K E Bozdağ | PubMed Health

PubMed2000

Lipoid proteinosis.

A 21-year-old man presented with a complaint of hoarseness as well as lesions along the eyelids and on his knees, elbows, and fingers. Hoarseness had developed in childhood, followed by lesions along the eyelids and on the elbows and fingers. He had developed lesions on the knees, feet, scrotum, penis, and axilla over the last 2 years. His parents were relatives, but nobody in the family showed similar features. His physical examination was normal. On dermatologic examination, there were beaded papules along the eyelids and small, yellow-white infiltrations on the tongue, buccal mucosa, palate, scrotum, and penis (Fig. 1). His tongue was firm and its mobility was limited. He also had infiltration of the frenulum (Fig. 2) and warty and hyperkeratotic papular and nodular lesions on the hands, knees, elbows (Fig. 3), and axilla. The laboratory findings and laryngoscopic examination were normal. Ophthalmologic, neurologic, and psychiatric examinations were also normal. Electroencephalogram (EEG), electromyogram (EMG), craniography, and cranial computed tomography (CT) scanning revealed no abnormality. The histopathologic examination of the skin biopsy specimens obtained from the axilla, elbow, and dorsum of the hand showed hyperkeratosis and periodic acid-Schiff-positive (PAS(+)) staining hyaline material around the dermal capillaries and sweat glands, and confirmed the diagnosis of lipoid proteinosis.

Adult↗

PubMed2000

Degos' disease.

A 47-year-old woman presented with slightly pruritic, burning lesions on her submammary area and legs of 3.5 years' duration. Similar lesions had appeared on her arms and feet. She was hospitalized at the Pulmonary Diseases Hospital, Izmir, with a complaint of thoracic pain, and was diagnosed with nonspecific pleuritis by pulmonary X-ray, thoracic computed tomography (CT) scanning, bacterial cultures of phlegm, and pleural biopsy examination. Fifteen days after leaving hospital, she was admitted to the Surgical Clinic of Atatürk Training Hospital, Izmir, with a complaint of severe abdominal pain, and diagnosed with nonspecific peritonitis. On dermatologic examination, she had multiple, scattered papules (2-3 mm in diameter), with a typical, central, white, porcelain-like zone of atrophy, on the trunk (Fig. 1), legs (Fig. 2), and arms. The lesions were encircled by a slightly raised erythematous border. Some of the lesions had coalesced giving polycyclic atrophic areas and ulcerations. Histopathologic examination of the skin biopsy specimen showed hyperkeratosis, epidermal atrophy, dermo-epidermal separation, edema, and necrosis in the papillary dermis (Fig. 3). Fibrinoid necrosis and thrombosis were seen in the papillary dermis and in the vessels below the lesions. The patient was diagnosed with Degos' disease from these clinical and histopathologic findings. She was treated with anti-inflammatory drugs. One year later, the patient was hospitalized at the Department of Internal Medicine of Atatürk Training Hospital, Izmir, with a diagnosis of pericarditis and pleuritis.

Diagnosis, Differential↗