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

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 ?
Porokeratosis, in general, is a group of diseases that transmitted by autosomal dominant inheritance.
annular erythemas, lupus erythematosus and porokeratosis is preferably taken from the active edge.
Porokeratosis was by far the most common in white Northern Europeans, at nearly 8%, and sebaceous hyperplasia was common in all groups (more than 20% to about 27%) except in the black African/Caribbean and South Asian groups.
Inflammatory linear verrucous epidermal nevus Linear epidermal nevus Linear psoriasis Linear lichen planus Linear verruca plana Linear porokeratosis Linear Darier's disease Blaschkitis
Porokeratosis (PK) is an uncommon disorder of epidermal keratinization characterized by the appearance of one or more atrophic patches surrounded by a unique ridge-like border, histologically termed a cornoid lamella (1).
Porokeratosis (PK), first described in 1983, is a chronic parakeratotic skin disorder.
Porokeratosis represents a rare, chronic, progressive skin disorder, most often seen in males, clinically characterized by atrophic patches surrounded by a hyperkeratotic ridge-like border.
Contrasting with the topography of uninvolved skin, the surface of psoriatic lesions and actinic porokeratosis demonstrates alterations in the regular geometric pattern of the shallow skin lines.
have also found MVK mutations in patients with disseminated superficial actinic porokeratosis, a severe rare chronic cutaneous disorder with high genetic heterogeneity, which causes hyperkeratosis and noncontagious dry itchy lesions worsened by sun exposure; mevalonate might have a role in regulating calcium-induced keratinocyte differentiation and protecting keratinocytes from apoptosis induced by ultraviolet radiations [27].
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. Am J Dermatopathol 1987; 9: 240-242.
The hyperkeratotic variant of porokeratosis Mibelli is a distinct entity: clinical and ultrastructural evidence.