Rare dermatological disease management in low income settings

According to the Global Burden of Disease Study 2013 1 , skin diseases are the fourth leading
cause of disability worldwide, yet dermatology is not often a global health priority. Anja & Co.
present “ Recognition and management of congenital ichthyosis in low income setting”, a case
of a 3 week old Gambian female brought to an urban hospital by her mother due to the
abnormal appearance of her skin since birth. “ On examination, her skin showed thick fissured
hyperkeratosis..associated mild eclabium and more pronounced ectropium with difficulty in eye-
closure bilaterally” 2 .

The use of Telemedicine in a setting where “it was the first such diagnosis to be made at this
hospital” 3 proved to a useful tool to access specialists, dertamologists and opthamologists, for
diagnosis consult and treatment plan. In low and medium income countries the access to
specialists and diagnostics tools, such as genetic testing, can be scarce. This case shows the
value of such tool, especially in rare diseases.

Barriers to successful maintenance of care include access to medication, presentation for follow
ups, time availability of a care giver and stigma of disease.

Tailoring a treatment plan to fit individuals according to their socio economic status, locally
available resources, and ability for follow up can increase positive medical outcomes.
Decreasing complications and increasing compliance is an essential for optimal management.
Part of medical care revolves around educating the family and / or society about the disease.“ I
felt very sad after my baby was born. Her skin was very dry and others in the village were
worried she was cursed” “The hospital took care of her and told me why it happened….I’m very
happy the hospital helped me and my daughter” 4 . Traditional beliefs play an important role in
many cultures around the globe and we must work alongside them. The consequence of
negative tradition beliefs can lead to failure to seek medical treatment, social isolation 5 and
neglect of the affected individual, underlying the importance disease education.

This case report sheds light on diagnosing, treating and managing a rare dermatological
disease in a setting with limited resources.

1: JAMA Dermatol. 2017 May 1;153(5):406-412. doi: 10.1001/jamadermatol.2016.5538.
2: Saso A, Dowsing B, Forrest K, et al. BMJ Case Rep 2019;12:e228313. doi:10.1136/bcr-2018-
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3: Saso A, Dowsing B, Forrest K, et al. BMJ Case Rep 2019;12:e228313. doi:10.1136/bcr-2018-
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4: Saso A, Dowsing B, Forrest K, et al. BMJ Case Rep 2019;12:e228313. doi:10.1136/bcr-2018-
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5: Ahogo Kouadio C, Enoh J, Gbery Ildevert P, et al. Lamellar Ichthyosis in SubSaharan Africa:
Social Stigmatization and Therapeutic Difficulties. JAMA Dermatol 2017;153:476–7.