Bergman r, sezin t, indelman m, helou wa, avitanhersh e. Biopsy was taken from hyperpigmented papule keeping epidermodysplasia verruciformis edv, dariers disease and acrokeratosis verruciformis. A 66yearold man presented to the clinic with a 4week history of painful, edematous, and scaly ears, a condition that was later accompanied by. Nonfamilial acrokeratosis verruciformis of hopf koreamed. Acrokeratosis verruciformis of hopf avh is a rare, benign skin condition, characterised by wartlike papules on the hands and feet. Darier oder um klinisch ungewohnliche veranderungen des m. Acrokeratosis verruciformis of hopf, nonfamilial acrokeratosis verruciformis introduction acrokeratosis verruciformis akv is a rare hyperkeratotic genodermatosis, which was first described by hopf in 19311. Ob es sich hierbei um eine acrokeratosis verruciformis hopf. Darier disease is an inherited skin condition characterized by wartlike blemishes on the body. Acrokeratosis verruciformis of hopf akv is a rare genodermatosis presenting as multiple plane wartlike lesions symmetrically distributed on dorsum of hands and feet. Acrokeratosis verruciformis of hopf abstract europe pmc. It has autosomal dominant inheritance, meaning that only one copy of the affected gene needs to be inherited to develop the disease.
Acrokeratosis verruciformis of hopf case report ncbi. Acrokeratosis verruciformis is a very rare, heritable hyperkeratotic dermatosis that was originally described by hopf in 1931. Acrokeratosis verruciformis of hopf clinically mimicking. 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. 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. List of rare diseases and synonyms orphanet slidelegend. The rarity is defined according to the european legislation defining a prevalence threshold of not more than 5 affected persons per 0 regulation ec n1412000 of the european parliament and of the council of 16. It is a genetic disorder that is usually manifested at or following the birth of the child. Acrokeratosis verruciformis of hopf along lines of blaschko. If one parent is affected, the chance of a child developing acrokeratosis verruciformis of hopf is 1 in 2 50%. It has autosomal dominant inheritance, meaning that only one copy of the affected gene needs to be inherited to develop the. Pdf on jan 1, 2016, sudhav damarla and others published acrokeratosis verruciformis. It usually presents with multiple small, flat, fleshcolored, warty papules on dorsa of.
Pdf acrokeratosis verruciformis of hopf hopf disease. They usually first appear in late childhood or early adulthood and often occur on the scalp. It is characterized by multiple, localized, symmetrical, flat. Acrokeratosis verruciformis of hopf avh is a rare genodermatosis characterized by keratotic lesions on the dorsum of the hands and feet. Jan 23, 2017 acrokeratosis paraneoplastica is a rare acral psoriasiform dermatosis associated with internal malignancy, most frequently squamous cell carcinoma scc of the upper aerodigestive tract.
Porokeratosis is a medical condition in which the process of keratinization is affected and disrupted. An unusual presentation find, read and cite all the. Acrokeratosis verruciformis of hopf is a rare genodermatosis with an autosomal dominant mode of inheritance. It is a rare genodermatosis that develops during infancy or early childhood with no sexual predilection. One form of acrokeratosis, acrokeratosis verruciformis \ v. Acrokeratosisverruciformisahnliche veranderungen beim. In addition, an acquired epidermodysplasia verruciformis like syndrome has been described in patients with impaired cellmediated immunity, mainly hivinfected subjects.
Guttate leukoderma and acrokeratosis verruciformis of hopf. Acrokeratosis verruciformis of hopf, nonfamilial acrokeratosis verruciformis introduction acrokeratosis verruciformis akv is a rare hyperkeratotic genodermatosis, which was first described. Acrokeratosis paraneoplastica is a rare acral psoriasiform dermatosis associated with internal malignancy, most frequently squamous cell carcinoma scc of the upper aerodigestive tract. It is a disorder of keratinization, characterized by multiple, flattopped, skincolored keratotic lesions resembling plane warts typically observed on the dorsum of the hands and feet. Feb 26, 2019 acrokeratosis paraneoplastica is also known as bazex syndrome and is a rare paraneoplastic syndrome, a constellation of findings, which is due to the underlying presence of a systemic malignancy. Acrokeratosis verruciformis is a rare genodermatosis with an autosomal dominant mode of inheritance. It usually presents with multiple small, flat, fleshcolored, warty papules on dorsa of the hands, feet, knees, elbows, and on forearms.
They usually first appear in late childhood or early adulthood and often occur on the scalp, forehead, upper arms, chest, back, knees, elbows, andor behind the ear. The topic acrokeratosis verruciformis you are seeking is a synonym, or alternative name, or is closely related to the medical condition acrokeratosis verruciformis of hopf. It typically presents as multiple, small, flat, wartlike papules on the dorsum of the hands and feet and. Unusual aspects of the case were late onset of the lesions and lack of a famil. We report a patient with exuberant clinical lesions and highlight the importance of clinical and histopathological examination for. Acrokeratosis medical definition merriamwebster medical.
Acrokeratosis paraneoplastica definition of acrokeratosis. It is a genetic disorder that is usually manifested at or. Pathology outlines epidermodysplasia verruciformis. Ob es sich hierbei um eine acrokeratosis verruciformis hopf bei m. Discussion hopf1 first described a localized disorder ofkeratinization for which he coined the term akv. Acrokeratosis verruciformis of hopf is an autosomal dominant genodermatosis usually presenting with multiple planar wartlike lesions, typically observed on the dorsum of the hands and feet. A familial analysis revealed 24 cases within six generations in a family, establishing.
The patient presents patches of atrophy on the skin, these having distinctive clinical and histopathological. Acrokeratosis verruciformis of hopf is an autosomal dominant genodermatosis usually presenting with multiple planar wartlike lesions, typically observed on the dorsum of the hands, feet, elbows, and. A case of 24 years old female is described with multiple hyperpigmented, hyperkeratotic papules on the left side of body since 5 years along the. Congenital or posttransplant abnormal susceptibility to clinical hpv infection, which causes only asymptomatic. Nilofar g diwan, nidhi b jivani, pragya ashok nair department of dermatology and venereology.
It is characterized by multiple, skincolored, flattopped, hyperkeratotic papules on the dorsal aspects of the hands and feet, which occasionally extend to the forearms and lower legs. Acrokeratosis verruciformis is a rare autosomal dominant disorder appearing at birth or in early. Epidermodysplasia verruciformis ev is a rare inherited genodermatosis characterized by chronic infection with human papillomavirus hpv leading to polymorphous cutaneous lesions and high risk of developing non melanoma skin cancer. Pdf acrokeratosis verruciformis of hopf case report.
Mar 25, 2018 acrokeratosis verruciformis of hopf avh is a rare, benign skin condition, characterised by wartlike papules on the hands and feet. Nov 29, 2016 darier disease is an inherited skin condition characterized by wartlike blemishes on the body. Acrokeratosis verruciformis is a disorder of keratinization characterized by multiple flattopped, skin. Wang pg, gao m, lin gs, yang s, lin d, liang yh, et al. Acrokeratosis verruciformis of hopf is due to an abnormal atp2a2 gene. A 67yearold white man had typical signs of acrokeratosis verruciformis of hopf. Differential diagnosis includes squamous cell carcinoma, acrokeratosis verruciformis see these terms, tinea versicolor, and generalized verrucosis of other origin. Apart from the typical skincolored, flat, warty papules on the dorsum of the. These papules may develop on other sites either in groups or discretely. A rare, genetic, acrokeratoderma disease characterized by multiple, symmetrical, asymptomatic. A congenital disorder characterized by warty papules of the hands, feet, knees, and elbows. Other features of darier disease may include nail abnormalities, such as red and.
Acrokeratosis verruciformis of hopf akv is a rare autosomal dominant cutaneous disorder first described by hopf in 1931. Here are links to possibly useful sources of information about acrokeratosis verruciformis. Acrokeratosis verruciformis of hopf jama dermatology jama. Acrokeratosis verruciformis of hopf showing p602l mutation in atp2a2 and overlapping histopathological features with darier disease. Acrokeratosis verruciformis of hopfomicsgroupjournal of. Acrokeratosis verruciformis of hopf avh is a rare, autosomal dominant genodermatosis that was first described by hopf in 1931. Acrokeratosis verruciformis of hopf or simply acrokeratosis verruciformis. International journal of allied medical sciences and clinical. It is a disorder of keratinization, characterized by multiple, flattopped, skincolored. Pdf a 54 yearold woman with a 3year history of rheumatoid arthritis ra consulted us because of weight loss, fever and skin eruption. Nilofar g diwan, nidhi b jivani, pragya ashok nair department of dermatology and venereology, pramukhswami medical college, karamsad, gujarat, india. Acrokeratosis verruciformis is a disorder of keratinization characterized by multiple flat. Acrokeratosis verruciformis of hopf is an autosomal dominant genodermatosis of unknown etiology, with no sexual predilection.
Acrokeratosis neoplastica may resolve if the underlying cancer is completely removed and the return of the condition may indicate relapse of underlying cancer. May 11, 2018 acrokeratosis verruciformis is a rare genodermatosis with an autosomal dominant mode of inheritance. Pdf acrokeratosis verruciformis of hopf along lines of. Acrokeratosis verruciformis of hopf clinically mimicking epidermodysplasia verruciformis. Porokeratosis treatment, pictures, symptoms and causes. Acrokeratosis verruciformis of hopf is an autosomal dominant genodermatosis usually presenting with multiple planar wartlike lesions, typically observed on the dorsum of the hands, feet, elbows, and knees. Acrokeratosis verruciformis hopf and dariers disease.
As most palmoplantar keratodermas, focal acral hyperkeratosis is generally inherited. Acrokeratosis verruciformis definition of acrokeratosis. Darier disease genetic and rare diseases information center. The disease is very rare and the pathogenesis remains unknown.
Apart from the typical skincolored, flat, warty papules on the dorsum of the hands and feet, examination may reveal thickening of palmar skin and punctate keratoses on the palms and the soles 2. Acrokeratosis verruciformis of hopf rege v l, hede r v. Acrokeratosis verruciformis medigoo health medical tests. Acrokeratosis verruciformis acrokeratosis verruciformis of hopf is an autosomal dominant trait usually manifested in early childhood as skin colored warty papules on the dorsum of the hands and feet, on. Dermis acrokeratosis verruciformis hopf information on the. Herein, we contribute a case of a 56year old woman who presented with the rarelydescribed guttate leukoderma of darier disease and acrokeratosis verruciformis of hopf. Journal of infectious diseases and salman and osman nfect. The leading primary care society for dermatology and skin surgery. As most palmoplantar keratodermas, focal acral hyperkeratosis is generally inherited by dominant autosomic transmission, even though sporadic cases may occur. The syndrome of acrokeratosis paraneoplastica typically precedes the diagnosis of malignancy. Epidermodysplasia verruciformis genetic and rare diseases. Focal acral hyperkeratosis, described for the first time by dowd et al. Every entity is defined by its clinical homogeneity, regardless of its etiology or the number of causing genes identified.
Acrokeratosis verruciformis is a disorder of keratinization characterized by multiple flattopped, skincolored keratotic lesions resembling plane warts typically observed on the dorsum of the hands and feet. Acrokeratosis verruciformis of hopf akv is a rare disorder of keratinization inherited in an autosomal dominant fashion. It is characterized by multiple, skincolored, flattopped, hyperkeratotic. Skin inflammatory nontumor epidermodysplasia verruciformis. Akv is a rare genodermatosis characterized by keratotic. Acrokeratosis verruciformis of hopf is a rare disorder and characterized by fleshcoloured, wartlike, flat papules on the dorsum of the hands and feet. Feb 01, 2010 differential diagnosis includes squamous cell carcinoma, acrokeratosis verruciformis see these terms, tinea versicolor, and generalized verrucosis of other origin. Genetic heterogeneity in acrokeratosis verruciformis of.
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