engleski

Case report of a young female patient with squamous cell carcinoma arising from unilateral linear porokeratosis

Introduction: Linear porokeratosis (LP) is a rare clinical variant of porokeratosis that usually presents at birth or in early childhood as a result of genetic mosaicism. This keratinization disorder is characterized by multiple atrophic macules or patches, each surrounded by a hyperkeratotic border, arranged in one or multiple lines along a limb, or unilaterally on the trunk, face or neck. Case report: A 31-year old female patient presented to our department due to sudden appearance of several reddish nodules on the right thigh, which developed within two months after a sunburn. These lesions appeared on the background of extensive skin changes involving the right side of the trunk and the right leg that had been present since birth. Examination revealed numerous confluent hyperpigmented macules with a slightly depressed center and raised keratotic edge, distributed in a blaschkoid pattern on the right half of the trunk, right gluteal region and along the right lower extremity. On the extensor surface of the right thigh, there were several erythematous, keratotic papules measuring 3 to 10 mm, clinically resembling actinic keratoses, with the largest one centrally ulcerated. Incisional biopsy sampled from the edge of one of the “background” brownish patches showed atrophic and hyperkeratotic epidermis with cornoid lamella, the hallmark histopathologic finding in porokeratosis. Excisional biopsy of the ulcerated papule revealed a microinvasive squamous cell carcinoma. Further treatment employed throughout the 4-year follow-up period included cryotherapy, adapalene, photodynamic therapy with 5-aminolevulinic acid and ingenol mebutate. This led to partial regression in the treated zones, which smoothed out and the redness diminished. Conclusion: Linear porokeratosis is a rare form of porokeratosis with significant risk of malignant transformation into basal or squamous cell carcinoma. This can particularly occur in the setting of immunosuppression or it may be triggered by the additionally acquired, UV-induced damage, as was the case in our patient. Thus, education on strict sun protection is of utmost importance in the management of patients with this rare genodermatosis, along with the continuous clinical follow-up aimed at early detection and timely treatment of keratinocytic malignancies.

linear porokeratosis ; squamous cell carcinoma ; photodynamic therapy ; ingenol mebutate

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