Histologic examination of the biopsy specimen showed hyperkeratosis, acanthosis, and dilated blood vessels in dermal papillae.
Papillon-Lefèvre syndrome is a rare genodermatosis, affecting 1 to 4 persons per million. No sex or racial predominance has been detected. Papillon-Lefèvre syndrome belongs to a group of disorders of keratinization known as palmoplantar keratodermas. It is generally thought to be inherited in an autosomal recessive manner, although our case had an unusual inheritance pattern (paternal grandmother to father to son) for that mode of transmission. It is characterized by redness and thickening of the palms and soles, psoriasiform plaques of the elbows and knees, and periodontosis, resulting in loss of deciduous and permanent teeth. It is frequently associated with cutaneous and systemic pyodermas.
Psoriasiform Plaques and Periodontal Infection—Diagnosis. Arch Dermatol. 2005;141(6):779–784. doi:10.1001/archderm.141.6.779-e