Porokeratotic eccrine ostiumnaevus D22.9

Marsden, 1979

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.

Male and female sexes are equally affected.

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

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

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.

If necessary, excision. Otherwise conservative care.

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.).

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