Etretinate improves localized porokeratosis of mibelli. Porokeratosis is a dermatosis which results from a specific alteration of keratinization 1. Our case presented with a single lesion over the nose. Classic porokeratosis of mibelli or pm is the second most common subtype of porokeratosis, an uncommon skin condition that results in the manifestation of small patches of lesions of varying sizes on skin. The most distinctive feature is the border surrounding the patch, this being obviously hyperkeratotic. The lesions develop as annular, dry plaques surrounded by an elevate keratotic margin and sometimes also a furrow. Treatment depends on the size, location, and aggressiveness of porokeratosis in each affected individual.
Disseminated superficial porokeratosis and disseminated superficial actinic porokeratosis. A rare case of multiple variants of porokeratosis in the. Familial disseminated plaque type porokeratosis with multiple. Porokeratosis of mibelli the classic porokeratosis of mibelli presents in infancy to early adulthood as an asymptomatic to slightly pruritic, brown to skincolored, annular plaque with a peripheral hyperkeratotic, ridgelike scale.
Case study by acta dermatovenerologica alpina, pannonica et adriatica. Porokeratosis differential diagnoses medscape reference. Different morphological variants of hypertrophic porokeratosis and disseminated lesions of porokeratosis of mibelli. A case of porokeratosis of mibelli in a chinese woman effectively managed by 5% topical imiquimod cream kam tim michael chan1, ching yi hui2 and matthew ming wai ma3 1department of dermatology, hong kong academy of medicine, hong kong 2dr michael k. Pm initially appears as a small, asymptomatic, or slightly pruritic lesion develops that slowly expands over a. Lesions can range in size from 1 to 2 mm up to several centimeters in diameter. What factors may later activate the area to develop clinical lesions of linear porokeratosis are not known. The term porokeratosis refers to skin lesions with a thinned centre surrounded by a ridgelike border called the cornoid lamella. James, md philadelphia, pennsylvania the localized type of porokeratosis of mibelli is most commonly found on the distal extremities. The patient presents patches of atrophy on the skin, these having distinctive clinical and histopathological features. Punctate porokeratosis which is usually associated with the zosteriform or mibelli variant.
A 60yearold woman wilh plaquetype psoriasis of 30 years duration was treated with etretinate. Hence porokeratosis of mibelli should be a differential diagnosis for any such plaques over nose. Aug 01, 2011 rare disease, consists of an atrophic center bordered by a peripheral grooved keratotic ridge. Porokeratosis can be present at birth or not develop until adult life 2, depending on the type of. Porokeratosis is a disorder of keratinization showing a welldefined lesion with a hyperkeratotic ridge on the border that contains the coronoid lamella. Porokeratosis of mibelli pm is a lesion characterized by a surrounding cornoid lamella with variable nonspecific findings eg, atrophy, acanthosis, verrucous hyperplasia in the center of the lesion that typically presents in infancy to early childhood. There are a number of different types of porokeratoses, those best defined are described below. Porokeratosis of mibelli can give rise skin cancer. Porokeratosis is a group of skin conditions in which there is abnormal keratinisation. Congenital erosive and vesicular dermatosis healing with reticulated supple scarring is a rare disease first reported by cohen et al in 1985. The common genetic porokeratosis types are very rare in this country, but every now and then we see a localized porokeratosis only in one or two sites of the body like the case described in the scrotum 5. Porokeratosis is a skin disorder that generally manifests in grownups. Two male patients with a localized type of porokeratosis limited to the genitalia are reported.
Porokeratosis of mibelli induced by topical corticosteroid. Porokeratosis of mibelli is characterized by keratotic plaques, sharply delineated from adjacent normal skin by a raised, horny ridge composed of a keratotic column of cells with a characteristic histology, the cornoid lamella. Linear porokeratosis of mibelli jama dermatology jama network. Linear porokeratosis of mibelli would seem to be best explained on the basis of an abnormal clone of cells carrying the porokeratosis trait distributed along a dermatome area early in embryogenesis. Disseminated superficial actinic porokeratosis case report 2. They arise from clones of cells with varying degress of dysplasia. The chance of porokeratosis becoming cancerous is increased if the affected area is large, located on the limbs, or has been present for a long time. Some lesions can develop into malignant carcinomas. Pk is disorder of keratinization characterized by annular lesions surrounded by raised sharply marginatedkeratotic borders with a characteristic histopathological finding named cornoid lamella. Porokeratosis the term porokeratosis refers to skin lesions with a thinned centre surrounded by a ridgelike border called the cornoid lamella. Different types include porokeratosis of mibelli, linear porokeratosis and disseminated superficial actinic porokeratosis dsap. Milwaukee, wi a patient with a lifelong linear porokeratosis was treated with a carbon dioxide laser.
Porokeratosis may be divided into the following clinical types 532 plaquetype porokeratosis also known as classic porokeratosis and porokeratosis of mibelli is characterized by skin lesions that start as small, brownish papules that slowly enlarge to form irregular, annular, hyperkeratotic or verrucous plaques. Dermis porokeratosis of mibelli information on the diagnosis. Apr 29, 2016 porokeratosis of mibelli is a rare, chronic disorder of epidermal keratinization characterized by hyperkeratotic papules or plaques surrounded by a great wall of chinalike elevated border. The clinical and histopathologic features supported a diagnosis of porokeratosis of mibelli pm.
Etretinate improves localized porokeratosis of mibelli janis p. It begins as one or a few small, brownish bumps that grow into raised, bumpy patches. Various types of porokeratosis have been identified. The bumps have a prominent border with a thin ridge, which means the edges are easily.
The following is a case of a young male who presented more than one form of pk simultaneously. Colocalization of lichen planus and porokeratosis of mibelli. Uncommon chronic progressive disorder of epidermal differentiation in combination with parakeratosis. A case of porokeratosis of hands and feet turning into a. Histopathological examination of the keratotic plug revealed findings of distinct epidermal depressions. Apr 28, 20 classic porokeratosis, the most common variant, was first described in 1893 by vittorio mibelli, hence the name porokeratosis of mibelli pm. The centre is often atrophic but may be hyperkeratotic. The term porokeratosis defines this family and refers to a characteristic skin lesion with a thinned center and a surrounding ringlike border. Porokeratosis of mibelli genetic and rare diseases. The lesion resolved while the patient was on the drug and continued to resolve after she had discontinued therapy. Sep 24, 2018 neri i, marzaduri s, passarini b, patrizi a. Porokeratosis treatment, pictures, symptoms and causes.
Bloom and abramowitz 1943 described porokeratosis of mibelli in an italian man and his 2 sons. Classic porokeratosis of mibelli is one type of porokeratosis usually appearing in childhood, but lesions may be present at birth, puberty or later. Two male patients with a localized type of porokeratosis limited to. Aug 24, 2012 porokeratosis of mibelli is a skin condition that usually develops in children or young adults. Pdf on dec 1, 2014, nidhi singh and others published classic porokeratosis of mibelli find, read and cite all the research you need on researchgate. To date only cases of this disease have been reported. Brown skin porokeratosis manifests itself through reddish lesions and spots on various parts of the body mainly appearing on the legs as well as the back part of the body. Such lesions may not always be clinically diagnosed, hence it requires histopathological examination.
Identification of a locus for disseminated superficial actinic porokeratosis at chromosome 12q23. Dermis porokeratosis of mibelli information on the. Porokeratosis of mibelli presents as a single welldemarcated circular plaque that slowly expands in a centripetal fashion with central atrophy and raised border. The skin lesions that result are dry and atrophic, with a welldefined ridgelike hyperkeratotic border called a cornoid lamella best seen on dermoscopy 1. Lesions appear most often on the limbs and show a tendency to. Classic porokeratosis, the most common variant, was first described in 1893 by vittorio mibelli, hence the name porokeratosis of mibelli pm. One article showed that porokeratosis of mibelli progressed to skin cancer in approximately 8% of affected individuals. Pearls% look,for,characteristic, cornoidlamella, center,oflesionmay, show,epidermal,atrophy, and,a,lichenoid, dermatitis,always,cut, deepers,to,see,entire, lesion. Lesions of porokeratosis are thought to arise from disordered keratinization, though the exact pathogenesis remains uncertain.
We report a very unusual case of porokeratosis of hands and feet. Porokeratosis of mibelli is a rare type with onset in childhood. It is seen in men more than twice as often as women and may first appear in childhood or young adulthood. Porokeratosis of mibelli most often affects the limbs, particularly the hands and feet, lower leg, neck, shoulders, face or genitals, although any part of the body may be affected including the mucous membranes 5.
Aug 24, 2012 porokeratosis of mibelli can give rise skin cancer. Its prevalence is relatively higher in ladies than gents. Sep 12, 2017 classic mibelli porokeratosis pm appearance. Porokeratosis of mibelli centro hospitalar do porto.
Porokeratosis is a rare, acquired or inherited disorder of keratinization characterized by one or more atrophic macules or patches, each surrounded by a distinctive hyperkeratotic, ridgelike border called cornoid lamella picture 1d 1,2. Clinical forms include the classic mibelli type, disseminated superficial actinic form, multiple punctuate palmoplantar, nevoid linear, facial, mucosal and the solitary discrete planter 22,23,24,25 facial porokeratosis appears varieties. It consists of a heterogeneous group of disorders inherited in a autosomal dominant fashion. Koley s, sarkar j, choudhary s, dhara s, choudhury m, bhattacharya s. Porokeratosis of mibelli department of dermatology. This case report involves a 20yearold man with unilateral punctate porokeratosis. Health, general nail diseases care and treatment health aspects psychic trauma research retinoids drug therapy trauma psychology.
A solitary porokeratosis of mibelli over the nose is uncommon. At least 5 clinical subtypes of porokeratosis have been identified. This conssts of a tier of parakeatotic marginal material. Examination of the father and sons showed that one had only annular lesions, and the 2 others had in addition the linear and gyrate plaque lesions.
It may be caused by genetics, uv rays or immunosuppression. Porokeratosis of mibelli is transmitted as an irregular autosomal dominant trait but may occur spontaneously. Porokeratosis may be divided into the following clinical types 532 plaquetype porokeratosis also known as classic porokeratosis and porokeratosis of mibelli is characterized by skin lesions that start as small, brownish papules that slowly enlarge to form irregular, annular, hyperkeratotic or verrucous plaques 533. The four main clinical variants were classical porokeratosis of mibelli 56%, disseminated superficial actinic porokeratosis dsap 18%, disseminated superficial porokeratosis dsp 11%, and linear porokeratosis %. A classical lesion showing an annular plaque with normal or atrophic centers surrounded by a keratotic ridge 9. These small brownish bumps may increase in size over time. Pdf solitary porokeratosis of mibelli at an unusual site. They arise from clones of cells with varying degrees of dysplasia. Pk has a wide variety of manifestations including classical plaquetype porokeratosis of mibelli. Porokeratosis is a skin condition involving lesions with thin center surrounded by raised ridges. May be associated with a slight increase in neoplasia basal cell and squamous cell carcinoma. Even if there is a coexistence of various clinical forms 3, the one between porokeratosis of mibelli and disseminated superficial form is rarely described. The patient was treated with a 4week trial of imiquimod cream followed by an 8week trial of fluorouracil cream, without success.
The lesions usually reach only a few centimetres in size but giant porokeratomas can be as large as 10 to 20 cm wide 6. Treatment of porokeratosis of mibelli with combined use of. Porokeratosis is an assorted group of five genetic disorders. Porokeratosis of mibelli may sometimes harm normal tissue underlying the affected area.
Porokeratosis pk consists of abnormal keratinization of the epidermis of uncertain etiology and was first described by mibelli in 1893. We report familial autosomal dominant with reduced penetrance disseminated plaque type mibellis type porokeratosis in a father and son. Squamous cell carcinoma in lesions of disseminated. Carver college of medicine department of dermatology 200 hawkins drive 40025 pomerantz family pavilion iowa city, ia 522421089.
Linear porokeratosis associated with multiple squamous. These include porokeratosis of mibelli, dsap, punctate porokeratosis, porokeratosis palmaris et plantaris disseminata, and linear porokeratosis. The laser was successful in removing the patients hyperkeratotic plantar lesion. Porokeratosis a mutant clonal keratosis of the epidermis. Porokeratosis of mibelli of upper lip extending into oral.
The patient presented an 8year history of numerous asymptomatic keratotic papules and pits with linear distribution on his left palmar surface and fifth finger of the left hand. Porokeratosis is a skin disease characterized by a thickening of the stratum corneum together with progressive centrifugal atrophy. Out of 55 cases, there were 22 cases of porokeratosis of mibelli, 17 cases of disseminated superficial actinic porokeratosis dsap, 15 cases of disseminated superficial porokeratosis and one case of linear. Chromosomal instability associated with susceptibility to malignant disease in patients with porokeratosis of mibelli. Porokeratosis of mibelli pm is the second most common form of porokeratosis, accounting for about a third of reported cases. Hyperkeratotic variant of porokeratosis in a patient with hepatitis. Porokeratosis is a specific disorder of keratinization characterized histologically by the presence of cornoid lamella. Other variants, including linear porokeratosis, punctate porokeratosis, and porokeratosis of the palms and soles, have also been described. Chan dermatology clinic, hong kong 3diagnostix pathology laboratories ltd.
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