Porokeratosis mibelli Q82.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

hyperkeratosis concentrica; Hyperkeratosis figurata centrifugata atrophicans; keratoatrophic nevus; Keratoatrophodermia; Keratoatrophodermia hereditaria chronica et progressiva; keratodermia excentrica; Mibelli type I; Mibell's disease; Parakeratosis centrifugata atrophicans; parakeratosis mibelli; POROK1; porokeratosis 1

History
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Mibelli, 1893

Definition
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Rare, irregularly autosomal-dominantly inherited ((POROK1; OMIN:175800), multifocal differentiation disorder of the epidermis with parakeratosis in the middle of hyperkeratosis, which belongs to the porokeratoses (naming: poros = opening; keras = horn; incorrect designation, because the cornification anomaly does not originate from the follicular epithelium or the acrosyringium).

Manifestation
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Mainly occurring during childhood and adolescence, but also in later adulthood.

Men are affected 2x (3x) as frequently as women.

Apparently, however, families with more than 1 variant of a porokeratosis type exist, so that a strict separation must be questioned (Sybert).

Localization
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Extremities (especially forearms, lower legs and back of the hands) are affected, rarely also the trunk, face or glans penis. Infection of the mucous membranes and rarely involvement of the cornea is possible.

Clinical features
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Mostly single or few (rarely multiple: differentiation from the disseminated type is difficult!), rapidly growing, brownish keratotic papules and plaques.

Initial: 0.1-0.2 cm large, mostly solitary, yellowish, yellowish or reddish-brown, asymptomatic papules with an adherent, non-detachable central horn spine. In case of permanent lateral pressure (e.g. by footwear) the horn papules can become painful. By gradual growth of area and thickness (random) confluence of the individual papules. This results in roundish, circine or garland-shaped plaques up to 0.5-10.0 cm in size (or even larger) with a verrucous or slightly atrophic surface. Accompanying symptoms: Nail deformities.

Histology
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Vented parakeratosis, cornoid lamella, atypical basal cells. Lymphohistiocytic infiltrate exists in the upper corium.

General therapy
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Semi-annual skin checks to exclude tumours.

External therapy
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Trial vitamin A acid in low concentration R256. Alternatively, urea-containing and/or salicylic acid-containing external preparations (e.g. salicylvaseline Lichtenstein, R228, R227, R108, R105 ). Therapy successes with 5% Imiquimod cream (Aldara) are described on the basis of casuistics.

Internal therapy
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Test with acitretin (neotigason) 0,5 mg/kg bw/day.

Operative therapie
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Removal by cryosurgery (open spray method), electrocoagulation,CO2 or Erbium-YAG laser. Therapy successes are not satisfactory.

Progression/forecast
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Progression. Spontaneous regression with formation of atrophic scars and later carcinoma development (possible in 17% of cases).

Note(s)
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In multiple flocks, porokeratosis superficialis disseminata actinica should be considered in the first instance.

Literature
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  1. Agarwal S, Berth-Jones J (2002) Porokeratosis of Mibelli: successful treatment with 5% imiquimod cream. Br J Dermatol 146: 338-339
  2. Ehlers G et al (1971) Porokeratosis Mibelli with multiple precanceroses and squamous cell carcinomas. Dermatologist 22: 68-73c
  3. Judge MR et al (1990) Disseminated porokeratosis in an infant with craniosynostosis. Br J Dermatol 123: 249-254
  4. Mibelli V (1893) Contributo alla studio della ipercheratosi dei canali sudoriferi (porokeratosi). G Ital Dermatol Venereol (Torino) 28: 313-355
  5. Sybert VP (2010) Genetic Skin Disorders (end ed.).Oxford University Press, S 89-92
  6. Wallner JS et al (2003) Verrucous porokeratosis of Mibelli on the buttocks mimicking psoriasis. Cutis 72: 391-393
  7. Weidner T et al (2017) Treatment of Porokeratosis: A Systematic Review. At J Clin Dermatol 8:435-449

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

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Last updated on: 29.10.2020