Porokeratotic eccrine ostiumnaevus D22.9

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 15.08.2022

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Synonym(s)

PEODDN; Porokeratotic eccrine and ostial dermal duct naevus

History
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Marsden, 1979

Definition
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Rare hamartoma with eccrine differentiation (see also Hamartoma, eccrine), which clinically resembles comedone nevus, but occurs preferentially on the palms and soles, where pilosebaceous follicles are absent. It is a cornification disorder involving the intraepidermal ductus eccrinus (acrosyringium), characterized in pathology by an eccrine hamartoma and a cornoid lamella.

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Manifestation
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Male and female sexes are equally affected.

Localization
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Especially the lower extremities, palms of the hands and soles of the feet are affected. Rarely located on the trunk.

Clinical features
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Asymptomatic, yellowish or yellow-brownish, hyperkeratotic papules and plaques arranged in the Blaschko lines. Frequently interspersed are firmly adherent "comedone-like" keratoses.

Histology
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When magnified, clear epidermal intussusceptions with prominent hyperkeratosis, acanthosis and parakeratosis become apparent. In places ampoule-like enlargement of acrosyringies with focal parakeratosis. The glandular part of the eccrine apparatus is not affected.

Therapy
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If necessary, excision. Otherwise conservative care.

Case report(s)
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The 29-year-old patient noticed pruritic skin lesions on the right lateral malleolus for 8 years. Pathological examination revealed multiple small epidermal invaginations with overlying parakeratotic cornoid lamellation, loss of granular layer, and few dyskeratotic cells at the base of the epidermal invagination. After clinicopathologic correlation, a diagnosis of porokeratotic eccrine ostial and dermal duct nevus (PEODDN) was made (see Fig.).

Literature
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Chowdhary B et al (2019) Porokeratotic Eccrine Ostial and Dermal Duct Nevus and Porokeratotic Eccrine and Hair Follicle Nevus: Is Nomenclature "Porokeratotic Adnexal Ostial Nevus" More Appropriate? Indian Dermatol Online J 10:478-480.
Levinsohn JL et al.(2014) A Somatic p.G45E GJB2 Mutation Causing Porokeratotic Eccrine Ostial and Dermal Duct Nevus. JAMA Dermatol 151:638-641.
Marsden RA et al (1979) Comedo naevus of the palm - a sweat duct naevus? Br J Dermatol 101: 717-722.
Murphy LA et al (2004) Porokeratotic eccrine and ostial dermal duct naevus. Clin Exp Dermatol 29: 333-334
Naraghi MM et al (2013) Porokeratotic eccrine ostial and dermal duct nevus. Case Rep Dermatol Med:953840.
Vasudevan B et al.(2015) A unique association of unilateral idiopathic calcinosis cutis with ipsilateral porokeratotic eccrine ostial and dermal duct nevus. Pediatr Dermatol 32:e8-12.