Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2019 Aug 20;12(8):e228313.
doi: 10.1136/bcr-2018-228313.

Recognition and management of congenital ichthyosis in a low-income setting

Anja Saso  1   2 , Benjamin Dowsing  2 , Karen Forrest  3 , Mary Glover  4
Affiliations
Case Reports

Recognition and management of congenital ichthyosis in a low-income setting

Anja Saso et al. BMJ Case Rep. .

Abstract

We report the case of a 3-week old girl in The Gambia who presented to hospital with an undiagnosed skin disorder evolving since birth. Using telemedicine to seek specialist dermatology advice abroad, she was diagnosed with and managed for suspected congenital lamellar ichthyosis. Poor early recognition and limited resources, for both acute and chronic care, created significant challenges to optimal management; these were overcome, in part, by adopting a common sense, back-to-basics approach to treatment and by empowering the parents to take ownership of their infant's daily skin and eye care. This case highlights key global health issues associated with managing chronic, often debilitating, paediatric dermatological conditions in a low-income setting; namely, poor access to important diagnostic tools and medications, lack of experience and expertise in the management of severe skin disease and its associated complications, absence of long-term community support, alternative health beliefs and risk of sociocultural stigma.

Keywords: congenital disorders; dermatology; global health; ophthalmology; paediatrics.

PubMed Disclaimer

Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Hyperkeratosis, scaling and fissuring of the skin of a baby with suspected congenital ichthyosis.
Figure 2
Figure 2
Facial manifestations of congenital ichthyosis: ectropium and eclabium.
See this image and copyright information in PMC

References

    1. Mazereeuw-Hautier J, Vahlquist A, Traupe H, et al. . Management of congenital ichthyoses: European guidelines of care, part one. Br J Dermatol 2019;180 10.1111/bjd.17203 - DOI - PubMed
    1. Mazereeuw-Hautier J, Hernández-Martín A, O’Toole EA, et al. . Management of congenital ichthyoses: European guidelines of care, part two. Br J Dermatol 2019;180 10.1111/bjd.16882 - DOI - PubMed
    1. Richard G. Molecular genetics of the ichthyoses. Am J Med Genet C Semin Med Genet 2004;131C:32–44. 10.1002/ajmg.c.30032 - DOI - PubMed
    1. Ahogo Kouadio C, Enoh J, Gbery Ildevert P, et al. . Lamellar Ichthyosis in Sub-Saharan Africa: Social Stigmatization and Therapeutic Difficulties. JAMA Dermatol 2017;153:476–7. 10.1001/jamadermatol.2016.5792 - DOI - PubMed
    1. Amoako Johnson F, Padmadas SS, Matthews Z. Are women deciding against home births in low and middle income countries? PLoS One 2013;8:e65527 10.1371/journal.pone.0065527 - DOI - PMC - PubMed

Publication types