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Dermatology Online Journal Apr 2020Porokeratosis is a heterogeneous group of dermatoses with alterations of keratinization. Histologically, they are characterized by the presence of cornoid lamellae.... (Review)
Review
Porokeratosis is a heterogeneous group of dermatoses with alterations of keratinization. Histologically, they are characterized by the presence of cornoid lamellae. Eruptive pruritic papular porokeratosis (EPPP) or the inflammatory form of disseminated superficial porokeratosis (or eruptive disseminated porokeratosis) is an infrequent variant, characterized by pruritic erythematous papules or annular lesions. We present a 72-year-old woman with EPPP, exhibited by pruritic lesions on the extremities and back, and review the literature concerning this condition. We found 32 cases of EPPP or inflammatory disseminated superficial porokeratosis (including the current case) reported in the literature, with a median age of 66 years (range, 13-84); 59.3% were men. Eruptive pruritic papular porokeratosis was associated with various neoplasms in 31.2% of cases. Six patients had an associated viral infection. Response to treatment was poor in most cases. Eruptive pruritic papular porokeratosis resolved spontaneously in 75% of cases. Median time to resolution was 6 months (range, 1-24). Eruptive pruritic papular porokeratosis (or inflammatory disseminated superficial porokeratosis/eruptive disseminated porokeratosis) is an infrequent variant of porokeratosis characterized by intense pruritus and spontaneous resolution in most individuals. Eruptive pruritic papular porokeratosis can be associated with neoplasms and screening for malignancies is recommended if clinically indicated.
Topics: Aged; Female; Humans; Male; Middle Aged; Porokeratosis; Pruritus; Remission, Spontaneous; Skin
PubMed: 32621680
DOI: No ID Found -
Indian Journal of Dermatology,... 2016
Topics: Adult; Facial Dermatoses; Female; Humans; Male; Porokeratosis; Sunlight; Young Adult
PubMed: 27088949
DOI: 10.4103/0378-6323.174391 -
Metabolites Nov 2023Porokeratosis is a heterogeneous group of keratinising disorders characterised by the presence of particular microscopic structural changes, namely the presence of the... (Review)
Review
Porokeratosis is a heterogeneous group of keratinising disorders characterised by the presence of particular microscopic structural changes, namely the presence of the cornoid lamella. This structure develops as a consequence of a defective isoprenoid pathway, critical for cholesterol synthesis. Commonly recognised variants include disseminated superficial actinic porokeratosis, disseminated superficial porokeratosis, porokeratosis of Mibelli, palmoplantar porokeratosis (including porokeratosis palmaris et plantaris disseminata and punctate porokeratosis), linear porokeratosis, verrucous porokeratosis (also known as genitogluteal porokeratosis), follicular porokeratosis and porokeratoma. Apart from the clinical presentation and epidemiology of each variant listed, this review aims at providing up-to-date information on the precise genetic background, introduces imaging methods facilitating the diagnosis (conventional and ultraviolet-induced fluorescence dermatoscopy, reflectance confocal microscopy and pathology), discusses their oncogenic potential and reviews the literature data on the efficacy of the treatment used, including the drugs directly targeting the isoprenoid-mevalonate pathway.
PubMed: 38132857
DOI: 10.3390/metabo13121176 -
Case Reports in Dermatology 2023Porokeratosis is a group of well-known clinically distinct entities, characterised by different clinical aspects, but sharing a single common histological aspect, namely...
Porokeratosis is a group of well-known clinically distinct entities, characterised by different clinical aspects, but sharing a single common histological aspect, namely the cornoid lamella. Usually, porokeratosis occurs in the limbs and trunk, while it rarely involves the face, especially as an exclusive, single, and solitary lesion. We report the case of a 52-year-old Caucasian woman, with an 11-month history of a 2-cm slowly growing solitary, keratotic lesion on her left cheekbone. The patient did not present other cutaneous lesions on the face, as well as in other body sites. A cutaneous biopsy showed epidermal hyperplasia with multiple, sharply defined cornoid lamella, associated with an underlying attenuation of the granular layer and scattered dyskeratotic cells in the spinous layer. The superficial dermis underneath showed a mild lymphocytic infiltrate and fibrosis with remodelled collagen bundles. A final diagnosis of solitary facial porokeratosis was made.
PubMed: 37899946
DOI: 10.1159/000530936 -
Dermatology Online Journal Aug 2009A 22-year-old man presented with a two-year history of warts on the penis, scrotum, and thighs. Physical examination showed multiple annular plaques with thin,...
A 22-year-old man presented with a two-year history of warts on the penis, scrotum, and thighs. Physical examination showed multiple annular plaques with thin, threadlike borders on the penis and scrotum. The biopsy specimen showed a cornoid lamella with underlying dyskeratotic cells that was consistent with porokeratosis. Genital porokeratosis is a rare condition that may be misdiagnosed as a sexually transmitted disease.
Topics: Genital Diseases, Male; Humans; Male; Penile Diseases; Porokeratosis; Scrotum; Young Adult
PubMed: 19891931
DOI: No ID Found -
Dermatology Online Journal Mar 2018Genitogluteal porokeratosis is a disorder of keratinization that may present in men in their fourth decade of life. We describe a 52-year-old human immunodeficiency... (Review)
Review
Genitogluteal porokeratosis is a disorder of keratinization that may present in men in their fourth decade of life. We describe a 52-year-old human immunodeficiency virus (HIV)-positive man with history of anal squamous cell carcinoma who developed verrucous lesions on the buttocks and genitals. The buttock lesions presented shortly after radiotherapy for anal carcinoma a decade prior, whereas the genital lesions presented three months prior in areas treated with injectable medication for erectile dysfunction. Skin biopsy revealed a cornoid lamella, leading to the diagnosis of genitogluteal porokeratosis. The buttock lesions were treated with shave excision and the genital lesions were treated with topical agents. Using the PubMed database, a literature search was performed with combinations of the following key words: acuminata, condyloma, cornoid lamella, genital, genitogluteal, HIV, penile, porokeratosis, verrucous, vulvar. The generated papers and their references were reviewed. To the best of our knowledge, we present the first reported case of genitogluteal porokeratosis in an HIV-positive man. Notably, these lesions developed in sites of prior radiation or injection. This condition should be included in the differential diagnosis of chronic lesions of the genitals and buttocks in patients with HIV and/or history of radiation treatment and/or trauma to the genitogluteal region.
Topics: Biopsy; Buttocks; Diagnosis, Differential; HIV Seropositivity; Humans; Male; Middle Aged; Penis; Porokeratosis; Scrotum; Skin
PubMed: 29634882
DOI: No ID Found -
Journal of Clinical Medicine Apr 2021Mevalonate kinase-associated diseases (MKAD) are caused by pathogenic mutations in the mevalonate kinase gene () and encompass several phenotypically different rare and...
Mevalonate kinase-associated diseases (MKAD) are caused by pathogenic mutations in the mevalonate kinase gene () and encompass several phenotypically different rare and hereditary autoinflammatory conditions. The most serious is a recessive systemic metabolic disease called mevalonic aciduria, and the most recently recognized is disseminated superficial actinic porokeratosis, a dominant disease limited to the skin. To evaluate a possible correlation between genotypes and (1) the different MKAD clinical subtypes or (2) the occurrence of severe manifestations, data were reviewed for all patients with variants described in the literature (N = 346), as well as those referred to our center (N = 51). The genotypes including p.(Val377Ile) (homozygous or compound heterozygous) were more frequent in mild systemic forms but were also sometimes encountered with severe disease. We confirmed that amyloidosis was more prevalent in patients compound heterozygous for p.(Ile268Thr) and p.(Val377Ile) than in others and revealed new associations. Patients homozygous for p.(Leu264Phe), p.(Ala334Thr) or compound heterozygous for p.(His20Pro) and p.(Ala334Thr) had increased risk of severe neurological or ocular symptoms. All patients homozygous for p.(Leu264Phe) had a cataract. The variants associated with porokeratosis were relatively specific and more frequently caused a frameshift than in patients with other clinical forms (26% vs. 6%). We provide practical recommendations focusing on phenotype-genotype correlation in MKAD that could be helpful for prophylactic management.
PubMed: 33917151
DOI: 10.3390/jcm10081552 -
Indian Dermatology Online Journal 2023
PubMed: 37521240
DOI: 10.4103/idoj.idoj_408_22 -
Indian Journal of Dermatology 2022We report a case of porokeratosis ptychotropica with a rare manifestation. Dermoscopy showed dotted vessels, cerebriform pattern, white scales, and brown and greyish...
We report a case of porokeratosis ptychotropica with a rare manifestation. Dermoscopy showed dotted vessels, cerebriform pattern, white scales, and brown and greyish white tracks in the periphery over a red-brown background. A skin biopsy confirmed the diagnosis from the presence of cornoid lamellae.
PubMed: 36998821
DOI: 10.4103/ijd.ijd_1145_20 -
JAAD Case Reports Nov 2022
PubMed: 36186413
DOI: 10.1016/j.jdcr.2022.08.044