Clinico-pathologic profile of skin cancers in oculocutaneous albinism at Universitas Academic Hospital

Abstract

Background: Oculocutaneous albinism (OCA) is a genetic disorder found worldwide. Skin cancer is a significant risk for people with albinism, particularly in Africa, where it is a major cause of death. Many patients delay seeking medical care until their skin lesions are in advanced stages.

Aim: The aim of this study was to describe the clinico-pathological profile of skin cancers in patients with albinism at their initial presentation to our dermatology outpatient department.

Setting: This study was conducted at the dermatology department of Universitas Academic Hospital, Bloemfontein, South Africa.

Methods: A retrospective descriptive study covering June 2009 to July 2019 was conducted. Only records of oculo-cutaneous albinism patients diagnosed with skin cancer during their initial visit were included.

Results: Eighty-six patients with albinism were recorded, 37% (n = 32) of whom had skin cancer at their first visit. Females (81%) were more affected than males (19%). The majority of skin cancers were squamous cell carcinomas (SCCs) (54%) and basal cell carcinomas (BCCs) (46%). No melanomas were found. Most SCCs were classified as aggressive (80.4%), compared to 30.8% of BCCs.

Conclusion: Almost 40% of OCA patients presented with skin cancer at their initial visit, highlighting the need for strengthening primary healthcare systems’ efficiency in ensuring early referrals for OCA patients.

Contribution: Education, socioeconomic support and awareness campaigns are sine qua non actionable factors that could help encourage early medical evaluation for all OCA patients.