Dermatological and Epidemiological Profiles of Patients with Albinism in São Paulo, Brazil, between 2010 and 2017: A Cross-Sectional Study

Abstract

Introduction: Oculocutaneous albinism is an autosomal recessive disease caused by complete absence of or decrease in melanin biosynthesis in melanocytes. Due to the reduction or absence of melanin, albinos are highly susceptible to the harmful effects of ultraviolet radiation and are at greater risk of actinic damage and skin cancer. There are no epidemiological data on the incidence of albinism in Brazil. Objective: To analyze the clinical and epidemiological profile of patients with albinism treated by the Pró-Albino Program of the Dermatology Clinic of Santa Casa de Misericórdia from its beginning in 2010 until 2017. Methods: In this cross-sectional study, the records of all consecutive albino patients admitted to the service in the study period were reviewed. Sociodemographic data, family history, and dermatological clinical data were collected. Results: Between March 2010 and April 2017, 191 patients were admitted, of whom 109 were female (57.07%) and the age range was 0-92 years, with > 30% under the age of 18 years. Consanguinity among that enable the diagnosis and early treatment of these lesions, health education, and the use of photoprotective measures can reduce morbidity and mortality and improve the quality of life of patients with this rare genetic condition.

Skin Cancers in People with Albinism in Togo in 2019: Results of Two Rounds of National Mobile Skin Care Clinics

“…We have identified 54 cases of keratinocytes skin carcinomas dominated by BCC (57.4%), with some patients having multiple cases and types. This finding of multiple lesions has already been made in the literature in this population [8,9,16,17], including 14% (4 cases) of combined BCC and SCC in Brazil [8], one case of multiple BCC in India [16] and one case of SCC, Bowen's disease in Japan [17]. In our study, there was a predominance of BCC with a BCC/SCC ratio of 1.47.…”

“…In our study, the prevalence of keratinocytes skin cancers was 11.8%, lower than the 26% reported in Brazil [8], 25% in Tanzania [11], 23% in South Africa [12] and 20,98% in Nigeria [13], but higher than the 4.6% reported in France [14]. The reason for our low rate would be that other PWA in more remote areas, which do not even have access to health care centres, have not been affected by these activities.…”

“…As in our study in Nigeria [13], malignant skin lesions comprising 55% of BCCs, 22% of SCCs, 18% basosquamous carcinoma and 5% collision tumour (BCC and SCC). In Brazil [8], it was 62% BCC, 51% SCC and 7% melanoma. These three studies including ours, are cross sectional community based studies.…”

“…While the mobile consultation strategy is the one we have used in the campaigns, the majority of our consultations are limited to urban PWA. Moreover, PWA are the target of prejudice and social exclusion and have limited access to specialized medical care and resources [8,15].…”

“…PWA develop actinic damage at an early age and suffer from skin cancers in the second to fourth decade of life [6,7]. In most cases, several cancers occur aggressively in those who have not followed protective measures and have had relatively high levels of sun exposure [8]. African PWA are more prone to skin cancers because they live near the equator where exposure to the sun's ultraviolet rays is very high [9].…”