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Arch Dermatol. 2005;141:779-784.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

Diagnosis: Papillon-Lefèvre syndrome.

MICROSCOPIC FINDINGS

Histologic examination of the biopsy specimen showed hyperkeratosis, acanthosis, and dilated blood vessels in dermal papillae.

DISCUSSION

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.

The gene responsible for Papillon-Lefèvre syndrome can be found on chromosome 11q14-q21, specifically in the interval D11S4082-D11S931, and encodes cathepsin C.^1-2 The cathepsin C gene encodes a cysteine lysosomal protease . . . [Full Text of this Article]

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Arch Dermatol. 2005;141(6):779.
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