Linear porokeratosis with multiple squamous cell carcinomas successfully treated by electrochemotherapy. (17th August 2016)

• Record Type: Journal Article
• Authors: Sommerlad, M.; Lock, A.; Moir, G.; McGregor, J.; Bull, R.; Cerio, R.; Harwood, C.
• Is Part Of: British journal of dermatology. Volume 175:Number 6(2016)
• Page Start: 1342
• Page End: 1345
• Publication Date: 2016-08-17
• Subjects: Dermatology -- Periodicals; Skin -- Diseases -- Periodicals; 616.5
• DOI: 10.1111/bjd.14669
• Languages: English
• ISSNs: 0007-0963

Abstract

Summary: Porokeratosis is a clonal epidermal disorder of keratinization characterized by annular lesions with an atrophic centre and a hyperkeratotic edge. The cornoid lamella is the histopathological hallmark. Six clinical variants are recognized: porokeratosis of Mibelli; disseminated superficial porokeratosis; disseminated superficial actinic porokeratosis (DSAP); porokeratosis plantaris et palmaris disseminata; punctate porokeratosis and linear porokeratosis. Linear porokeratosis is the type most frequently associated with malignant transformation into squamous cell carcinoma (SCC). It is thought to represent a mosaic form of DSAP and has an incidence of less than 1 in 200 000; treatment options are limited. We describe a patient with systematized linear porokeratosis and multiple SCCs who was successfully treated with bleomycin electrochemotherapy (ECT), a form of intralesional chemotherapy. In view of their large number, the individual SCCs were treated with bleomycin ECT. One year post‐treatment the patient remains tumour free. To our knowledge, this is the first case of multiple SCCs treated by ECT in the context of systematized linear porokeratosis. Our case highlights the challenges associated with diagnosing and managing this unusual form of porokeratosis. Abstract : What's already known about this topic? Linear porokeratosis is a rare variant of porokeratosis thought to represent a mosaic form of disseminated superficial porokeratosis. The diagnosis may be difficult to make. Patients are often initially diagnosed with variants of psoriasis or epidermal naevi. Linear porokeratosis has the highest rate of malignant transformation. Treatment can be challenging due to the multiplicity and location of tumours. What does this study add? This is the first report of multiple squamous cell carcinomas in the context of linear porokeratosis being successfully treated with electrochemotherapy (ECT). ECT resulted in significant and sustained clearance of linear porokeratosis. We illustrate the importance of multidisciplinary team input in the diagnosis and management of systematized linear porokeratosis. Linked Comment: Olasz and Kis. Br J Dermatol 2016;175 :1148–1149 .