Erdheim-Chester Disease

Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis of unknown etiology, characterized by multi-organ involvement. ECD is usually diagnosed on the basis of characteristic radiologic and histopathological findings. Lesions may be skeletal and/or extraskeletal in location, and may include the skin, lung, heart, and central nervous system. We describe here a 68-year-old man with multiple yellowish plaques and a pinkish nodule on his face and scalp. He had been previously diagnosed with diabetes insipidus, and recently complained of coughing and dyspnea. Imaging studies showed multiple osteosclerotic lesions of the bones, a moderate amount of pericardial effusion, and multifocal infiltrative lesions in the perirenal space. Histopathological examination of the skin lesions revealed dermal infiltration of foamy histiocytes with multinuclear giant cells. Moreover, laparoscopic biopsy of the perirenal tissue revealed fibrosis with infiltrating foamy histiocytes being CD68-positive and S100-negative. Based on these findings, he was diagnosed with ECD with extraskeletal manifestations, and treated with interferon-alpha.

A Case of Metastatic Cellulitis Caused by Stenotrophomonas maltophilia / 대한피부과학회지

Stenotrophomonas maltophiliais a nonfermentative Gram-negative bacillus, and this is an emerging pathogen for nosocomial infection. It is commonly associated with pneumonia and catheter-related bacteremia, and especially in immunocompromised patients. Cutaneous infection with Stenotrophomonas maltophilia is uncommon, yet it may cause therapeutic problems because of its intrinsic resistance to common antibiotics and the immunodeficiency of the affected host. Herein, we report on a case of metastatic cellulitis secondary to Stenotrophomonas maltophilia bacteremia, and this developed in an immunocompromised patient. He was successfully treated with intravenous trimethoprim-sulfamethoxazole.

A Case of Stucco Keratosis / 대한피부과학회지

Stucco keratosis is a clinically distinct variant of seborrheic keratosis, which presents exclusively on the lower legs as white-gray keratotic warty papules. When the lesion is removed, a peripheral collarette of scale remains without bleeding. We report a case of stucco keratosis on the dorsum of the feet and ankles of a 79-year old man. Pathologic findings showed characteristic epidermal hyperplasia of the "church spire" pattern with basket-weave hyperkeratosis. He was treated with topical urea and lactic acid for 2 months and showed improvement.

A Case of Calcifying Aponeurotic Fibroma in an Adult / 대한피부과학회지

Calcifying aponeurotic fibroma is an unusual, but well-characterized soft tissue tumor that typically involves the extremities. The age at presentation ranges from birth to 64 years of age. However, the majority of cases are seen in children with a median age of 12 years. Histologically, this tumor shows a poorly circumscribed, fibrotic mass extending into fat with areas of dense hyalinization, fibrosis and scattered calcification. Due to its infiltrative growth, calcifying aponeurotic fibroma has a high rate of local recurrence after surgical treatment. We recently encountered a 53-year-old Korean man with a solitary, hyperkeratotic, subcutaneous nodule on the sole of his foot. After histopathologic examination, the lesion was diagnosed as calcifying aponeurotic fibroma. During the follow-up period for 3 months, there was no increase in size of the lesion.