Clinicopathologic Manifestations of 36 Korean Patients with Acute Generalized Exanthematous Pustulosis: A Case Series and Review of the Literature

BACKGROUND: Acute generalized exanthematous pustulosis (AGEP) is a rare and severe subtype of drug eruption, characterized by acute, extensive, non-follicular, sterile pustules on an erythematous background, accompanied by fever and leukocytosis. OBJECTIVE: The purpose of this study was to characterize AGEP in Korean patients in terms of clinical, laboratory, and pathologic findings. METHODS: Thirty-six patients (M:F=17:19) with AGEP were identified from an extensive review of medical records over a 15 year period. All patient cases were confirmed by biopsy and fulfilled the diagnostic criteria. RESULTS: The patient ages ranged from 4~80 years (37.6+/-19.4). The incubation period was 1~23 days. The duration of disease was 5~14 days. Neutrophilia (36/36), high CRP (14/36), and eosinophilia (30/36) were common laboratory findings. A history of drug administration existed in 23 of 36 patients; herbal medications, lacquers and radiocontrast media were the unique causative drugs. Spongioform subcorneal or intraepidermal pustules in the epidermis was observed in all patients. Thirty-six patients were subdivided into 2 groups: group A (n=23) was strongly associated with known agents; and group B (n=13) had no identified causative agents. There was no significant difference between the 2 groups. CONCLUSION: Our results demonstrate the characteristic features of AGEP in Korean patients as follows: lower identification of causative agents; herbal medications, lacquers, and radiocontrast media were the main causative agents; and no significant differences existed between the 2 groups.

Congenital Eccrine Poroma on the Left Temple / 대한피부과학회지

Eccrine poroma is a benign, rare cutaneous neoplasm that clinically represents as solitary, reddish and pink papule or nodule chiefly on the soles or sides of the feet. But it can occur anywhere wherever sweat glands are found. Eccrine poroma generally arises in the middle-aged and elderly people and do not usually exceed 20 mm in diameter. To the best our knowledge, no congenital cases of eccrine poroma have been reported in the Korean literature. We, herein, report a rare case of eccrine poroma arising on the left temple in a 45-year-old man since birth.

A Case of Lymphomatoid Keratosis

Lymphomatoid keratosis (LK) is considered to be a rare variant of cutaneous lymphoid hyperplasia, with epidermotropism. We herein report a case of LK which developed on the abdomen of an elderly Korean woman. A 60-year-old woman presented with a 10-year history of a pruritic, solitary, brown to black plaque on the abdomen. Histopathologically, the specimen showed hyperkeratosis, parakeratosis, acanthosis and Pautrier's micro-abscess in the epidermis, and a lichenoid infiltration of lymphocytes in the dermis, which expressed both B cell and T cell lineage on the immune-histochemical staining. Based on these clinical and histopathological findings, our case was diagnosed as LK. To our knowledge, this is the first case report of LK in the Korean dermatologic literature.

Prevalence of Atopic Dermatitis among Korean Adults Visiting Health Service Center of the Catholic Medical Center in Seoul Metropolitan Area, Korea

The point prevalence of atopic dermatitis (AD) among Korean adults visiting the Health Service Center for health check-ups was assessed. AD was diagnosed based on the questionnaires filled by patients and through physical examination by dermatologists. A total of 3,563 persons completed the questionnaires, and 2,032 persons were examined by the dermatologists. According to the questionnaires, the prevalence of AD was 7.1%, and from the dermatologists' examination, it was 2.6% overall, which decreased with age significantly. The prevalence in men was higher than that of women. Grading the severity of AD according to their eczema area and severity index scores, 70.6% were classified as being mild, 25.5% moderate, and 3.9% severe. Interestingly, concomitance of psoriasis and AD was found in 0.5% of those examined by the dermatologists. Our results suggest that AD is one of the most common skin diseases not only in children but also in adults in Korea.

Two Cases of Sequential Composite Lymphoma / 대한피부과학회지

It is uncommon that two phenotypically different lymphomas develop in the same person, and especially in the skin. Composite lymphoma is defined as two distinctly demarcated types of non-Hodgkin's lymphoma (NHL) or its association with Hodgkin's lymphoma (HL) within a single organ or tissue. Discordant lymphoma is defined as two types of NHL or the association of HL with NHL at different anatomic sites in the same person. Sequential lymphoma and simultaneous lymphoma are defined as two different types of lymphoma that occur in the same person at different times and at the same times, respectively. We herein report on two cases of sequential composite lymphomas that were limited to the skin. A 57-year-old woman who had had been diagnosed with HL at the face had been treated with chemotherapy for 1.5 years. 6 months later, the patient revisited the dermatologic clinic of our hospital for a one-month history of erythematous nodules on her chest and upper extremities. Punch biopsy was performed and the pathologic diagnosis was mantle cell lymphoma. She refused further treatment. A 68-year old woman, who had been diagnosed as having extranodal marginal zone B cell lymphoma, had been treated with chemotherapy and radiation therapy for one year. After one year, she revisited dermatologic clinic of our hospital for a several-month history of erythematous nodules on her extremities. Punch biopsy from the lesion was performed and the pathologic diagnosis was cutaneous diffuse large B cell lymphoma, the leg type. She was scheduled for chemotherapy (rituximab with cyclophosphamide, adriamycin, vincristine and prednisolone (R-CHOP)). To the best of our knowledge, our cases are the first such cases in the Korean dermatologic literature.

Two Cases of Elephantiasis Neurofibromatosa Treated with Surgery / 대한피부과학회지

Elephantiasis neurofibromatosa is the clinical subtype of neurofibromatosis and this clinically represents as a large, ill-defined, soft mass with soft tissue overgrowth and hairy pigmented patches. Histopathologically, an infiltrating and well-demarcated mass that is not encapsulated and the mass is composed of proliferating spindle-shaped cells can be observed in the dermis and subcutis. Mitotic activities should not exist. We herein report on two cases of elephantiasis neurofibromatosa that were treated with surgery. Both patients had had difficulty in walking due to a huge mass that had been on their left legs for more than 20 years. After surgery, they reported improvement of their ambulation.

A Case of Giant Chondroid Syringoma on the Nose / 대한피부과학회지

Chondroid syringoma is rare, benign tumor of the sweat gland. It clinically presents as intradermal or subcutaneous nodule on the head and neck, measuring 5 to 30 mm in diameter. Giant chondroid syringoma can be defined as chondroid syringoma that exceed 30 mm at the point of greatest diameter if it occurs on the head and neck area. Most chondroid syringomas on the head and neck do not exceed 30 mm in diameter. We herein, report a case of giant chondroid syringoma of the nose in an 87 year-old woman.