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LETTER TO THE EDITOR
Year : 2019  |  Volume : 8  |  Issue : 1  |  Page : 77-79

An Unusual location of porokeratosis of Mibelli


Department of DVL, P.E.S. Institute of Medical Sciences and Research, Kuppam, Andhra Pradesh, India

Date of Submission22-Jan-2014
Date of Acceptance22-Jun-2016
Date of Web Publication26-Apr-2019

Correspondence Address:
Dr. Yugandar Inakanti
Department of DVL, P.E.S. Institute of Medical Sciences and Research, Kuppam - 517 425, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.257158

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How to cite this article:
Inakanti Y, Peddireddy S, Nagaraja A, Kotla S. An Unusual location of porokeratosis of Mibelli. J NTR Univ Health Sci 2019;8:77-9

How to cite this URL:
Inakanti Y, Peddireddy S, Nagaraja A, Kotla S. An Unusual location of porokeratosis of Mibelli. J NTR Univ Health Sci [serial online] 2019 [cited 2019 Sep 16];8:77-9. Available from: http://www.jdrntruhs.org/text.asp?2019/8/1/77/257158



Sir,

Porokeratosis (PK) is characterized by annular plaques with an atrophic centre and a hyperkeratotic peripheral ridge; pathologically, it is characterized by cornoid lamella which is mainly inherited in an autosomal dominant form,[1] first described by Mibelli in 1893. These lesions are most commonly found on the extremities, but can also be found on the genitalia, oral mucosa, and cornea.

This report describes a 26-year-old male patient, with porokeratosis of Mibelli (PKM), which was confirmed histopathologically. The rarity of this disorder, its clinical exuberance, and the destructive character of the lesions, as well as nasal involvement is unusual in this form of porokeratosis. The presenting history, clinical findings, biopsy results, and available literature are reviewed.

A 26-year-old male, married, born of nonconsanguineous parents, presented with multiple skin lesions over the nose. The lesions were slowly progressive with no signs of self-healing. The physical examinations were within normal limits. On systemic examination, no abnormality was identified, and laboratory investigations revealed normal values.

The clinical examination revealed annular papules, annular plaques with central clearing, and raised peripheral edge of different sizes varying from 2 mm to 20 mm over the dorsum of the nose and ala nasi, distributed bilateral symmetrically [Figure 1]. A full-depth skin biopsy was obtained from the outer part of the lesion with a 2 mm disposable skin biopsy punch and then subjected to histopathology.
Figure 1: Annular plaques and papules with central clearing with raised peripheral edge, i.e., cornoid lamella clinically visible border between normal and abnormal epidermal cells

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The histopathology revealed foci of epidermal invagination filled with keratin and parakeratotic cornoid lamella. Dermis showed a mild-to-moderate infiltrate of lymphocytes [Figure 2] and [Figure 3], with above findings, a diagnosis of classical PKM was made. Topical tretinoin was prescribed for the management of the lesion.
Figure 2: Epidermal invagination of keratin around glands, abnormal keratinocytes forming cornoid lamella

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Figure 3: Abnormal keratinocyte with pyknotic nuclei few, dyskeratotic cells, parakeratotic cells forming column known as cornoid lamella and dermal infiltrate with lymphocytes

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Porokeratosis is a clonal disorder of keratinization characterized by one or more atrophic patches surrounded by a clinically and histological distinctive hyperkeratotic ridge-like border called the cornoid lamella. PKM may be familial, inherited as an AD disorder, with onset in the childhood or sporadic with later onset.

Clinical variants are classic PKM, disseminated superficial actinic porokeratosis, punctate porokeratosis, porokeratosis palmaris et plantaris disseminate, and linear porokeratosis.[1] PKM is the most characteristic and distinctive variant of the five described forms of porokeratosis. The classical form of PKM consists of a single plaque or a small number of plaques of variable size which most often affects the limbs, particularly the hands and feet, the neck, and shoulders, rarely oral mucosa and cornea.[2]

Porokeratosis may be associatiated with diabetes mellitus, human immunodeficiency virus infection,[3] liver disease, renal transplant, and hematologic or solid organ malignancy.

Histopathology of porokeratosis shows hyperkeratotic lesion with cornoid lamella which represents visibly raised margin of the lesion and is a parakeratotic column overlying a small vertical zone of dyskeratotic and vacuolated cells within the epidermis along with focal loss of granular cell layer; mild lymphocytic infiltrate may be seen. The atypical keratinocytes show abnormal differentiation, however, they do not show an increased rate of proliferation.[4]

Our case showed annular papules, annular plaques with central clearin, g and raised peripheral edge over the nose and cornoid lamella on histopathological examination, which confirmed diagnosis plaque type variant of classical PKM.

The management of porokeratosis is individualized. Sun protection, emollients, and observation for signs of malignant degeneration are mandatory. It has been treated with topical 5-fluorouracil, CO2 laser vaporization, cryotherapy, topical retinoids, imiquimod, shave excision, curettage, linear excision, and photodynamic therapy.

A classical form of porokeratosis over the nose is an uncommon condition. Usually patients never seek advice because lesions may remain asymptomatic. Histopathological examination is required for diagnosis and early identification of neoplastic changes. Hence, we report a male patient with PKM over the nose, which is a rare entity. Only two cases have been reported so far.[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Biswas A. Cornoid lamellation revisited: apropos of porokeratosis with emphasis on unusual clinicopathological variants. Am J Dermatopathol. 2015 37:145-55.  Back to cited text no. 1
    
2.
Bacharach-Buhles M, Weindorf N, Altmeyer P. Porokeratosis Mibelli gigantea. Hautarzt 1990;41:633-5.  Back to cited text no. 2
    
3.
Rodriguez EA, Jakubowicz S, Chinchilla DA, Carril A, Viglioglia PA. Porokeratosis of Mibelli and HIV-infection. Int J Dermatol 1996;35:402-4.  Back to cited text no. 3
    
4.
Jurecka W, Neumann RA, Knobler RM. Porokeratoses: Immunohistochemical, light and electron microscopic evaluation. J Am Acad Dermatol 1991;24:96-101.  Back to cited text no. 4
    
5.
Chaudhary RG, Bilimore F, Katare SK. Large annular plaque with central atrophy over nose. Indian J Dermatol Venerol Leperol 2009;75:552.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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