Solitary Malignant Gastrointestinal Stromal Tumor Associated with a Neurofibromatosis Type I

From the Department of Dermatology, College of Medicine, The Catholic University of Korea, Kangnam St. Mary's Hospital, 505, Banpo-Dong, Seocho-Gu, 137-040, Seoul, Korea, Gastrointestinal stromal tumors are usually late manifestations of neurofibromatosis (von Recklinghausen's disease) and most become clinically apparent in middle-aged patients as multiple benign tumors. To our review of the literature, solitary malignant stromal tumor of gastrointestinal tract is exceptionally rare in von Recklinghausen's disease. We herein present a case of solitary jejunal stromal tumor in a 50-year-old woman with NF1, which histopathologically showed a malignant change and combined smooth muscle-neural type.

Two Cases of Acute Generalized Exanthematous Pustulosis / 대한피부과학회지

Acute generalized exanthematous pustulosis(AGEP) is characterized by the abrupt onset of widespread pustules on an erythematous base and rapid spontaneous healing. Most cases appear to be related to drug reactions, mainly antibiotics, viral infections, and hypersensitivity to mercury. We report two cases of acute generalized exanthematous pustulosis which presented with widespread tiny pustules on the whole body. Histopathologic examinations of both cases showed subcorneal neutrophilic pustules with perivascular polymorphous cellular infiltration. We suspected that the possible cause of two cases was inhalant mercury and carbamazepine.

A Case of Oncocytoma / 대한피부과학회지

Parotid oncocytoma is a benign salivary gland neoplasm that represent approximately 1% of all salivary gland tumors. Histopathologically, this condition consists of oncocytes, which are polygonal cells with abundant granular eosinophilic cytoplasm, ultrastructually showing an increased number of mitochondria. We herein report a typical case of oncocytoma of the parotid gland in a 73-year-old woman who presented a 2X2 cm, reddish firm nodule on the right postauricular area for 3 years.

A Case of Solitary Cutaneous Reticulohistiocytoma Occurring on the Tragus / 대한피부과학회지

Solitary cutaneous reticulohistiocytoma is characterized by a solitary reddish nodule or papule confined to the skin without evidence of arthritis or underlying systemic illness. We report a case of solitary cutaneous reticulohistiocytoma in a 41-year-old female, who presented with an asymptomatic, firm, dome-shaped nodule on the tragus of left ear. Histopathologically, numerous multinucleated giant cells with a ground glass appearance was shown in the dermis. Immunohistochemical stains of CD68 was positive. The lesion was completely excised and no evidence of recurrence was observed.

A Case of Lyme Disease Presenting with Erythema (Chronicum) Migrans / 대한피부과학회지

Lyme disease (Lyme borreliosis) is a vector-borne infection primarily transmitted by Ixodes species and caused by a spirochete, Borrelia burgdorferi. This disease affects multiple areas such as skin, joints, eyes, nervous and cardiovascular systems. Erythema (chronicum) migrans, borrelial lymphocytoma and acrodermatitis chronica atrophicans are the cutaneous hallmarks of Lyme disease. A 31-year-old man presented with an annular centrifugally expanding erythematous plaque with central punctum on the left scapular area for about 2 months. He was bitten by a tick 3 months ago in Kangwondo. Serologic examinations revealed increased levels of Ig M and Ig G antibodies to borrelial antigen in consecutive tests performed at an interval of three weeks. Western blot for Borrelia burgdorferi was also positive. The skin lesion showed prompt response to the tetracycline therapy and was cured after 2 weeks.

A Case of Keratosis Punctata of the Palmar Creases

Keratosis punctata of the palmar creases (KPPC) is rare skin condition characterized by punctiform hyperkeratotic pits confined to the palmar and digital creases. Although this condition has been regarded as a variant of classical punctate keratoses, there are some differences between classical punctate keratosis and KPPC. We herein report a case of KPPC in a 22-year-old man who had numerous, tiny, hyperkeratotic pits limited to the palmar creases of both hands with typical histologic findings.

A Study about the Skin and General Disease Pattern of the Vietnam Veterans Exposed to Dioxin / 대한피부과학회지

BACKGROUND: Vietnam veterans have suffered from a number of skin and general diseases. But in Korea, there are few reports about the relationship between dioxin and skin disease and dermatologists need more information about it. OBJECTIVE: The purpose of this study was to evaluate the skin and general disease pattern of Koreans who have a history of exposure to dioxin in Vietnam. METHODS: 332 Vietnam veterans who visited St. Mary's hospital between July, 2001 and September, 2001. We perfomed a clinical evaluation, physical examination, medical history, serum and urine chemistry, electrocardiogram, electrodiagnostic study, and a roentgenographic study. For dermatologic evaluation, pathologic examination, KOH mount, fungus culture, and phototest were performed. RESULTS: 1. Among the 332 participating soldiers in the Vietnam war, the mean age of patient was 56.9 years old (the youngest-53 years, the oldest-61 years old). 2. The prevalence of hypertension, hyperlipidemia, diabetes mellitus, liver disease, and peripheral neuropathy of subjects was 12.0%, 6.9%, 5.7%, 4.5%, and 1.2%. 3. The prevalence of xerotic eczema, seborrheic dermatitis, psoriasis vulgaris, photoallergic dermatitis, and chronic urticaria of subjects was 3.9%, 3.9%, 0.9%, 0.6%, and 0.3%. CONCLUSION: The prevalence of general diseases was not significant between Vietnam veterans and controls.

A Case of Solitary Fibrofolliculoma / 대한피부과학회지

Fibrofolliculoma is a rare benign follicular neoplasm histopathologically characterized by central dilated follicular infundibulum surrounded by well-circumscribed, thick mantle of fibrotic tissue, with anastomosing strands of follicular epithelium extending into the stroma. This condition usually occurs in multiple and rarely solitarily. Herein we report on a 34-year-old man with a solitary, skin colored, bean sized nodule on his chin which histopathologically shows a typical fibrofolliculoma.

A Case of Aplasia Cutis Congenita / 대한피부과학회지

Aplasia cutis congenita(ACC) is a rare skin condition in which localized or widespread areas of skin are absent at birth. Clinically, lesions usually appear circular or oval, sharply outlined ulcer, resulting in healing with scarring. The most common site of this condition is scalp, especially on the vertex although other areas of the body may also be involved. Other congenital malformations have been associated with aplasia cutis congenita. Herein we report a 6-year-old male infant with round, well-marginated, smooth surfaced, brownish scar about 2 cm diameter on the vertex of scalp. The lesion was erythematous, crusted ulcer at the time of birth. Histopathologic finding of the lesion showed flattening and thinning of epidermis, fibrosis in the dermis and the absence of adnexal structures except single immature hair follicle.

Two Cases of Type V Hyperlipoproteinemia and Eruptive Xanthomas associated with Diabetes Mellitus

We report two cases of type V hyperlipoproteinemia and eruptive xanthomas associated with diabetes mellitus in a 44-year-old woman and a 25-year-old woman. They had asymptomatic, diffuse, erythematous yellowish papules on the extremities and buttocks. The laboratory studies showed increased serum blood glucose, total cholesterol, triglyceride, increasing pre-β and chylomicron bands on electrophoresis of lipoprotein. Histopathologic findings of erythematous yellowish papules on the thigh and buttock revealed aggregation of foam cells in the dermis. They have been treated with diet control, hypoglycemic agent (gliclazide) and hypolipidemic drug (gemfibrozil), and are under continued supervision.