Papillon–Lefevre syndrome in two brothers: Innocuous or dangerous?

Two brothers, born of second-degree consanguineous marriage, presented with syndromic diffuse palmoplantar keratoderma with transgrediens and progrediens, along with generalized severe periodontitis, premature loss of permanent teeth and psoriasiform plaques over the elbows, knees, and gluteal region. There was a history of palmoplantar hyperhidrosis and recurrent skin infections. Based on the clinical picture, a diagnosis of Papillon– Lefevre syndrome was made. Pyogenic liver abscesses, cerebral and renal abscesses, and dural calcification are associated complications, which were not present in our patients. Early diagnosis and multidisciplinary approach (systemic retinoids, meticulous dental care, and dentures) are imperative to improve the prognosis.