Presentation
Zurück zum Zitat Hendrix JD, Greer KE (1995) Erythrokeratodermia variabilis present at birth: case report and review of the literature. [springermedizin.de]
Article / Publication Details First-Page Preview Abstract A case of Gottron’s syndrome or symmetrical progressive erythrokeratoderma is presented. [karger.com]
Case Report A 13-year-old male that presented to our institution with the onset of symmetrically distributed hyperkeratotic plaques over the dorsum of the hands and the extensor aspect of the forearms, elbows and knees, from the age of 3, asymptomatic [cyberleninka.org]
Transitory well outlined erythematous plaques with a geographical appearance, are present in EKV and may affect any cutaneous surface.1,2,3 It is usually seen in childhood, and the erythema tends to fade with time. [ncbi.nlm.nih.gov]
Entire Body System
- Turkish
Akman A, Masse M, Mihci E, Richard G, Christiano AM, Balle BJ, et al Progressive symmetrical erythrokeratoderma: Report of a Turkish family and evaluation for loricrin and connexin gene mutations Clin Exp Dermatol. 2008;33:582–4 8. [journals.lww.com]
Progressive symmetrical erythrokeratoderma: Report of a Turkish family and evaluation for loricrin and connexin gene mutations. Clin Exp Dermatol. 2008;33:582-4. 5. [cyberleninka.org]
Progressive symmetrical erythrokeratoderma: report of a Turkish family and evaluation for loricrin and conexin gene mutations. Clin Exp Dermatol. 2008;33:582–4. [PubMed] [Google Scholar] 7. Chu DH, Arroyo MP. [ncbi.nlm.nih.gov]
- Asymptomatic
Case Report A 13-year-old male that presented to our institution with the onset of symmetrically distributed hyperkeratotic plaques over the dorsum of the hands and the extensor aspect of the forearms, elbows and knees, from the age of 3, asymptomatic [cyberleninka.org]
An asymptomatic clinical case of classic PSEK is described below with family involvement. CASE REPORT Female patient, 55 years old, phototype III, referred the onset of skin lesions from the age of 5, with progressive growth until the age of 18. [ncbi.nlm.nih.gov]
Skin
- Skin Disease
0022-202x.2001.01468.x Martin, Connexins and skin disease: insights into the role of beta connexins in skin homeostasis, Cell Tissue Res, № 360, с. 645 https://doi.org/10.1007/s00441-014-2094-3 Scott, Connexins in epidermal homeostasis and skin disease [ouci.dntb.gov.ua]
In: Barker J, McGrath J (eds) Cell adhesion and migration in skin disease, Band 8. Harwood academic publishers, pp 9–26 Frank J, Christiano, AM (2001) The cornified cell envelope. [springermedizin.de]
Skin disease Keyword (es) Eritroqueratodermia progresiva Estudio caso Hombre Disqueratosis Eritrodermia Hiperqueratosis Enfermedad hereditaria Piel patología Classification Pascal 002 Biological and medical sciences / 002B Medical sciences / 002B08 Dermatology [pascal-francis.inist.fr]
Suga Y, Jarnik M, Attar PS, Longley MA, Bundman D, Steven AC, et al Transgenic mice expressing a mutant form of loricrin reveal the molecular basis of the skin diseases, Vohwinkel syndrome and progressive symmetric erythrokeratoderma J Cell Biol. 2000 [journals.lww.com]
Transgenic mice expressing a mutant form of loricrin reveal the molecular basis of the skin diseases, Vohwinkel syndrome and progressive symmetric erythrokeratoderma. J Cell Biol. 151:401–12. 200. [PMC free article] [PubMed] [Google Scholar] 6. [ncbi.nlm.nih.gov]
- Hyperkeratosis
An orthokeratotic hyperkeratosis in a basket-weave pattern. [ncbi.nlm.nih.gov]
Figure 2: H and E, section of the skin (elder sibling) showing hyperkeratosis, acanthosis, papillomatosis with follicular plugging (×40) Figure 3: H and E, section of the skin (younger sibling) showing hyperkeratosis, acanthosis, elongated rete pegs overlying [journals.lww.com]
Language Spanish, Castilian Keyword (fr) Erythrokératodermie progressive Etude cas Homme Dyskératose Erythrodermie Hyperkératose Maladie héréditaire Peau pathologie Keyword (en) Progressive erythrokeratoderma Case study Human Dyskeratosis Erythroderma Hyperkeratosis [pascal-francis.inist.fr]
Skin biopsies were taken from the lesions near the elbows showing epithelial hyperplasia with hyperkeratosis, regular acanthosis, elongation and anastomosis of rete ridges, pigmentation of the basal layer and perivascular lymphohistiocytic infiltrate [cyberleninka.org]
- Keratosis
However, in 2000, Suga et al.[9] established LOR defect-related keratosis associated with human PSEK and Vohwinkel syndrome in transgenic mice, thereby, validating the conclusion by Ishida-Yamamoto et al.[3] in 1997. [ncbi.nlm.nih.gov]
Progressive symmetric erythro-keratosis associated with oligodontia, severe caries, disturbed hair growth and ectopic nail: A new syndrome? Dermatology. 2008;217:347–50 4. [journals.lww.com]
- Hyperpigmentation
Polarized dermoscopy showed white scaly lines over focal areas of hyperpigmentation (Fig. 3). [cyberleninka.org]
The plaques were thicker on the elbows and there was a welldefined, brownish-colored hyperpigmentation halo on the inguinal region. [ncbi.nlm.nih.gov]
- Erythema
Clinical symptoms of the priori-proband: (a) and (b): Symmetric keratinized erythema with clear boundaries on the bilateral palms, back of the hands, and wrists; (c): Symmetric keratinized erythema with clear boundaries on the bilateral back of the feet [ncbi.nlm.nih.gov]
Treatment
The chosen treatment for erythrokeratodermias is oral retinoids. The limiting factor for their use is the side effects, especially in children. [ncbi.nlm.nih.gov]
Patients need to be counseled about the chronicity of their condition and the limitation of temporary improvements with the treatment options available. REFERENCES 1. Darier J. Erythrokeratodermie verruqueuse en nappes, symetrique et progressive. [cyberleninka.org]
Zurück zum Zitat Van de Kerkhof PCM, Steijlen PM, Van Dooren-Greebe RJ et al. (1990) Acitretin in the treatment of erythrokeratodermia variabilis. [springermedizin.de]