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Year : 2013  |  Volume : 4  |  Issue : 3  |  Page : 262-263  

Porokeratosis of Mibelli: Giant variant


Department of Dermatology, L. T. M. Medical College and General Hospital, Mumbai, Maharashtra, India

Date of Web Publication24-Jul-2013

Correspondence Address:
Ganesh Avhad
Room no. 304, New RMO Hostel, L. T. M. Medical College and General Hospital, Sion, Mumbai - 400 022, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.115547

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How to cite this article:
Avhad G, Jerajani H. Porokeratosis of Mibelli: Giant variant. Indian Dermatol Online J 2013;4:262-3

How to cite this URL:
Avhad G, Jerajani H. Porokeratosis of Mibelli: Giant variant. Indian Dermatol Online J [serial online] 2013 [cited 2019 Dec 11];4:262-3. Available from: http://www.idoj.in/text.asp?2013/4/3/262/115547

A 45-year-old female presented with hyperpigmented, solitary, annular plaque on her right buttock since 12 years. She also gave a history of gradual increase in the size of the lesion [Figure 1].
Figure 1: Single erythematous, oval, scaly plaque 18 cm X 15 cm with characteristic peripheral ridge with central atrophy

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Physical examination showed a single erythematous, circular, scaly plaque 18 cm × 15 cm with characteristic peripheral ridge measuring 12 mm. Histopathology showed stacked parakeratosis within epidermal invagination and underlying absent granular layer, which was suggestive of coronoid lamella [Figure 2].
Figure 2: Histopathology of plaque showing invagination of epidermis with prominent cornoid lamella and underlying loss of the granular layer, inset showing dyskeratotic cells (shown by arrow) seen beneath the coronoid lamella (100X)

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Porokeratosis is a benign, rare, genetically determined autosomal dominant disorder of epidermal keratinization, characterized clinically by hyperkeratotic papules or plaques surrounded by a thread like elevated border that expands centrifugally. Multiple etiologies are proposed in the clonal proliferation of keratinocytes, like chronic sun exposure, Hepatitis B and C infection, HIV and immunosuppresion.

The classic lesion of porokeratosis [1] was first described by Mibelli in 1893. It is usually seen during childhood as one or multiple annular plaques with central atrophy and elevated keratotic borders usually greater than 1 mm in height that have a longitudinal furrow typically seen in the center of the ridge. This ridge expands over a period of time. It affects men twice as often as women. The lesion may be hypopigmented or hyperpigmented, scaly, atrophic, hairless and anhidrotic. It commonly develops on the extremities but has also been known to occur on the face, buccal mucosa, genitalia, palms and soles. [2]

Giant porokeratosis is considered to be a morphological variant of porokeratosis of Mibelli with a diameter of up to 20 cm and surrounding wall of 1 cm. [3]

As there is risk of development of squamous cell carcinoma in giant porokeratosis (10%), early diagnosis and treatment is necessary. [4]

There are various modalities of treatment like topical 5-fluorouracil, imiquimod, oral retinoid, CO2 laser ablation, 585-nm pulsed dye laser radiation, Grenz ray radiation, Nd:YAG laser radiation, cryotherapy, dermabrasion, surgical excision and electrodesiccation. [5]

 
   References Top

1.Judge MR, McLea WH, Munro CS. Disorder of keratinization. In: Burn T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of dermatology. 7 th ed. Oxford: Blackwell Science; 2004. p. 34.75-7.  Back to cited text no. 1
    
2.Pizzichetta MA, Canzonieri V, Massone C, Soyer HP. Clinical and Dermoscopic Features of Porokeratosis of Mibelli. Arch Dermatol 2009;145:91-2.   Back to cited text no. 2
[PUBMED]    
3.Raychaudhury T, Valsamma DP. Giant porokeratosis. Indian J Dermatol Venereol Leprol 2011;77:601-2.   Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.Maubec E, Duvillard P, Margulis A, Bachollet B, Degois G, Avril MF. Common skin cancers in porokeratosis. Br J Dermatol 2005;152:1389-91.  Back to cited text no. 4
[PUBMED]    
5.Agarwal S, Berth-Jones J. Porokeratosis of Mibelli: successful treatment with 5% imiquimod cream. Br J Dermatol 2002;146:331-44.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]


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