porokeratosis

(redirected from Mibelli disease)

porokeratosis

 [por″o-ker″ah-to´sis]
a rare, chronic, progressive autosomal dominant skin disorder, seen most often in males, usually first appearing in early childhood, and characterized clinically by the presence of craterlike patches that have central atrophy with an elevated thick keratotic border and enlarge to form lesions. adj., adj porokeratot´ic.

po·ro·ker·a·to·sis

(pō'rō-ker'ă-tō'sis),
A rare dermatosis in which there is a thickening of the stratum corneum with an anular keratotic rim or cornoid lamella surrounding progressive centrifugal atrophy; cutaneous carcinoma has been reported to arise in the lesions.
Synonym(s): Mibelli disease
[G. poros, pore, + keratosis]

porokeratosis

/po·ro·ker·a·to·sis/ (por″o-ker″ah-to´sis) a hereditary dermatosis marked by a centrifugally spreading hypertrophy of the stratum corneum around the sweat pores followed by atrophy. Called also p. of Mibelli. porokeratot´ic

porokeratosis

A clonal disorder of cutaneous keratinisation characterised by atrophic patch(es) on the skin surrounded by a hyperkeratotic rim called the cornoid lamella.

Risk factors
Genetic predisposition, exposure to UV light and external beam radiation, immunosuppression (e.g., HIV), lymphoma, iatrogenic related to organ transplantation.

Prognosis
Malignancy develops in 10% of lesions, usually squamous or basal cell carcinomas.

Clinical variants of porokeratosis
• Classic porokeratosis of Mibelli.
• Disseminated superficial actinic porokeratosis (DSAP).
• Porokeratosis palmaris et plantaris disseminata (PPPD).
• Linear porokeratosis.
• Punctate porokeratosis.
• Other variants include hyperkeratosis types, a pruritic papular variant, and a verrucous variant localised to the buttocks which mimics psoriasis.

po·ro·ker·a·to·sis

(pōr'ō-ker'ă-tō'sis)
A rare dermatosis in which there is a thickening of the stratum corneum with an anular keratotic rim or cornoid lamella surrounding progressive centrifugal atrophy; cutaneous carcinoma has been reported to arise in the lesions.
Synonym(s): Mibelli disease.
[G. poros, pore, + keratosis]

Mibelli,

Vittorio, Italian dermatologist, 1860-1910.
Mibelli angiokeratomas - telangiectatic small papules of the extremities, common in adolescent girls.
Mibelli disease - Synonym(s): Mibelli syndrome
Mibelli syndrome - eruption of miliary translucent papules. Synonym(s): Mibelli disease; porokeratosis