Non-melanoma skin cancer in the context of albinism with an associated facial nerve palsy

Abstract

Introduction and importance

Albinism, or the loss of pigment formation by otherwise healthy melanocytes, is quite common in Sub-Saharan Africa and is most likely caused by consanguinity. The sun's ultraviolet (UV) radiation and albinism have been both related to non-melanoma skin cancer (NMSC). Non-melanoma skin cancer (NMSC) and melanoma are the two main types of skin cancer. NMSC accounts for the large majority of all skin cancers, they are located in parts of the body that are chronically exposed to sunlight, such as the head, face, neck, and dorsum of the hand.

Case presentation

We present a case of NMSC in a female person with albinism (PWA) presenting with a left facial ulceration and right facial deviation accompanied by intense pain.

Clinical discussion

The face is home to several vital structures including the facial nerve, which when affected by a tumoral aetiology, albeit uncommon could lead to facial nerve palsy having consequences such as facial appearance changes with potential negative psychosocial effects adding to the weight of the tumour.

Conclusion

Increase awareness of NMSCs and its risk factors, as well early diagnosis and treatment is of the essence and can help reduce morbidity and mortality.

Highlights

• •Presentation of an extensive left facial non melanoma skin cancer lesion in a person with albinism.
• •We present a person with albinism with an associated grade V House Brackmann left facial palsy and intense pain
• •Sub Saharan Africa has the world’s highest rate of albinism
• •Non-melanoma skin cancer are linked to UV exposure which is abundant in this region
• •Increasing awareness on non-melanoma skin cancer especially in person with albinism will help prevent such cases

1. Introduction

Non-melanoma skin cancer (NMSC) alongside melanomas are the two major types of skin cancers. Non-melanoma skin cancers are the more frequent type and photoexposed body regions, especially the head and neck regions are the sites of predilection [1].

Albinism includes a group of disorders, characterized by little or no melanin production, which affects the skin, hair, and eyes coloration, and is inherited in an autosomal recessive manner [2]. Africans are more likely to develop cutaneous cancers because they live near the equator where the exposure to ultraviolet radiation of the sun is very high and more so in people with albinism where the reduction or absence of the protective skin pigment melanin, predisposes them to sunburns and subsequent dysplastic skin changes which may later progress to malignant transformation [3]. In PWA, as well, NMSCs are more represented with melanomas being rare [4,5].

The facial nerve or cranial nerve 7 is a mixed nerve that includes the motor, sensory, and parasympathetic nerve fibers [6] which control the muscles of facial expression, taste perception in the anterior two thirds of the tongue, and parasympathetic supply to the salivary and lacrimal glands [7]. Also, the facial nerve has a complex anatomy and exits the skull through the stylomastoid foramen, an opening near the parotid gland.

Facial nerve palsy, the most frequent cranial nerve palsy, can be unilateral or bilateral with a tumoral aetiology being uncommon, accounting for less than 5 % [8]. This condition induces facial appearance changes and facial function changes as well [9] potentially leading to negative psychosocial effects in the affected patients.

Cutaneous skin cancer with perineural invasion as well as infratemporal, extratemporal, and skull base malignancies, are examples of occult malignant causes of facial nerve palsy [7].

2. Case presentation

A 33-year-old female with albinism presented with a left facial ulceration that had been present within the last three years involving the oral cavity (Fig. 1), accompanied by intense pain and a facial deviation to the right (Fig. 2) present at rest with barely perceptible mouth movement. Also present was a left lagophthalmos (inability to close her eye) and absence of the left nasolabial fold (Fig. 1). The patient reported no personal past history of cancer and no family history of cancer as well. The rest of her history was unremarkable.

Fig. 1.

Frontal view showing left facial paralysis accompanied with right facial deviation, an impaired left eye closure and an absence of the left nasolabial fold.

Fig. 2.

Exposure of left salivary glands as seen by of drooling of saliva mixed with blood over her garment.

The patient sought self-medication and traditional medicine as a remedy and the patient didn't seek medical help until her condition became severe and the pain became intolerable. The diagnosis retained was a left facial Non melanoma skin cancer with an associated secondary left facial nerve palsy (Grade V according to the House Brackmann classification). The patient was in continual search of an antalgic posture. We gave her intravenous analgesics to relieve her pain and transferred her to the oncology department for further therapy. Unfortunately, we received no feedback and lost contact with the patient.

The lesion undoubtedly touched the facial nerve due to its proximity to the stylomastoid foramen (Fig. 3), resulting in this facial deviation. Due to lack of financial means no work ups could be carried out.

Fig. 3.

Lateral view showing extensive lesion's proximity to the stylomastoid foramen.

3. Discussion

Cameroon is located in Sub-Saharan Africa, with significant sunlight exposure throughout the year, and UV radiation from sunlight is a known risk factor for developing NMSC [8].

We have shown that in Cameroon, skin cancer knowledge is lower than in other population-based studies [10], and our patient's delayed presentation to a healthcare facility further demonstrates a lack of knowledge about NMSCs and the risk factors associated with them in our local context.

Our patient was from the West region (Bamiléké group), which has been shown to have the highest prevalence of skin cancers [1] and PWA [11] which could be due to high inbreeding or heterozygote advantage of the carrier [11]. In Cameroon, the number of NMSC cases has doubled over the last decade and this is due to in part to the increase in diagnosis capabilities [1]. Worldwide, the incidence of NMSC has increased from 1,951,299 (1990) to 6,353,687 (2019), so too has the Disability adjusted life years (DALYs) due to NMSC. Worryingly, population expansion and aging are predicted to cause a future increase of this incidence and DALYs [12].

Pertaining to age, our patient was 33 years old which is consistent with previous research on PWA [1], however a sharp decrease of NMSCs has been observed in the older patients [1,11],which could be attributed to greater skin cancer death rates [11,13].

This left unilateral facial nerve palsy presentation we observed could be caused by local compression, direct infiltration or by paraneoplastic process of the facial nerve by this tumour [14]. Furthermore, facial nerve involvement is determined by the anatomical course of the nerve and tumour location, with our lesion situated in proximity to the extracranial opening of the facial nerve [14] which is the stylomastoid foramen.

In terms of psychological impact, facial palsy has the potential to have a detrimental influence on psychosocial welfare and quality of life, as evidenced by withdrawal from social activities, anxiety, poor body image, and depression. This may be more pronounced in women due to cultural influences such as increased societal emphasis on female appearance [9,15]. Another possible explanation for this negative impact is a decreased capacity to depict facial expressions, which are essential in good social interactions and communication [9]. Furthermore, as albinism is a genetic disease, genetic counseling to PWA and their families will help curb misconceptions [16], reduce stigma and the negative psychological influence and hence encouraging them to be more proactive in skin cancer surveillance.

The severe pain and distress the patient was experiencing could be explained by pain due to perineural invasion [17].

4. Conclusion

Given the high prevalence of albinism in Sub-Saharan Africa, as well as the region's year-round exposure to high levels of sunlight and UV radiation, which are known risk factors for developing NMSC, sensitization on this pathology, its risk factors and simple preventive measures, as well as early diagnosis and treatment, are critical to reducing patient morbidity and mortality.

Protective clothes, sun-screening products, and avoiding outdoor professions can all help to mitigate the sun's harmful effects. Regular examinations of PWA for early diagnosis and treatment of premalignant or malignant lesions should be emphasized and carried out in an anti-cancer facility.

This case report has been reported in accordance with the SCARE criteria [18].

Ethical approval

Ethical approval for this study (Number 218/UY1/FMSB) was obtained from the Institutional Review Board of the Faculty of Medicine and Biomedical Sciences of the University of Yaoundé, Cameroon on the 14th February 2020.

Funding

None.

Author contribution

Authors	Conception and design of study/review/case series	Acquisition of data: laboratory or clinical/literature search	Analysis and interpretation of data collected	Drafting of article and/or critical revision	Final approval and guarantor of manuscript
NGOKWE	NGOKWE	NGOKWE	NGOKWE	NGOKWE	NGOKWE
Ntep	Ntep	Ntep	Ntep
NOKAM	NOKAM	NOKAM
KOUAMOU	KOUAMOU	KOUAMOU
BENGONDO		BENGONDO	BENGONDO	BENGONDO

Guarantor

Dr. Zilefac Brian Ngokwe.

Registration of research studies

None.

Informed consent

The authors obtained written consent from patients for their photographs and medical information to be published in print and online and with the understanding that this information may be publicly available. Patient consent forms were not provided to the journal but are retained by the authors.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.