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Porokeratosis of Mibelli

definition

Uncommon chronic progressive disorder of epidermal differentiation in combination with parakeratosis. Porokeratosis of Mibelli is transmitted as an irregular autosomal dominant trait but may occur spontaneously. The lesions develop as annular, dry plaques surrounded by an elevate keratotic margin and sometimes also a furrow. The centre is often atrophic but may be hyperkeratotic. Lesions appear most often on the limbs and show a tendency to centrifugal spread. The face, genitalia, oral mucosa and cornea may also be affected. There may be gradual progression and spontaneous regression leaving mildly atrophic scars. There is a risk of malignant transformation, especially after the first five decades, sqamous cell carcinoma being the most commonly associated tumour. Bowen’s disease and basal cell carcinoma have also been reported.

synonyms

Porokeratosis of Mibelli, Parakeratosis Mibelli, Porokeratosis Excentrica

UMLS

Mibelli Porokeratosis, Porokeratoses, Porokeratosis, Porokeratosis of Mibelli, Porokeratosis, NOS

images

3 images found for this diagnose diagnosis: Porokeratosis of Mibelli diagnosis: Porokeratosis of Mibelli diagnosis: Porokeratosis of Mibelli