Author: Prof. Dr. med. Peter Altmeyer
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Last updated on: 22.01.2022
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Synonym(s)
comedo-follicular nevus; comedo nevus; Comedon nevus; keratotic nevus; Naevus comedonicus syndrome; nevus comedonicus; Nevus comedonicus syndrome
Definition
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Rare, epidermal nevus of the hair follicles, characterized by grouped, mostly linearly arranged "comedone-like", symptomless follicular horny cysts.
Occurrence/Epidemiology
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Incidence: approx. 2-3/100.000 inhabitants/year.
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Etiopathogenesis
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Unknown; familial clustering of comedones nevi has been described in some families(Naevus comedonicus syndrome). In some cases defects of a thyrosine kinase receptor have been detected.
Manifestation
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Mostly during childhood (1-10 years), but also from birth or late manifested in adulthood.
Localization
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Face, also capillitium, on the trunk often segmental, on the extremities linear, also occurring on hairless body regions.
Clinical features
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Organoid epidermal nevus manifested in linear, band-shaped or completely bizarre arrangement (Blaschko line pattern).
Usually unilateral, bizarrely limited, skin-coloured or even hyperpigmented lesion consisting of grouped, sharply defined, flat, often splatter-like, skin-coloured depressions, skin-coloured papules and nodules (epidermal cysts) and injected 0.2-0.3 cm dilated follicular openings (comedone-like) containing a black horn plug. In pronounced cases, a sieve-like pattern can be created by a glim-like formation of comedones.
This results in a knitting pattern like overall picture (see illustration).
Histology
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Follicular keratosis with dilatation of the infundibulum filled with laminated corneocytes, no sebaceous gland involvement, no signs of inflammation.
Differential diagnosis
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Acne vulgaris, Ulerythema ophryogenes.
Therapy
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Expression of disturbing comedones; if necessary excision of the entire area.
Note(s)
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In a few cases, comedonal nevus is a partial symptom of an epidermal nevus syndrome (see nevus , epidermal) with ispilateral cataract and bone defects as well as neurological defects (comedonal nevus syndrome as a neuro-cutaneous disease).
A variant of comedonal nevus is "epidermolytic comedonal nevus" with acantholytic areas in the surface epithelium.
Literature
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- Adya KA et al (2020) Epidermolytic nevus: An instance of mosaic epidermolytic hyosis. Indian Dermatol Online J 11:272-273.
- Alpsoy E et al (2005) Nevus comedonicus syndrome: a case associated with multiple basal cell carcinomas and a rudimentary toe. Int J Dermatol 44: 499-501
- Bongiorno MR et al (2003) Nevus comedonicus immunohistochemical features in two cases. Acta Derm Venereol 83: 300-301
- Ferrari B et al (2015) Nevus comedonicus: a case series. Pediatr Dermatol 32:216-219
- Ito T et al (2013) Bilateral nevus comedonicus syndrome. Yonago Acta Med 56:59-61
- Kaliyadan F et al (2014) Nevus comedonicus of the scalp. Skinmed 12:59-60
- Kargi E et al (2003) Nevus comedonicus. Plast Reconstr Surg 112: 1183-1185.
- Kofmann S (1885) A case of rare localization and distribution of comedones. Arch Dermatol Syphil (Berlin) 32: 177-178.
- Lefkowitz A et al (1999) Nevus comedonicus. Dermatology 199: 204-207
- Nilles M et al (1992) Nevus follicularis keratosus: clinic, histology and histogenesis. Dermatologist 43: 205-209
- Tchernev G et al (2013) Nevuscomedonicus: an updated review. Dermatol Ther (Heidelb) 3:33-40.
- Yadav P et al (2015) Nevus comedonicus syndrome. Indian JDermatol 60: 421
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Incoming links (9)
Atrophodermia vermiculata; Comedo-follicular nevus; Comedon nevus; Corneal clouding; Epidermal nevus organoid; Keratotic nevus; Komedo; Naevus comedonicus syndrome; Trichilemmocystic nevus;
Outgoing links (3)
Acne (overview); Epidermal nevus (overview); Ulerythema ophryogenes;
Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Authors
Last updated on: 22.01.2022
Author: Prof. Dr. med. Peter Altmeyer
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Articlecontent
- Definition
- Occurrence/Epidemiology
- Etiopathogenesis
- Manifestation
- Localization
- Clinical features
- Histology
- Differential diagnosis
- Therapy
- Note(s)
- Literature
- References
- Authors
