Late-onset Papillon-Lefevre syndrome with pyogenic liver abscesses: report of one case

A 25-year-old woman living in Hamedan, Iran, presented originally at 7 years of age with erythematous, hyperkeratotic lesions on the palms and soles with extension to the dorsal side of the hands and feet. Involvement of the elbows and knees was also seen. From 12 years of age, she started to lose her teeth. At the same age, she experienced fever, chills, malaise, myalgia, and right upper quadrant abdominal pain. With a diagnosis of pyogenic liver abscesses, the patient underwent successful surgical treatment. Examination revealed erythematous, hyperkeratotic, scaling plaques on the palms and soles, dorsal side of the hands and feet (Fig. 1), elbows and knees. All the teeth were missing from the mouth (Fig. 2), and she used a dental prosthesis. A surgical scar was observed on the right upper quadrant of the abdomen (Fig. 3). Skull X-ray and computed tomography scan were normal. Skin biopsy of the dorsal right hand demonstrated hyperkeratosis, focal parakeratosis, hypergranulosis, and acanthosis with a mild inflammatory infiltrate around the vessels (Fig. 4). Figure 1. Hyperkeratotic plaques on the hands and feet Download figure to PowerPoint Figure 2. Loss of permanent teeth Download figure to PowerPoint Figure 3. Surgical scar on the right upper quadrant of the abdomen and hyperkeratotic plaques on the hand Download figure to PowerPoint Figure 4. Hyperkeratosis, focal parakeratosis, hypergranulosis, and acanthosis (hematoxylin and eosin, ×40) Download figure to PowerPoint