Familial dyskeratotic comedones L85.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 25.02.2022

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Synonym(s)

diffuse familial comedones; familial dyskeratotic comedones

History
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Rodin et al. 1967

Definition
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Autosomal dominant, generalized cornification anomaly characterized by disseminated, innumerable, approximately 0.2-0.3 cm, black (follicular but also non-follicular) comedone-like papules with central hyperkeratosis. The entire integument is affected, except for the mucous membranes, palms, and capillitium. The keratoses can be expressed by lateral pressure without bleeding.

Occurrence/Epidemiology
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First appearance usually during puberty, sometimes together with acne vulgaris.

Etiopathogenesis
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In the familial form, mutations in PEN-2 are detectable.

Localization
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Proximal extremities, trunk, face.

Histology
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Cyst-like follicular and interfollicular depressions of the surface epithelium with ortho- and parakeratotic keratinization. The cyst epithelium may be thinned in sections; dyskeratosis with acantholysis in places. Electron microscopy demonstrates similarities to Hailey-Hailey disease.

Therapy
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Generally caring with Hanrstoff-containing Externa. Experiment with systemic retinoids.

Literature
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  1. Carneiro SJ et al (1972) Familial dyskeratotic comedones. Arch Dermatol 105:249-251.
  2. Cheng MJ et al. (2014) Familial disseminated comedones without dyskeratosis: report of an affected family and review of the literature. Dermatology 228:303-306.
  3. Maddala RR et al (2016) Familial dyskeratotic comedones: A rare entity. Indian Dermatol Online J 7:46-48.
  4. Prabha N et al (2018) Familial dyskeratotic comedones. Skinmed 16:273-274.
  5. Rodin HH et al (1967) Diffuse familial comedones.Arch Dermatol 95: 145-146.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 25.02.2022