Excellent response to adalimumab in a patient with Papillon–Lefèvre syndrome: A case report

Author:

Almukhadeb Eman1ORCID,Alanazi Wael2,Alshareef Rahaf3,Altukhaim Feras1ORCID,Albalbeesi Amal1

Affiliation:

1. Department of Dermatology, College of Medicine King Saud University Riyadh Saudi Arabia

2. King Salman Specialist Hospital Hail Saudi Arabia

3. General Directorate of Research and Studies Ministry of Health Riyadh Saudi Arabia

Abstract

AbstractPapillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disease that presents clinically with palmoplantar keratoderma and periodontitis and results in early‐onset dental loss. PLS management is difficult. We report a 30‐year‐old female with PLS who had unfavourable response to topical keratolytic creams, oral isotretinoin and narrow‐band ultraviolet‐B (NB‐UVB) phototherapy but showed significant clinical improvement to adalimumab.

Publisher

Wiley

Reference13 articles.

1. Deux cas de kératodermie palmaire et plantaire symmétrique (maladie de Meléda) chez le frère et la soeur. Coexistence dans les deux cas d'altérations den‐ taires graves;Papillon MM;Bull Soc Fr Dermatol Syphiligr,1924

2. Papillon- Lefevre Syndrome: Report of a case and its management

3. Loss-of-function mutations in the cathepsin C gene result in periodontal disease and palmoplantar keratosis

4. Mutations of the cathepsin C gene are responsible for Papillon-Lefèvre syndrome

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1. Biologics for inherited disorders of keratinisation: A systematic review;Australasian Journal of Dermatology;2023-12-21

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