A 5-year-old boy presented with widespread asymptomatic hyperpigmented verrucous plaques since 3 months of age. The lesions were distributed in a linear manner along Blaschko's lines on trunk and extremities and were accentuated in flexures and around joints. There was no history of blistering or redness and no other family member was affected. Ichthyosis hystrix (of Curth and Macklin) and generalized linear/mosaic epidermolytic hyperkeratosis (EHK) were considered in the differential diagnosis. Biopsy from both trunk lesion and lesion on knee revealed characteristic epidermolytic hyperkeratosis, thereby clinching the diagnosis of systematized linear EHK.
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J Clin Med
December 2024
Department of Oncologic Dermatology-Elias Emergency University Hospital, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania.
: Vulvar epidermolytic hyperkeratosis (EHK) is an exceedingly rare dermatological condition, often presenting as solitary or multiple lesions in the vulvar region. Due to its clinical resemblance to other vulvar disorders, such as condyloma acuminatum, Bowenoid papulosis, and squamous cell carcinoma, vulvar EHK poses significant diagnostic challenges. While individual case reports and small case series have documented instances of vulvar EHK, comprehensive studies systematically consolidating the clinical, histopathological, and therapeutic aspects of this condition remain lacking.
View Article and Find Full Text PDFAm J Dermatopathol
January 2025
Department of Dermatology, University Hospitals Cleveland Medical Center, Cleveland, OH; and.
Background: Focal acantholytic dyskeratosis (FAD) and epidermolytic hyperkeratosis (EHK) are common incidental epidermal histologic findings within dysplastic nevi biopsies. We evaluate whether areas of FAD and EHK within dysplastic nevi biopsies stain with immunostains used to characterize melanocytic neoplasms.
Methods: In this case series, a natural language search of histopathology reports from our institution in the past year (2020-2021) identified dysplastic nevus biopsies with concurrent FAD and/or EHK.
J Dermatol
September 2024
Department of Dermatology, UMCG Center of Expertise for Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Epidermolytic ichthyosis (EI) is a type of congenital ichthyosis, characterized by erythema and blistering at birth followed by hyperkeratosis. EI is caused by pathogenic variants in the genes KRT1 and KRT10, encoding the proteins keratin 1 (KRT1) and keratin 10 (KRT10), respectively, and is primarily transmitted by autosomal-dominant inheritance, although recessive inheritance caused by nonsense variants in KRT10 is also described. The keratins form a network of intermediate filaments and are a structural component of the cytoskeleton, giving strength and resilience to the skin.
View Article and Find Full Text PDFPediatr Dermatol
September 2024
Department of Dermatology, University of Michigan, Ann Arbor, Michigan, USA.
Int J Dermatol
November 2024
Research Laboratory, KK Women's and Children's Hospital, Singapore.
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