Porokeratosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Porokeratosis is a specific disorder of keratinization that is characterized histologically by the presence of a cornoid lamella, a thin column of closely stacked, parakeratotic cells extending through the stratum corneum with a thin or absent granular layer.[1]
Historical Perspective
Porokeratosis, a disorder of epidermal keratinization was first described by Mibelli in 1893. Since then it has been classified into 6 types.
Classification
Porokeratosis may be divided into the following clinical types:
However, additional types have also been described:[2]
Pathophysiology
Porokeratosis has a characteristic histomorphologic feature known as a cornoid lamella.
Causes
The aetiology of porokeratosis is still unclear. An autosomal dominant inheritance has been established for classic porokeratosis Mibelli, disseminated superficial actinic porokeratosis, disseminated superficial porokeratosis, porokeratosis palmaris et plantaris disseminata.
Risk Factors
- Sun exposure
- Virus infection
- Immunosuppression
- Radioactivity
Epidemiology and Demographics
It frequently appears in childhood but may appear at any age, especially in nonhereditary cases, with a higher incidence in males
Diagnosis
History and Symptoms
Physical Findings
Classic porokeratosis Mibelli consists of a single plaque or a small number of plaques of variable size, usually located unilaterally on limbs. . DSP is a variant of PM characterized by small erythematous or pigmented keratotic papules with central atrophy, located on the trunk, genitals, palms, and soles