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Year : 2015  |  Volume : 6  |  Issue : 6  |  Page : 452-453  

Porokeratosis and malignancy: Incidental or causal association?

Department of Dermatology, Govt. Medical College, Calicut, Kerala, India

Date of Web Publication17-Nov-2015

Correspondence Address:
Najeeba Riyaz
Arakkal, Chalapuram, Calicut, Kerala
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Source of Support: None, Conflict of Interest: None

PMID: 26753153

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How to cite this article:
Riyaz N. Porokeratosis and malignancy: Incidental or causal association?. Indian Dermatol Online J 2015;6:452-3

How to cite this URL:
Riyaz N. Porokeratosis and malignancy: Incidental or causal association?. Indian Dermatol Online J [serial online] 2015 [cited 2021 Oct 27];6:452-3. Available from: https://www.idoj.in/text.asp?2015/6/6/452/169717

Porokeratosis is a unique genodermatosis inherited by an autosomal dominant trait.[1] It is characterized by hyperkeratotic lesions with atrophic center and raised wall-like border, which histologically show the classical cornoid lamella. The five distinct clinical types of porokeratosis include the classical Mibelli, disseminated superficial actinic porokeratosis (DSAP), porokeratosis palmaris et plantaris disseminata (PPPD), linear, and punctate types. Rarely pruritic and ptychotropic variants have also been described.

A mutant clone of epidermal cells is thought to be the cause for cornoid lamella, predisposing affected patients to malignancy.[2] The exact risk of cutaneous malignancy developing in porokeratosis is unknown. Radiation therapy could be a precipitating factor for development of malignancy.[1] Other important trigger factors for the development of malignancy in porokeratosis include ultraviolet radiation, electron beam therapy, and immunosuppression associated with malignancies such as lymphoma, HIV infection, or due to immune-modulatory drugs used for autoimmune diseases or following organ transplantation.[3] Development of squamous or basal cell carcinomas has been reported in all forms of porokeratosis.[4] Chromosomal instability and reduced immune surveillance with overexpression of p53 are hypothesized to play a role in the development of cutaneous malignancies within porokeratosis [4],[5] and throughout epidermis in Bowenoid lesions.[6] Gene expression profiles reveal an upregulation of mRNAs of hyperproliferative keratins, calcium-binding proteins, connexin 26 and 30 and involucrin in the cornoid lamellae, as in psoriasis.[7]

In a 30-year study by Sasson et al. to know the frequency of malignancy in porokeratosis, 7.5% (21/281 patients) developed malignancy. Increased risk of malignancy was observed in linear, large, and long-standing cases. Squamous cell carcinoma developing in linear and giant types of porokeratosis, has been reported.[7],[8] Metastasis although rare, has occurred in a giant lesion along with hypercalcemia.[7]

There are reports of epithelioma [9] and basal cell carcinoma [10] arising from porokeratosis of Mibelli, DSAP, as well as in PPPD, and linear porokeratosis possibly from the mutant clone of cells. Chromosomal instability of cultured fibroblasts from patients with porokeratosis of Mibelli is thought to have a causal role for the associated malignancy.[11]

Premalignant lesions such as Bowen's disease,[12] cutaneous horns,[13],[14] and dysplasia at the base of cutaneous horn [15],[16] may arise from porokeratosis, which may progress to malignancy. Porokeratosis is now considered a premalignant condition, with certain groups of patients at greatest risk for malignant transformation.[3]

In view of the overexpression of p53 gene(whichhas a wide spectrum of mutations) in porokeratosis, it is possible that malignant transformation is likely in long-standing lesions with prolonged sun exposure irrespective of the type of porokeratosis. Hence a close follow up of lesions is mandatory. Although there is only one report of malignant melanoma arising from porokeratosis [17] so far, in view of the malignant potential of porokeratosis in general, the present report of malignant melanoma in DSAP could be more than an incidental association and hence may be significant.

   References Top

Wolff S. Porokeratosis. In: Irwin F, Eisen AZ, Wolff K, editors. Fitzpatricks's Dermatology in General Medicare. Vol. 1. 6th ed. New York: McGraw-Hill; 2003. p. 532-7.  Back to cited text no. 1
Reed RJ, Leone P. Porokeratosis: A mutant clonal keratosis of the epidermis. I. Histogenesis. Arch Dermatol 1970;101:340-7.  Back to cited text no. 2
Breneman DL, Breneman JC. Cutaneous T-cell lymphoma mimicking porokeratosis of Mibelli. J Am Acad Dermatol 1993;29:1046-8.  Back to cited text no. 3
Sasson M, Krain AD. Porokeratosis and cutaneous malignancy. A review. Dermatol Surg 1996;22:339-42.  Back to cited text no. 4
Magee JW, McCalmont TH, Leboit PE. Overexpression of p53 tumor suppressor protein in porokeratosis. Arch Dermatol 1994;130:187-90.  Back to cited text no. 5
Sertznig P, von Felbert V, Megahed M. Porokeratosis: Present concepts. J Eur Acad Dermatol Venereol 2012;26:404-12.  Back to cited text no. 6
Murase J, Gilliam AC. Disseminated superficial actinic porokeratosis co-existing with linear and verrucous porokeratosis in an elderly woman: Update on the genetics and clinical expression of porokeratosis. J Am Acad Dermatol 2010;63:886-91.  Back to cited text no. 7
Sasaki S, Urano Y, Nakagawa K, Nagae H, Nakanishi H, Arase S. Linear porokeratosis with multiple squamous cell carcinomas: Study of p53 expression in porokeratosis and squamous cell carcinomas. Br J Dermatol 1996;134:1151-3.  Back to cited text no. 8
Sawai T, Hayakawa H, Danno K, Miyauchi H, Uehara M. Squamous cell carcinoma arising from giant porokeratosis: A case with extensive metastasis and hypercalcemia. J Am Acad Dermatol 1996;34:507-9.  Back to cited text no. 9
Cort DF, Abdel-Aziz AH. Epithelioma arising in porokeratosis of Mibelli. Br J Plast Surg 1972;25:318-28.  Back to cited text no. 10
Sarkany I. Porokeratosis of Mibelli with basal cell epithelioma. Proc R Soc Med 1973;66;435-6.  Back to cited text no. 11
Taylor AM, Hamden DG, Fairburn EA. Chromosomal instability associated with susceptibility to malignant disease in patients with porokeratosis of Mibelli. J Natl Cancer Inst 1973;51:371-8.  Back to cited text no. 12
Coskey RJ, Mehregan A. Bowen disease associated with porokeratosis of Mibelli. Arch Dermatol 1975;111:1480-1.  Back to cited text no. 13
Riyaz N, Nair VL. Multiple cutaneous horns arising from porokeratosis. Indian J Dermatol Venereol Leprol 1994;60:151-2.  Back to cited text no. 14
  Medknow Journal  
Yesudian DP, Krishnan SG, Jayaraman M, Janaki VR, Yesudian P. Occurrence of squamous cell carcinoma and multiple cutaneous horns in porokeratosis. Indian J Dermatol Venereol Leprol 1995;61:326-7.  Back to cited text no. 15
[PUBMED]  Medknow Journal  
Saritha M, Kumari R, Thappa DM, Rajesh NG, Verma SK. Benign giant cutaneous horn formed by giant porokeratosis of Mibelli with dysplasia. Indian J Dermatol Venereol Leprol 2013;79:433-5.  Back to cited text no. 16
[PUBMED]  Medknow Journal  
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. 17


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