{"doi":"10.1111/ijd.16607","title":"Xeroderma pigmentosum: An experience from Zanzibar","abstract":"Zanzibar is a semi-autonomous island located off the coast of Tanzania in East Africa.1 The population is around 1.7 million, according to the last census in 2022. There are two main islands, Unguja and Pemba, with the only referral hospital (Mnazi Mmoja) located on Unguja Island. Zanzibar communities share similar cultures and beliefs as most coastal cities. Intermarriage (consanguinity) is a common practice, especially in Pemba Island, suspecting it to be the main factor associated with the increased number of xeroderma pigmentosum (XP) cases in the community, as was explained in many previous literatures.2 XP is a rare genetic disorder caused by inherited mutations in gene-encoding proteins that play critical roles in nucleotide excision repair (NER) or postreplication repair of DNA damage from ultraviolet (UV) light resulting in extreme sensitivity to sunlight, sunburn, and pigmentary changes in the skin leading to greatly elevated incidence of skin cancer.2, 3 XP has no known therapy and is controlled by self-restraint from direct sunlight. During XP clinics review, sun protective gear should be provided to reduce the risk of skin cancers and loss of sight, that is, long-sleeved clothes, wide-rim hats, sunglasses, vitamin D tablets, and sunscreens with SPF 50 and above. Using these items is a lifelong commitment that parents and caregivers should be aware of and be committed to.3, 4 Here, we present data from the XP clinics collected over 3 years with a gradual increase in patient numbers as awareness and access to services were scaled up. The data were collected through the different outreaches in Pemba Island and the dermatology clinic at Mnazi Mmoja Hospital. Over these 3 years, we conducted three XP outreach clinics on Pemba Island and four on Unguja Island, and we saw 56 patients with XP who were diagnosed clinically. The majority were male (30, 53.6%), with a mean age of 8.7 years ranging from 6 months to 30 years. Most of them were from Pemba Island (44, 78.5%). Micheweni, a district in Pemba Island, was the most concentrated area with 32 (72.7%) XP patients (Table 1). All 56 XP patients had the classic presentation of fine scaling and freckle-like areas of hyperpigmentation (lentigines) and hypopigmentation, giving the “salt and pepper” appearance seen over the face and back of the neck (Figure 1). All XP patients were symptomatic, presenting with photophobia and burning sensation on minimal exposure to the sun, mainly on the face and scalp. Approximately 59.6% of our XP patients had tumors located primarily on chronic sun-exposed areas and the tongue (Figure 2). The tumors on the tongue could be explained by possible repetitive tongue trauma. A significant number of these children (19, 33.9%) died at a very young age; almost all were <10 years of age. Studies suggested skin cancer and neurodegenerative disease as the main culprit.5 However, only one child was diagnosed with autism. In summary, Micheweni has a significant number of patients with XP. This is suggestive of a possible considerable interaction between the families (consanguinity), which points out an important area of research in the future to establish causality. Significant strides have been made in establishing dermatological care in Zanzibar; although there is no dermatologist at Pemba, efforts have been made to conduct outreach services aiming at providing quality health services. To date, this remains a neglected disease in Zanzibar. Hence, more efforts are needed to sustain these XP outreach clinics. Ministry of Health Zanzibar and RDTC for collaboration. In the memory of the late Mohamed Abdalla (epidemiologist) who started this journey with us to establish a sustainable XP program.","journal":"International Journal of Dermatology","year":2023,"id":376506,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":3,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9578,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2023-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1140116,"name":"Abdulrahman Kh. Said","orcid":null,"position":1,"is_corresponding":false},{"id":1140117,"name":"Dhikra I. Haji","orcid":null,"position":2,"is_corresponding":false},{"id":1140118,"name":"Doriane Sabushimike","orcid":null,"position":3,"is_corresponding":false},{"id":817763,"name":"George Semango","orcid":"0000-0002-2212-4202","position":4,"is_corresponding":false},{"id":1140119,"name":"Daudi Mavura","orcid":null,"position":5,"is_corresponding":false},{"id":1140115,"name":"Hafidh S. Hassan","orcid":null,"position":0,"is_corresponding":true}],"reference_count":6,"raw_metadata":null,"created_at":"2026-07-19T01:16:32.368674Z","pmid":"36759963","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}