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CASE REPORT
Year : 2015  |  Volume : 6  |  Issue : 2  |  Page : 101-104  

Porokeratosis - Head to toe: An unusual presentation


1 Department of Dermatology, Raja Rajeswari Medical College and Hospital, Bengaluru, Karnataka, India
2 Department of Dermatology, Vydehi Institute of Medical Sciences and Research Hospital, Whitefield, Bengaluru, Karnataka, India

Date of Web Publication11-Mar-2015

Correspondence Address:
Dr. Ashok K Jaiswal
Department of Dermatology, Vydehi Institute of Medical Sciences and Research Hospital, Whitefield, Bengaluru - 560 066, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.153012

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   Abstract 

Porokeratosis is a disorder of epidermal proliferation in which many different clinical variants can be distinguished. Herein we report a rare case of porokeratosis involving almost all parts of the body with simultaneous occurrence of various forms of the disease in the same individual (classical porokeratosis of Mibelli, disseminated superficial actinic porokeratosis, hypertrophic porokeratosis, and genital porokeratosis).

Keywords: Porokeratosis, rare coexistence, uncommon sites


How to cite this article:
Bhaskar S, Jaiswal AK, Raj N, Reddy D. Porokeratosis - Head to toe: An unusual presentation. Indian Dermatol Online J 2015;6:101-4

How to cite this URL:
Bhaskar S, Jaiswal AK, Raj N, Reddy D. Porokeratosis - Head to toe: An unusual presentation. Indian Dermatol Online J [serial online] 2015 [cited 2019 Jun 16];6:101-4. Available from: http://www.idoj.in/text.asp?2015/6/2/101/153012


   Introduction Top


Porokeratosis is a rare autosomal dominant type of genodermatosis featuring abnormal epidermal keratinization. [1] Classically, five clinical variants are recognized: Classic porokeratosis of Mibelli (CPM), disseminated superficial porokeratosis, disseminated superficial actinic porokeratosis (DSAP), porokeratosis palmaris et plantaris disseminate, and linear porokeratosis. [2] There are also reports of atypical presentation like hypertrophic variety of porokeratosis. [3] Scalp, mucous membrane, palms, soles, inguinal folds, and genitalia are not commonly involved. [4],[5],[6]


   Case report Top


A 20-year-old male patient presented with multiple, itchy and hyperpigmented annular lesions over scalp, face, neck, oral mucosa, trunk, extremities, genitalia, palms, and soles of two years duration. There was a history of photosensitivity. Initially, the lesions appeared over face and neck, gradually spread to involve the scalp, oral mucosa, extremities, trunk, groin, genitalia, palms, and soles. To begin with, the lesions were small keratotic hyperpigmented papules that subsequently enlarged to form ring like plaques with central atrophy surrounded by a discrete ridge. On family screening, it was found that his mother and brother had similar lesions.

Cutaneous examination revealed multiple, superficial, annular, hyperpigmented, atrophic plaques 1 to 3 cm in diameter with a slightly raised peripheral border over the face and neck [Figure 1]. Lesions over the front of the right ear, palms, and soles showed typical furrows [Figure 2] and [Figure 3]. Verrucous annular plaques with central atrophy were noted over the right forearm [Figure 4], scrotum and penis [Figure 5]. The lesion over the scalp was associated with alopecia [Figure 6]. A milky white, raised, cord-like lesion with maceration was seen over mucosal surface of the lower lip [Figure 7]. A vitiliginous depigmented macule was present over glans penis and mucosal surface of prepuce [Figure 8]. Dermoscopic examination showed a "white track" structure with brown pigmentation in the inside of the track [Figure 8]. A provisional diagnosis of porokeratosis was made, which was confirmed by histopathological examination [Figure 9] and [Figure 10].
Figure 1: Annular hyperpigmented atrophic macules with slightly raised peripheral border over the face and neck

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Figure 2: Lesion over the palms showing typical furrow

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Figure 3: Classical porokeratotic lesion of Mibelli over the medial border of left foot

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Figure 4: Single, verrucous annular plaque with central atrophy over the right forearm

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Figure 5: Multiple verrucous annular plaques seen over the groin, scrotum and penis

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Figure 6: Scalp lesion with associated alopecia

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Figure 7: Vitiliginous depigmented macule present over glans penis and under the surface of the prepuce with a porokeratotic lesion

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Figure 8: Dermoscopic examination showed a "white track" structure with brown pigmentation in the inside of the track

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Figure 9: Histopathology in low power view showing parakeratotic column present superficially, overlying an effaced epidermis (H and E, ×10)

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Figure 10: Skin biopsy showing the characteristic coronoid lamellae (H and E, ×100)

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   Discussion Top


Mibelli described classical porokeratosis in 1893. [4] Porokeratosis can occur with a genetic predisposition and it can also be secondary to risk factors such as sun exposure, immune suppression, and ultraviolet exposure. The incidence of Bowen's disease and squamous cell and spinous cell carcinomas is 6.8-11% in this disease, hence follow-up is essential. The risk is greater if the patient has linear-type porokeratosis, or a large or long-standing lesion. [7] Etiopathogenesis of porokeratosis is obscure, but certainly complex and multifactorial. It has been suggested that the lesions of porokeratosis result from the peripheral expansion of an abnormal, mutant clone of epidermal keratinocytes (which would be inherited) located at the base of the parakeratotic column. [8]

Clinically, the basic lesion is a crateriform horny papule that gradually extends to form a circinate or gyrate plaque with a distinct ridge like border and atrophic center. Histopathologically, it is characterized by coronoid lamella. [9] Though the lesions of porokeratosis may involve any part of the body, lesions on the scalp, mucous membrane, palms, soles, and genitalia are regarded uncommon. [4],[5],[6] There are various reports of coexistence of multiple variants of porokeratosis in the same individual. [10],[11]

Keratolytic agents, topical 5-flourouracil, topical and oral retinoid agents, topical imiquimod, cryotherapy, photodynamic therapy, carbon dioxide laser ablation, dermabrasion, and excision are used for treatment, [12] but achieving complete resolution of lesions is difficult. In our case, since the lesions were generalized, we considered oral acitretin 25 mg after the baseline investigations. Cryotherapy was done for hypertrophic lesions, and photoprotection was advised. As the inheritance of the disease is autosomally dominant, the patient was educated of possible future transmission of the disease in his progeny; we lost the patient to further follow-up.

The interesting features of our case report are: (1) Porokeratotic lesions involving almost all parts of the body from head to toe (2) involvement of uncommon sites such as scalp, oral mucosa, palms, soles and genitalia (3) simultaneous occurrence of various subtypes - CPM, DSAP, hypertrophic porokeratosis (4) association with vitiligo of the glans penis and (5) a positive family history.

To the best of our knowledge, this unusual and varied presentation of porokeratosis in the same individual is being reported for the first time in the literature.

 
   References Top

1.
Suh DH, Lee HS, Kim SD, Cho KH, Kim KH, Park KC. Coexistence of disseminated superficial porokeratosis in childhood with congenital linear porokeratosis. Pediatr Dermatol 2000;17:466-8.  Back to cited text no. 1
    
2.
Elder DE, Johnson B, Elenitsas R. Lever's Histopathology of the Skin. 9 th ed. Philadelphia: JB Lippincott Co.; 2005. p. 145-6.  Back to cited text no. 2
    
3.
Rajendran N. Warty porokeratosis. Indian J Dermatol Venereal Leprol 1989;55:59-60.  Back to cited text no. 3
    
4.
Elisabeth CI, Schreiner W. Porokeratosis. In: Fitzpatrick's Dermatology in General Medicine. 5 th ed. New York: McGraw Hill Publication; 1999. p. 624-8.  Back to cited text no. 4
    
5.
Neri I, Marzaduri S, Passarini B, Patrizi A. Genital porokeratosis of Mibelli. Genitourin Med 1995;71:410-1.  Back to cited text no. 5
    
6.
Chen TJ, Chou YC, Chen CH, Kuo TT, Hong HS. Genital porokeratosis: A series of 10 patients and review of the literature. Br J Dermatol 2006;155:325-9.  Back to cited text no. 6
    
7.
Gutierrez EL, Galarza C, Ramos W, Tello M, De Paz PC, Bobbio L, et al. Facial porokeratosis: A series of six patients. Australas J Dermatol 2010;51:191-4.  Back to cited text no. 7
    
8.
Kanitakis J, Euvrard S, Faure M, Claudy A. Porokeratosis and immunosuppression. Eur J Dermatol 1998;8:459-65.  Back to cited text no. 8
    
9.
Judge MR, McLean WH, Munro CS. Disorders of keratinisation. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook Textbook of Dermatology. 8 th ed. UK; Wiley-Blackwell; 2010. p. 19.90-91.  Back to cited text no. 9
    
10.
Suárez-Amor O, Pereiro-Ferreirós M, Ginarte M, Peteiro C, Toribio J. Coexistence of linear porokeratosis and disseminated superficial actinic porokeratosis: A type 2 segmental manifestation. Acta Derm Venereol 2007;87:363-4.  Back to cited text no. 10
    
11.
Palleschi GM, Torchia D. Porokeratosis of Mibelli and superficial disseminated porokeratosis. J Cutan Pathol 2008;35:253-5.  Back to cited text no. 11
    
12.
Lee Y, Choi EH. Exclusive facial porokeratosis: Histopathologically showing follicular cornoid lamellae. J Dermatol 2011;38:1072-5.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]



 

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