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Palmoplantar keratoderma with progressive gingivitis and recurrent pyodermas
Tyler A Moss, Anne P Spillane1, Sam F Almquist
1Department of Dermatology, Kimbrough Ambulatory Care Center, 2480 Llewellyn Ave, Ste 5800, Fort Meade, MD 20755-5129, USA. anne.p.spillane.mil@mail.mil.
Papillon-Lefèvre syndrome (PLS) is a rare genetic disorder causing palmoplantar keratoderma, gingivitis, and skin infections. This review details a case study and discusses PLS pathophysiology, diagnosis, treatment, and related palmoplantar keratoderma syndromes.
Area of Science:
- Dermatology
- Genetics
- Oral Medicine
Background:
- Papillon-Lefèvre syndrome (PLS) is a rare autosomal-recessive disorder.
- It is characterized by palmoplantar keratoderma (PPK), severe periodontitis leading to premature tooth loss, and recurrent pyoderma.
- PLS results from mutations in the CTS1 gene, affecting keratinocyte differentiation and immune function.
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