Epidermolytic hyperkeratosis is a form of ichthyosis normally resistant to topical treatments. Female patient monitored since 1978 diagnosed with epidermolytic hyperkeratosis. Clinical examination showed generalized hyperkeratosis and scaling. Given that no other treatments were available at the time, the patient was initially treated with keratolytic, systemic vitamin A and moisturizers, with no improvement. In 1986, with the development of oral retinoids, etretinate was introduced. In 1998 this was replaced by acitretin. The patient is receiving 25 mg/day after 23 years of using oral retinoids. Significant improvement of the condition and patient's quality of life has been noted.
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SOX-10 and Melan-A Immunostaining in Areas of Focal Acantholytic Dyskeratosis and Epidermolytic Hyperkeratosis Within Dysplastic Nevi Biopsies: An Observational Study.
Am 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.
Variants in the L12 linker domain of KRT10 are causal to atypical epidermolytic ichthyosis.
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 PDFEpidermal nevi and epidermolytic hyperkeratosis: A review of cases, highlighting indications for biopsy and genetics referral.
Pediatr Dermatol
September 2024
Department of Dermatology, University of Michigan, Ann Arbor, Michigan, USA.
Article Synopsis
- * In rare cases, linear epidermal nevi can be passed down to children as epidermolytic ichthyosis, leading to severe skin symptoms at birth and ongoing skin issues.
- * Cases of EHK transmission are more common when multiple body areas are affected by epidermal nevi, indicating a higher genetic risk, thus a genetics consultation is advised for affected individuals.
Epidermolytic epidermal nevus on the genitalia caused by a mosaic KRT10 mutation.
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Treating epidermolytic ichthyosis and ichthyosis with confetti with epidermal autografts cultured from revertant skin.
Br J Dermatol
August 2024
Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Article Synopsis
- There is currently no effective treatment for epidermolytic ichthyosis (EI), a condition caused by genetic mutations in keratin genes (KRT1 or KRT10), leading to skin abnormalities.
- Patients with ichthyosis with confetti (IWC) exhibit some areas of normal skin due to genetic changes that occur in the affected tissues.
- A clinical trial was conducted to evaluate the use of cultured epidermal autografts (CEAs) derived from revertant skin cells, which showed varying success in preventing recurrence of ichthyosis lesions after transplantation, with 100% of patients experiencing some level of improvement four weeks post-transplant.
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