Dermatology Online Journal
Skin cancer in albinos at the University of Calabar Teaching Hospital, Calabar, Nigeria
- Author(s): Asuquo, M E
- Otei, O O
- Omotoso, J
- Bassey, E E
- et al.
Letter: Skin cancer in albinos at the University of Calabar Teaching Hospital, Calabar, Nigeria1. Department of Surgery
M E Asuquo1, O O Otei1, J Omotoso2, E E Bassey2
Dermatology Online Journal 16 (4): 14
2. Department of Pathology
University of Calabar Teaching Hospital, Calabar, Nigeria. firstname.lastname@example.org
We evaluated all the albinos with a histologic diagnosis of skin cancer seen in the University of Calabar Teaching Hospital, Calabar, Nigeria, during the 7-year period (January 2001 to December 2007). The ages, sex, clinical presentation/site(s), number of lesions per patient, type of skin cancer, treatment, outcome, and follow up were analyzed. This was compared with the total number of skin cancers. Of the skin cancers diagnosed, 11 percent were in patients with albinism.
Skin cancers are the most frequently diagnosed cancers in the United States. Of these, approximately 80 percent are basal cell carcinoma (BCC) and 20 percent squamous cell carcinoma (SCC) . The dark skin individuals of African descent are said to be far less likely than those with light skin to develop skin cancer . Oculocutaneous albinism is an established risk factor for some skin cancers in black Africans [3, 4]. It is genetically heterogeneous hypopigmentation. One of the two major autosomal recessive forms involves the tyrosinase gene (OCA1), whereas the other major form (OCA2) has recently been associated with alterations of the P gene on chromosome 15. OCA 2 is about twice as common as OCA1 in African and African-American populations . It presents as 2 phenotypes depending on the presence or absence of pigmented patches or ephelides [3, 6]. The biosynthesis of melanin pigment is reduced in the skin, hair, and eyes . Because of the lack of melanin, people with albinism are more susceptible to the harmful effects of ultraviolet radiation exposure, resulting in issues such as photophobia, decreased visual acuity, extreme sun sensitivity, and skin cancer . The head is the site most commonly affected and SCC has been found to be far more common than BCC in Africans [3, 9, 10]. High levels of exposure to ultraviolet radiation increase the risk of all the three major forms of skin cancer . We present a study of skin cancer in nine albinos in an attempt to evaluate the age and sex pattern, type of skin cancer, site of lesion, possible risk factors, and outcome of treatment in our region.
We evaluated all the albinos with a histologic diagnosis of skin cancer seen in the University of Calabar Teaching Hospital, Calabar, during the 7-year period (January 2001 to December 2007). The ages, sex, clinical presentation/site(s), number of lesions per patient, type of skin cancer, treatment, outcome, and follow up were analyzed. This was compared with the total number of skin cancers. Approval was obtained from the ethical committee for the study of skin cancers seen in our facility.
The population of Calabar, the capital of Cross River State (with a population of 3 million) is 400,000. The number of albinos in our population has not been reported. Eighty-two blacks were observed with skin cancer between January 2001 and December 2007. The 9 albinos observed during the period of study accounted for 11 percent of skin cancers. The ages of the 5 males and 4 females ranged from 21 through 60 (mean=34.7 years). Five patients presented with BCC, 5 had SCC, and one patient had melanoma. Out of these, two females had both BCC and SCC. (Table 1, Nos. 2 and 4). The five BCCs and 4 SCCs wereobserved onthe head and neck region. One patient each with BCC and melanoma had lesions located on the upper limb. Two cases of SCC were located on the upper limb and one BCC was located on the exposed upper part of the anterior chest wall.
The patients presented late with ulcers/fungating fleshy lesions within periods that ranged between 6 and 18 months (mean 9 months). There was no regional lymphadenopathy except in the patient with melanoma. All the patients were serologically negative for human immune deficiency virus (HIV). Secondary infection was sometimes found and the common isolates were Staphylococcus aureus, Pseudomonas, and Proteus. We did not have the ability to determine the number of patients that were OCA1 or 2.
The treatments, outcomes, and follow up are as shown on Table 1. The 7 BCC lesions were excised; the wounds healed and follow-up ranged from 6 months to 4 years. The outcomes of the SCC lesions excised, including those who had adjuvant cytotoxic chemotherapy, were poor with evidence of residual tumors (from inadequate excision) after surgery in 4 patients (Table 1). There was, however, a satisfactory result in a patient with SCC thatinvolved the left side of the face/ear managed by excision and cytotoxic chemotherapy, (methotrexate and cisplatin). At 18 months in this patient, the site of the tumor located on the left facial region had healed with no evidence of recurrence. However, he represented with an advanced right facial SCC 4 months ago and this led to a fatal outcome despite excision, chemotherapy, and radiotherapy (Table 1). The only patient with melanoma developed a recurrence 3 months after excision and had cytotoxic chemotherapy before he was lost to follow up (Table 1).
Oculocutaneous albinism is an established risk factor for some skin cancers in black Africans [3, 4]. In sub-Sahara Africa, the prevalence of albinism ranges from as low as 1:15,000 in east central state, southern Nigeria to as high as 1:1000 in the Tonga tribe in Zimbabwe . The estimated prevalence of albinism suggests the existence of tens of thousands of people living with albinism in Africa . The prevalence of albinism in our region has not been reported. The 9 albinos managed for skin cancer accounted for 11 percent of total skin cancers. Kromberg et al. , reported that 23.4 percent of albinos developed skin cancer out of 111 albinos studied in South Africa. In Cameroon, 271 patients with OCA were studied over 15 years with the highest prevalence in the Bamileke group (1 in 7900) thought to relate to high consanguinity .
In this study the mean age was 34.7 years; 5 patients were in the 3rd decade and 2 in the 6th decade. Ademiluyi and Ijaduola  reported that black patients presented between the 3rd and 4th decades, whereas the albinos presented a decade earlier. However, our study is at variance with the report by Yakubu and Mabogunje (northern Nigeria) , in which albinos seldom live more than 30 years. Three of our patients were in the 5th and 6th decades (Table 1). The relatively advanced age of these patients may be related to the lower cumulative solar exposure in the southeastern equatorial rain forest and possibly ethnicity .
Globally, the prevalence of melanoma in albinism remains relatively rare with about 30 cases in documented in the literature . Yakubu and Mabogunje in Zaria-northern Nigeria , George et al. in southwestern Nigeria , Datubo-Brown in Port Harcourt (south-south Nigeria) , and Kromberg in southern Africa , reported the absence of melanoma in their studies and highlighted the rarity of this tumor in albinos. Luande et al. in Tanzania reported SCC in 29 out of 33 patients with one melanoma and three BCC patients . We recorded a patient with melanoma observed on the right forearm, an uncommon site for this lesion in Africans; the common site is the soles of the feet in the black populations . This patient presented late with an advanced tumor beyond curative surgery.
The most common site was the head and neck, followed by the upper limb, which highlights the possible role of solar radiation as a risk factor. This is in keeping with other studies [3, 4]. No cancers were found on the trunk except the exposed upper part of the anterior chest wall and the lower limb, a fact highlighted in another study .
Merkel cell carcinoma and 10 BCCs have been described in a female HIV infected African albino; chemotherapy and radiotherapy were temporarily successful . None of our patients was proven to have HIV infection. Wound infection was treated by antimicrobials based on sensitivity and we used honcrivine (honey and acriflavine) for dressing. Honey plays 3 major roles, which include antimicrobial activity (contains inhibine), sloughing (enhances granulation tissue formation), and epitheliazation promotion .
Excision was generally effective and the outcomes of the BCCs were good;the lesions had healed without evidence of recurrence after a range of 6 months and 4 years of follow-up. Most patients with SCC and melanoma presented with advanced fungating lesions beyond curative surgery. There was one fatality in a patient with SCC.
Lookingbill et al. , studied 164 albinos in northern Tanzania and detected actinic cheilitis, actinic keratoses, and skin cancers in many patients. They concluded that sun protection methods are very important for the prevention of skin damage in albinos. Okoro AN , noted that the sun and society were hostile to albinos and recommended protective clothing, sun-screening agents, indoor occupations, and early treatment of actinic keratoses and skin cancer. Most of our patients presented late. We recommend that the public receive education on early institution of preventive measures. They should be encouraged to seek care early. Close follow-up is also required to improve the skin cancer outcomes and decrease the health care cost of skin cancers in albinos.
Albinism and solar radiation are risk factors for skin cancer. Skin cancer in albinos is common by the 3rd decade and additional skin cancer development is expected. Public health education about prevention, early presentation, and follow up should be encouraged in this population.
References1. Gross ND, Monroe M. Skin cancer: Squamous cell carcinoma updated Jan 15, 2009
2. Hahn SB, Kim DJ, Jeon CH. Clinical study of Marjolin’s ulcer. Yousei Med J 1990; 31: 234-241. [PubMed]
3. Kromberg JG, Castle D, Zwane EM, Jenkins T. Albinism and skin cancer in Southern Africa. Clin. Genet 1989; 36(1): 43-52. [PubMed]
4. Yakubu A, Mabogunje OA. Skin cancer in African albinos. Acta Oncol. 1993: 32(6): 621-622. [PubMed]
5. Durham-Pierre D, Gardner JM, Nakatsu Y, King RA, Francke U, Ching A, Aquaron R, del Marmol V, Brilliant MH. African origin of an intragenic deletion of the human P gene in tyrosinase positive oculocutaneous albinism. Nat Genet 1994; 7(2): 176-179. [PubMed]
6. Kedda MA, Steorens G, Manga P, Viljoen C, Jenkins T, Ramsay M. The tyrosinase positive oculocutaneous albinism gene shows locus homogeneity on chromosome 15q11-q13 and evidence of multiple mutations in south African negroids. Am J Hum Genet 1994; 54(6): 1078-1084. [PubMed]
7. Spritz RA, Fukai K, Holmes SA, Luande J. Frequent intragenic deletion of the P gene in Tanzanian patients with type11 oculocutaneous albinism (OCA2). Am J Hum Genet 1995; 56(6): 1320-1323. [PubMed]
8. Hong ES, Zeeb H, Repacholi MH. Albinism in Africa as a public health issue. BMC Public Health. 2006; 6: 212. [PubMed].
9. Datubo-Brown D D. Primary malignant skin tumours in Nigerians. J. Natl. Med Assoc. 1991 Apr; 83(4): 345-348. [PubMed]
10. Yakubu A, Mabogunje O A. Skin Cancers in Zaria. Trop. Doct. 1995; 25 suppl. 1: 65-67. [PubMed]
11. Glanz K, Saraiya M. Using evidence-based community behavioural interactions to prevent skin cancer. Opportunities and challenges for Public Health Practice. Prev Chronic Dis 2005 April 2(2) [serial online].
12. Aquaron R. Oculocutaneous albinism in Cameroon. A 15-year follow up study. Ophthalmic Paediatr Genet 1990; 11(4): 255-263. [PubMed]
13. Ademiluyi S A, Ijaduola G T. Occurrence and recurrence of BCC of the head and neck in Negroid and Albinoid Africans. J. Laryngol Otol. 1987 Dec; 101(12): 1324-11328. [PubMed]
14. Terenziani M, Spreafico F, Serra A, Poddam M, Cereda S, Belli F. Amelanotic melanoma in a child with oculocutaneous albinism. Med Paediatr Oncol 2003; 4: 179-180. [PubMed]
15. George AO, Ogunbiyi AO, Daramola OOM, Campbell OB. Albinism among Nigerians with malignant melanoma. Trop Doc 2005; 35: 55-56. [PubMed]
16. Luande J, Henschke CI, Mohammed N. The Tanzanian human albino skin. Natural history. Cancer 1985; 55(8): 1823-1828. [PubMed]
17. Colebunders R, Bottieau E, Van den Brande J, Colpaert C, Van Marck E. Merkel cell carcinoma and multiple basal cell carcinoma in an African albino woman with HIV infection. HIV Med. 2004; 5(6): 452-454. [PubMed]
18. Efem SEE. Clinical observations of wound healing properties of homey Br J Surg 1988; 75: 679-681. [PubMed]
19. Lookingbill DP, Lookingbill GL, Leppard B. Active damage and skin cancer in albinos in northern Tanzania: findings in 164 patients enrolled in an outreach skin care programme. J Am Acad Dermatol 1995; 32(4): 653-658. [PubMed]
20. Okoro AN. Albinism in Nigeria. A clinical and social study. Br J Dermatol 1975; 92(5): 485-492. [PubMed]
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