|Year : 2018 | Volume
| Issue : 3 | Page : 272-273
Focal actinic porokeratosis: A new variant?
Pihu Sethi1, Neeti Kumari1, Bhuvan Adhlakha2, Amrinder Jit Kanwar3
1 Department of Dermatology, Venereology and Leprosy, Sri Guru Ram Rai Institute of Medical and Health Sciences, Dehradun, Uttarakhand, India
2 Department of Pathology, S Nijalingappa Medical College, HSK Hospital and Research Centre, Navanagar, Karnataka, India
3 Department of Dermatology, Venereology and Leprosy, School of Medical Sciences and Research, Sharda Hospital, Greater Noida, Uttar Pradesh, India
|Date of Web Publication||28-Jun-2018|
Dr. Pihu Sethi
D-16, 2nd Floor, Chander Nagar, Ghaziabad - 201 011, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Porokeratosis is an autosomal dominantly transmitted disorder of keratinization predominantly affecting trunk and extremities. Many clinical variants have been described having a common histopathological feature of coronoid lamella-porokeratosis of Mibelli, superficial disseminate, disseminated superficial actinic porokeratosis, linear porokeratosis and punctuate palmoplantar porokeratosis. We present a case of 16-year-old female who had focal lesions of porokeratosis aggravated by sun exposure, over alae of nose confirmed by histopathology. The previous reports show quite a number of cases of isolated lesions of the disease on nose and perinasal area. This article highlights the actinic nature of focal facial porokeratosis.
Keywords: Coronoid lamella, focal actinic, porokeratosis
|How to cite this article:|
Sethi P, Kumari N, Adhlakha B, Kanwar AJ. Focal actinic porokeratosis: A new variant?. Indian J Paediatr Dermatol 2018;19:272-3
|How to cite this URL:|
Sethi P, Kumari N, Adhlakha B, Kanwar AJ. Focal actinic porokeratosis: A new variant?. Indian J Paediatr Dermatol [serial online] 2018 [cited 2020 Oct 21];19:272-3. Available from: https://www.ijpd.in/text.asp?2018/19/3/272/206089
| Introduction|| |
Porokeratosis is a disorder of keratinization which is transmitted in an autosomal dominant fashion, but sporadic cases are also seen. It is characterized clinically by annular atrophic plaques with serpiginous keratotic rim at the periphery. In the literature, many variants have been described - porokeratosis of Mibelli (PM), superficial disseminate, disseminated superficial actinic porokeratosis (DSAP), linear porokeratosis, and punctate palmoplantar porokeratosis. Some atypical presentations such as hypertrophic, warty, nodular, giant, genital porokeratosis, porokeratosis ptychotropica, and porokeratoma have also been mentioned., Few cases of isolated lesions of porokeratosis on face, particularly nasal and perinasal area have been described,,,, which cannot be put under any of the previous mentioned types. As involvement of the face, without any other body part, that too aggravated by sun exposure is not very common, focal actinic porokeratosis can be described as a variant.
| Case Report|| |
A 16-year-old muslim female, born of nonconsanguineous parents, presented with complaints of lesions over nose for 4 months. It started as small raised skin-colored lesions which gradually increased peripherally with raised margins and central clearing. She complained burning sensation, itching, and redness over the lesions after 10–15 min of exposure to sunlight which subsided in ½–1 h after coming back to shade. There was no history of oral or topical application of drugs, irradiation, or family history of similar complaints. On examination, brownish atrophic plaque measuring 3 cm in diameter, with surrounding thread-like border with few papules on the inner aspect of the border was seen over the left alae of nose [Figure 1]. There was no evidence of photodamage on the other areas of the face. Histopathological examination revealed coronoid lamella-keratin filled epidermal invagination with a parakeratotic column [Figure 2]. Initially, the patient was advised only photoprotection and moisturisers. Symptoms improved and there was mild decrease in the size of the lesion but did not heal completely. On follow-up, topical calcipotriol cream was also added with the regimen. Lesion further reduced in 2 months posttreatment.
|Figure 1: Three-centimeter atrophic plaque with hyperkeratotic rim at the left alae of nose|
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|Figure 2: Coronoid lamella with underlined absent granular layer (H and E, ×100)|
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| Discussion|| |
Porokeratosis is a disorder of keratinization which can be inherited in an autosomal dominant manner or can occur sporadically. It is seen in the age group of 20–30 years with males being more commonly affected. The pathogenesis of porokeratosis is still unclear; however, it has been described that there is focal clonal expansion of abnormal cells giving rise to a shape of coronoid lamella. Genetic mutations in SSH1 gene on chromosome 12 (encodes for a phosphatize enzyme which helps in actin dynamics), ARPC3 gene (involved in actin cytoskeleton pathway and interaction with adherent junctions in keratinocytes), and mevalonate kinase gene which helps in lipid metabolism.
Out of all the types, DSAP is the most commonly seen, followed by PM. Lesions of DSAP are characterized by annular atrophic plaques with serpiginous keratotic rim at the periphery mainly affecting trunk and extremities. Fifteen percent of DSAP cases may have facial involvement, whereas exclusive facial presentation is extremely rare. Isolated lesions on the nose are rarer.
Sharquie and Al-Baghdady described facial solar porokeratosis as a new variant, where classical lesions of porokeratosis were present only on face and were exacerbated by sun exposure, confirmed on histopathology. Many reports have shown isolated lesions of porokeratosis occurring over the face, particularly on alae of the nose or perinasal area.,,,,,
Facial solar porokeratosis is clinically described as single or multiple, skin colored or brownish atrophic papules or plaques with serpiginous keratotic rim at the periphery occurring mostly over distal part of nose. Lesions can be 0.1 cm to several centimeters in size and may be destructive. All the clinical variants which have been described share a common histopathological feature of coronoid lamella. Lesions of porokeratosis are chronic and slowly progressive. No standardized treatment for the disease has been described. First-line management includes photoprotection and 5-fluorouracil and also the role of calcipotriol, imiquimod, topical retinoids, and steroids have been described.
Many case reports with isolated lesions of porokeratosis on the face and scalp have been described. Although Thomsen et al. have described solitary actinic porokeratosis, a way back in 1979 on bald actinically damaged scalp, we are describing isolated facial lesions of porokeratosis, which are mainly limited to the nose and perinasal area with no evidence of photodamage over other sites of face. As DSAP is an established variant, focal lesions of porokeratosis particularly on distal part of the nose which are photo exaggerated, focal actinic porokeratosis, as a variant can be described if proved on a larger scale.
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[Figure 1], [Figure 2]