Disease directory
Porokeratosis
Published: September 18, 2026Updated: September 18, 2026
Important: Informational content intended for healthcare professionals. It does not replace clinical evaluation or establish a doctor-patient relationship.
Overview
- Definition and alternative names
- A heterogeneous group of keratinization disorders clinically characterized by annular plaques/papules with a hyperkeratotic border and central atrophy , and histologically by the cornoid lamella . Vargas-Mora 2020 , Kostopoulos-Kanitakis 2025
- Main clinical variants: Mibelli porokeratosis , disseminated superficial actinic porokeratosis (DSAP) , linear porokeratosis , punctate porokeratosis , disseminated palmar-plantar porokeratosis (PPPD) , and ptychotropic porokeratosis (genitogluteal/intergluteal). Pietkiewicz 2023 , Vargas-Mora 2020
- First description
- Mibelli porokeratosis was classically described in the late 19th century; modern revisions attribute the original description to Vittorio Mibelli (1893) . Vargas-Mora 2020
- Epidemiology (practical points)
- It is rare , but may be underdiagnosed; a Swedish national study estimated a prevalence of ~1/4132 (records 2001–2020). Inci 2023
- In clinical cohorts, PSDA/DSAP is usually the most common variant; hospital series have reported a predominance of DSAP and a typical age of consultation in middle adulthood. Leow 1996 , Inci 2023
- Factors that favor its appearance/expression: UV radiation , immunosuppression (e.g., transplantation/therapies), and certain drugs . Kostopoulos-Kanitakis 2025 , Lu 2021
- ICD-10 Codes (practical note)
- Porokeratosis (unspecified) → Q82.8 (Other specified congenital malformations of skin).
- Mibelli’s porokeratosis → Q82.8 (includes “Mibelli’s disease (porokeratosis)” in the index).
- Disseminated superficial actinic porokeratosis (PSDA/DSAP) → L56.5 .
- Disseminated superficial porokeratosis (DSP, non-actinic) → Q82.8 (no separate specific code in ICD-10-CM).
- Linear porokeratosis → Q82.8 (no separate specific code).
- Porokeratosis punctata → Q82.8 (usual when documented as porokeratosis) .
- Alternative if coded as palmoplantar punctate keratosis (when the condition is strictly palmoplantar and that diagnostic label is used): L85.2 .
- Porokeratosis palmaris, plantaris et disseminata (PPPD) → Q82.8 (no separate specific code).
- Porokeratosis ptychotropica → Q82.8 (no separate specific code).
- Diagnostic checklist (quick)
- Clinical presentation: annular morphology + raised keratotic border + distribution (sun-exposed vs linear vs palmoplantar vs genitogluteal). Vargas-Mora 2020
- Directed history: UV, immunosuppression/transplantation, drug timeline. Lu 2021 , Kostopoulos-Kanitakis 2025
- Biopsy: punch/incisional of the margin (ideally 4–6 mm including ridge). Vargas-Mora 2020
- Cancer surveillance: documenting suspicious lesions and follow-up plan. INCI 2023
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