: Wikipedia Source

This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

genodermatoses

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