|Year : 2015 | Volume
| Issue : 1 | Page : 23-28
An analysis of skin cancer in albinos in Ibadan
Samuel Adesina Ademola
Department of Plastic, Reconstructive and Aesthetic Surgery, University College Hospital, Ibadan, Nigeria
|Date of Web Publication||8-Oct-2015|
Samuel Adesina Ademola
Department of Surgery, College of Medicine, University College Hospital, Ibadan
Source of Support: None, Conflict of Interest: None
Context: Skin cancers are common among albinos living in Africa. Deleterious effect of ultraviolet rays from sunlight and nearness to the equator places the African Albinos at risk for skin cancer.
Aims: This study aims to present skin cancers in albinos as seen by a plastic surgeon, sensitize the public to the magnitude of dangers that albinos are exposed to, highlight the challenges faced in their management and suggest strategies for improved outcomes.
Settings and Design: A retrospective review of skin cancers among albinos was conducted at the University College Hospital, Ibadan, Nigeria.
Methods and Material: The charts, operation and cancer registry records of all albinos referred to a plastic surgery division over a ten year period was reviewed. Demographic data and relevant information relating to skin lesions were extracted.
Statistical Analysis: Descriptive analysis was done with the aid of statistical package for social sciences (SPSS) version 20.
Results: Nineteen patients with fifty nine skin lesions comprising 13 males and 6 females were reviewed. Mean age of the patients was 33.6 (SD 12.8) years, 50% were unmarried, over 90% were Christians and 44% were not employed. The lesions were on the face in 84.6% while scalp and neck lesions were present in 36.8 and 31.6% of patients respectively. In 73.7% of the patients, the lesions were advanced. Basal cell carcinoma and squamous cell carcinoma were of equal proportion.
Conclusions: Albinos in Nigeria should be exposed to public health intervention to reduce the incidence of skin cancers through targeted public health educational programmes; structured multicenter and population based research, surveillance, and improved access to healthcare.
Keywords: Albino, Ibadan, Nigeria, plastic surgeon, skin cancer
|How to cite this article:|
Ademola SA. An analysis of skin cancer in albinos in Ibadan. Nigerian J Plast Surg 2015;11:23-8
| Introduction|| |
Skin cancer is the most common type of cancer among Caucasians., In Africa, skin cancers occur less commonly since people there have dark skin but the albinos who lack pigment in their skin are more predisposed to the deleterious effects of ultraviolet ray and tend to present with skin cancers more commonly than the general population. The reason for the increased risk of skin cancers among these nonpigmented Africans is that they lack melanin, the pigment that protects the skin from the deleterious effects of ultraviolet rays, and this predisposes them to actinic skin damage and skin cancers.
It has been posited that there is a growing evidence of social discrimination and stigmatization directed toward albinos., As a result of this social discrimination, they are severely limited in seeking medical care when they have minor skin lesions and tend to present for medical care only when they have grotesque deformities as a result of advanced disease in many parts of sub-Saharan Africa.
The prevalence of albinism in Nigeria has been reported to be 1:15,000. When one considers that the population of the country is over 150,000,000 and the proximity of the country to the equator, there is no doubt that a great proportion of the individuals in the country are at great danger of developing skin cancers.
This observation, coupled with the analysis of the difficulty that albinos have in accessing healthcare as stated earlier and the advanced state of skin lesions that we encounter in these individuals suggest that albinism in Nigeria is a public health concern.
The aims of this paper, therefore, is to present skin cancers in albinos in the eye of the plastic surgeon, sensitize the public to the magnitude of dangers that albinos are exposed to, highlight the challenges faced in their management in a setting with limited resources, and suggest strategies for improved outcomes in them.
| Materials and Methods|| |
All patients with clinical diagnosis of skin cancers presenting to a major tertiary center in Nigeria were referred to the plastic surgery division of the hospital for evaluation. The chart, operation records, and cancer registry records of all albinos referred to this division between 1989 and 2009 were retrospectively reviewed.
The data that were extracted included the demographic data; information about the onset and duration of lesion, number of lesions, local extent, and type of treatment received, duration of follow-up; and the histology report.
The data retrieved were analyzed using descriptive statistics with the aid of Statistical Package for Social Sciences (SPSS) version 20 (SPSS-Inc., Chicago, US).
| Results|| |
The data of 19 patients with 59 skin lesions were retrieved. There were 13 males and 6 females with male: female ratio of 2.2:1. The mean age of the patients was 33.6 [standard deviation (SD) 12.8] years with the youngest patient being 15 years and the oldest being 67 years. However, 74% of the patients were below the age of 40 years. Fifty percent of the patients were not married, over 90% were Christians and 44% were unemployed. More than 30% of those who are employed are engaged in low-income-generating jobs [Figure 1].
Only 20% of the patients presented to the hospital within 1 year of the onset of lesion. The mean duration of lesion before presentation was 21.3 months (SD 13.4). Many of the patients have multiple lesions before they present[mean 3.1 (SD 2.0)] and more than half of the patients (58%) have between three and eight lesions at the time of presentation [Table 1].
Many of the lesions were quite large at the time of presentation, the mean of the widest diameter being 8.8 cm. Majority of the lesions were ulcers while some started as swellings that later ulcerated. A few of them presented with swellings alone.
The majority of the lesions were on the part of the body that is usually exposed to sunlight with the head and neck being the most affected parts of the body [Table 2]. The lesions were on the face in 84.2% of the patients while scalp and cervical lesions were seen in 36.8% and 31.6% of the patients, respectively. In 73.7% of the patients, the lesions were advanced with bony, orbital, nodal, or intracranial involvement.
Over 31.6% of patients had wide excision; 31.6% had a combination of excision, radiotherapy, and chemotherapy; 10.6% had palliative radiotherapy; and 26.3% defaulted with no treatment. Histology of the specimens removed at surgery revealed that there were equal proportion of basal cell carcinoma and squamous cell carcinoma.
It is significant to note that the number of Muslims were low in our patient population [Figure 2].
| Discussion|| |
Albinism refers to a group of genetic disorders that is characterized by lack of pigmentation of the skin, hair, and eyes due to a reduction or absence of melanin production. Albinism could be ocular or oculocutaneous. Oculocutaneous albinism (OCA) encompasses a heterogeneous group of genetic conditions with an autonomic recessive inheritance, and is characterized by hypopigmentation of the skin, hair, and eyes due to a reduced or complete lack of cutaneous melanin in skin. It is this variant (OCA) of albinism that predisposes the skin to actinic damage and subsequently skin cancers. In Africa, individuals with albinism, otherwise called albinos are easily recognized and are at great risk. They have sandy colored hair, white chalky skin, and light brown or blue eyes and are more predisposed to the deleterious effects of ultraviolet rays.
Lack of melanin in the skin of albinos makes them vulnerable to other medical and social challenges. While the medical challenges have direct impact on the health of the individuals, the social challenges are equally important as they are likely to influence their ability to protect themselves from health hazards and access healthcare when necessary. These conditions range from skin disorders (arising from the damaging effects of ultraviolet rays on the skin), ocular problems (as a result of poorly developed visual axis due to lack of pigment), and associated congenital anomalies.
The spectrum of skin disorders in albinos includes sunburns, spontaneous skin blisters, solar keratosis, ulcers, and cancers.
A review of our patient population in Ibadan shows that a vast majority of the patients (74%) are in their first four decades of life. These age groups constitute the young and economically active age group. Their challenges therefore may have severe limitation on their ability to generate income for themselves, their family, and society and have adverse impact on the economy of the nation. That albinos in Africa develop skin cancer at an early age have been commonly reported.,,, In all these reports, more than half of the patients were below 40 years. Previous reports  and the general belief that albinos die young may be related to the early onset and progression of skin cancers in them as well as due to other society-related issues that are discussed later in this paper. Data from this review show that at least one third of the individuals have more than three lesions within the first four decades of life, and half of the patients in our study would have developed advanced cancer within this same time.
This study did not review mortality in the patients. However, the high frequency of advanced disease at an early age may lay credence to the likelihood of high mortality. Traditionally, albinos were not expected to live up to the age of 40 years in some settings; however, with awareness campaigns and sun protection, they live for a considerably longer time. These reports, coupled with our findings, underscore the importance of early use of devices that protect against ultraviolet rays.
Only 20% of the patients in our review presented to the hospital within 1 year of onset of skin lesions. Early intervention allows for simpler surgical intervention and prospect of cure [Figure 3] while presentation to the hospital with advanced disease requires more complicated procedures for locoregional control of disease, need for adjuvant therapies, less likelihood of cure, and higher cost of treatment as depicted in [Figure 4].
|Figure 3: Example of relatively early skin cancer as seen in our service|
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|Figure 4: Example of advanced cancer with nodal and/or contiguous structure involvement|
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It is instructive to note that the time from onset to presentation in hospital was not influenced by the employment status of the patient in this study as almost the same proportion of people who are employed and those who were not employed presented to us late in the course of the disease. We are unable to completely rule out the lack of funds as a reason for late presentation for medical care because majority of those who were employed were engaged in low-income-generating jobs and as such may not have been able to afford the cost of treatment. Medical services in the setting of this study are catered for largely by out-of-pocket payment for services. Ability to provide enough funds by patients therefore has impact on the ability of the patients to come for treatment. In related studies on albinos in Africa, late access to treatment have been attributed to lack of funds., There may however be other issues that delay their contact with specialist care. We established in this review that more than half (57%) of the patients had treatment in some other hospital before accessing specialist care in our institution. Out of this number, only one third came to us within 1 year of onset of disease. The large majority still did not present early. We were not able to determine the length of time they were with the primary care physician before referral to specialist care. However, this is enough indication to advocate that physicians of first contact with albinos should have a low threshold for referring them for specialist care particularly when they have skin lesions so as to ensure that definitive diagnosis is made promptly and the necessary treatment is instituted early.
In agreement with previous reports, the head and neck region, followed by the upper limbs, were the most commonly affected part of the body in this study. These areas are the most commonly exposed to ultraviolet rays from sunlight as they are frequently not covered with clothing. Use of wide brimmed hats and long sleeved shirts could reduce the occurrence of cancers in these regions of the body. We however observed one patient who had the lesion on the posterosuperior aspect of the neck and it extended to the upper back. This appears to be an uncommon site for development of cancers that is reported to occur in the anterosuperior part of the chest in the few cases that have truncal involvement.
Presence of skin cancers on the trunk my also not be as rare as previously reported. A report from Tanzania  found a significantly high number of lesions on the trunk in histological analysis of biopsies from albinos. It is thought that adherence to wearing protective clothing may be less in some African regions because of the hot weather conditions and farming conditions. These may be important factors for African albinos who are relatively less educated and as such often settle for outdoor jobs. The current effects of global warming may also further worsen the difficulty with wearing protective clothing due to their uncomfortable nature.
Wide surgical resection with adequate margin is the mainstay of treatment of nonmelanoma skin cancers but when the disease is locally advanced or there is contraindication to surgery, adjuvant radiotherapy will be required. One third of our patient population had wide surgical resection and wound coverage with skin graft or flap. Another third required postoperative radiotherapy and chemotherapy because of the extent of the disease. A further quarter had unresectable cancers and radiotherapy was the only modality appropriate for their treatment. This underscores the advantage of early medical intervention in those who have developed skin lesions so as to increase the prospect of cure.
Histological evaluation of the lesions in our series revealed that squamous cell carcinoma and basal cell carcinoma were reported in equal proportion. Squamous cell carcinoma had been noted to be the commonest skin cancer in patients of African descent who develop skin cancers.,,,,
It also been reported that although squamous cell carcinoma is still the commonest tumor in Africans, the proportion of basal cell carcinoma appeared to be increasing over the years. Our report further supports the observation that basal cell carcinoma may not be as rare as it was thought to be among Africans especially among the albino population.
There are a number of social issues that our results have brought up. These issues are quite germaine and are likely to influence prevention, treatment and treatment outcomes in albinos with skin lesions.
Majority of our patients are either unemployed or are engaged in low-income-generating employment [Figure 1]. This may lay credence to the fact that they are less able to compete favorably with their peers with normal skin pigmentation. It may be because of their level of education or as a result of discrimination against them while seeking employment. This is likely to limit their income-generating capacity, their purchasing power, and ability to pay for their medical care in our setting where healthcare depends largely on out-of-pocket payments.
Many of them were also unmarried. The reason for this is related with the social issues that they frequently encounter. Albinos face considerable problems in relationships and this include interrelationship with the opposite sex. The reasons for the difficulty in getting a spouse may be due to their different skin color, the frequent ulcers that they develop, or the erroneous belief that they are sterile, that they have a short lifespan and that they have been cursed by the gods among other reasons. These misconceptions are firmly rooted in folklore and traditions in many of our societies such that prospective suitor will avoid albinos passionately.
It is worth noting that the number of Muslims were low in our patient population. While the reasons for this are not apparent from our review, this observation coupled with the lower number of females may suggest a relationship between dress codes and protection of albino skin from skin cancer as a result of ultraviolet rays. The large number of males in this study however contradicts this view as the dress codes for males do not differ across religious lines in the setting of this study. A larger study may explain the reason for this observation.
| Conclusion|| |
In conclusion, albinos are a group of individuals who are vulnerable to skin cancers due to the lack of protective pigment melanin in their skin. This vulnerability is further worsened when they live in the tropics where the intensity of the sun is high. The social discrimination that they are subjected to makes them seek medical attentions late in the course of the disease. Lack of epidemiological and clinical data on this group has also limited the ability to have effective strategies that will limit the skin cancer burden in them.
It is imperative that this population of individuals should be targeted for public health intervention to reduce the scourge of skin cancers in them. In targeting this population for intervention, public health educational programs needs to be organized, structured multicenter population based research and surveys needs to be conducted, the albinos themselves have to be educated on protective measures and the use of these measures should be encouraged. It is also important to organize outreach clinics particularly in the remote areas that are far from health facilities and there should be advocacy geared toward the provision of infrastructures that will protect against undue sun exposure in the schools and work places. Governments in Africa should institute policies that will enable albinos access healthcare easily and without cost. With these measures, the burden of skin cancers in albinos will be significantly reduced, and early access to medical intervention will improve the quality of life.
Special thanks to Professor O.M. Oluwatosin for his guidance and permission to include some of his patients in this review; Dr. O.A. Olawoye, Dr. A.O. Iyun, and Dr. A.I. Michael for their support in patient management. Professor C.A. Adebamowo provided inspiration for this review and invaluable advice at the inception of this study.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2]