porokeratosis

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
References in periodicals archive ?
In spiny keratoderma, also called punctate porokeratosis of the palms and soles, lesions protrude more and resemble "music box spines.
Porokeratosis (PK), first described in 1983, is a chronic parakeratotic skin disorder.
Porokeratosis is a specific keratinization disorder that manifests clinically as well-demarcated annular or linear keratotic plaques of various sizes and forms and with distinguished histology showing cornoid lamella, which is a column of closely packed parakeratotic cells extending through the stratum corneum.
There are seven clinical variants of which porokeratosis palmaris et plantaris disseminata (PPPD) is a very rare entity.
8 Actinic keratosis, warty dyskeratoma, solar lentigo, porokeratosis, prurigo nodularis, verruca, condyloma acuminatum, molluscum, seborrheic keratosis, lichenoid keratosis Melanoma 15 60.
A Chinese research team, led by Anhui Medical University and BGI, has found the strong genetic evidences of mevalonate kinase gene (MVK) mutations link to disseminated superficial actinic porokeratosis (DSAP).
A histological variant of disseminated superficial actinic porokeratosis.
The hyperkeratotic variant of porokeratosis Mibelli is a distinct entity: clinical and ultrastructural evidence.
Biopsy of this area showed a cornoid lamella, which is seen in porokeratosis.
The lesion you described is a callus, a wart, or a porokeratosis.