Papillon-Lefevre disease

MeSH: D010214ORPHA: 678

Overview

Human disease

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Papillon-Lefevre disease, sourced from HPO and Orphanet clinical annotations.

Abnormality of the dentitionSevere periodontitisGingivitisPeriodontitisPalmoplantar hyperkeratosisPalmoplantar keratodermaHypertrichosisHypopigmented skin patchesCigarette-paper scarsArachnodactylyAbnormal fingernail morphologyRecurrent skin infectionsAbnormal nail morphologyRecurrent respiratory infectionsGeneralized hirsutismSparse body hairCerebral calcificationOsteolysisSquamous cell carcinomaMelanomaAtrophy of alveolar ridgesPremature loss of primary teethNeoplasm of the skinNail dystrophyTooth agenesisChronic furunculosisLiver abscessRecurrent cutaneous abscess formationPustule

Classification & Codes

MeSH Code

D010214

Orphanet Code

ORPHA:678
Papillon-Lefevre disease
MeSHD010214
OrphanetORPHA:678
Treatments0 drug(s)
Symptoms on record29 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
Papillon-Lefevre disease | OrphanDrug