Kee Chan Moon

Clinicopathologic Analysis of 66 Cases of Erythema Annulare Centrifugum

Erythema annulare centrifugum (EAC) denotes a group of eruptions characterized by slowly migrating annular or configurate erythematous lesions. It can be classified histopathologically as the deep or superficial type. Although there are many case reports of EAC associated with various underlying conditions, no recent clinicopathologic studies exist. The purpose of this study was aimed at analyzing the clinical and histopathologic features of EAC. Sixty‐six patients who have been diagnosed as EAC by clinical and histopathological examination were collected. The medical records and skin biopsy specimens of these patients were reviewed retrospectively. There were 24 male patients and 42 female ones. The mean age was 39.7 years, and the mean durtion of the disease was 2.8 years. The lower extremities, particularly the thigh, were the most frequently involved locations. The most common clinical manifestation was large (>1 cm), scaly, erythematous, indurated plaques. Forty‐eight patients (72%) had combined diseases including cutaneous fungal infection (48%), such as tinea pedis, other skin diseases (18%), internal malignancies (13%), and other systemic diseases (21%). By histopathologic examination, 33 of 42 patients (78%) were identified as superficial type, and 9 patients (22%) were deep type. Therapeutic trials with systemic or topical corticosteroid and oral antihistamine did not affect the chronic and recurrent course of these patients. EAC is a chronic and recurrent disease despite treatment. EAC is thought to be highly associated with internal disease as well as with superificial fungal infection. However, it was difficult to prove a causal association. The recognition and exact diagnosis of EAC is important, because it may be a quite stressful condition and lead to unnecessary over‐treatments.

Toxic epidermal necrolysis: analysis of clinical course and SCORTEN-based comparison of mortality rate and treatment modalities in Korean patients.

Toxic epidermal necrolysis (TEN) is a rare, life-threatening, drug-induced cutaneous reaction. We herein report our experience regarding causes, clinical course, treatment and sequelae of TEN in Korean patients. In addition, we used the SCORTEN, a severity-of-illness score for TEN, to compare the predicted and actual mortality rates, and to evaluate the efficacy of treatment modalities. A retrospective study of 38 patients with TEN during a 13-year period (1990-2003) at the Asan Medical Center was performed. The mean involved body surface area was 49+/-17%. All except three cases were associated with medications, most commonly antibiotics, followed by non-steroidal anti-inflammatory drugs, acetaminophen and herbal remedies. Fourteen patients had a history of current infection, including upper respiratory infection, pneumonia and herpes simplex infection. The mean time from initial drug administration to the onset of TEN was 9.8+/-5.7 days. Twenty-one patients were treated with systemic corticosteroids. Fourteen received high dose intravenous immunoglobulin therapy. The actual mortality rate was 23.7% (9/38), not significantly different from the SCORTEN-predicted rate (25.5%, 9.699/38). Also based on SCORTEN, treatment with high dose intravenous immunoglobulin showed a trend to lower actual mortality than predicted mortality (standardized mortality ratio (SMR) = 0.425; 95% CI, 0.011-2.368), whereas corticosteroid therapy showed no such difference (SMR = 1.004; 95% CI, 0.369-2.187).

Cutaneous metastasis from large-cell neuroendocrine carcinoma of the urinary bladder expressing CK20 and TTF-1.

Large-cell neuroendocrine carcinoma (LCNEC) of the urinary bladder is very rare. Immunohistochemical and biochemical examinations have shown that neuroendocrine carcinomas (NECs) have features of neuroendocrine and epithelial differentiation. We describe the first case of cutaneous metastasis from LCNEC of the urinary bladder. The patient had been treated with partial cystectomy and chemotherapy for LCNEC of the urinary bladder, but a year later, he visited our clinic with a reddish mass on his scalp that was diagnosed as a cutaneous metastasis from LCNEC. The tumor cells were positive for the neuroendocrine markers, cytokeratin (CK) 20 and thyroid transcription factor-1. Most NECs, except for Merkel cell carcinomas, do not express CK20, whereas most urothelial carcinomas do express CK20. These results suggest that a histogenetic link may exist between NEC of the urinary bladder and urothelial carcinoma.

A Case of Primary Cutaneous Cd56+, Tdt+, Cd4+, Blastic Nk-cell Lymphoma in a 19-year-old Woman

The classification of blastic or blastoid natural killer (NK)-cell lymphoma is controversial. Reports of primary cutaneous blastic CD56+ NK-cell lymphoma are rare, which necessitates further clinicopathologic definition of this type of lymphoma. Most CD56+ lymphomas display angiocentric histologic features, especially in Asian patients, and these are mostly associated with the presence of Epstein-Barr virus (EBV) genome and with an aggressive clinical course. We report on a young woman with a primary cutaneous blastic NK lymphoma which showed no angiocentric features but showed an unusual immunophenotype; CD56+, TdT+, CD4+, EBV-, and germline configuration of T-cell receptor gene. This unusual lymphoblastic lymphoma seems to have an immature or progenitor NK cell lineage.

Cutaneous extranodal natural killer/T-cell lymphoma: A comparative clinicohistopathologic and survival outcome analysis of 45 cases according to the primary tumor site

BACKGROUND Differences in survival outcomes and prognostic factors of cutaneous extranodal natural killer/T-cell lymphoma (ENKTL) depending on primary tumor site are currently unknown. OBJECTIVE We sought to analyze the clinicopathological features and survival outcomes of cutaneous ENKTL according to primary tumor site. METHODS In all, 45 patients with cutaneous ENKTL were classified with: (1) primary cutaneous ENKTL, or (2) nasal ENKTL with cutaneous involvement. Clinicopathologic features, survival outcomes, and prognostic factors were analyzed using patients medical records. Survival outcomes were analyzed using the Kaplan-Meier method and compared using the log rank test. The Student t test, Fisher exact test, and linear by linear association test were used to analyze clinicopathologic differences between groups. RESULTS Clinical manifestations of cutaneous ENKTL included solitary or multiple subcutaneous nodules and cellulitis or abscess-like lesions. Primary cutaneous ENKTL demonstrated a less aggressive clinical course and better survival outcomes. The extent of cutaneous lesions demonstrated a significant effect on the prognosis of primary cutaneous ENKTL, but not on nasal ENKTL with cutaneous involvement. The presence of nasal lesions in primary cutaneous ENKTL was associated with poor prognosis. LIMITATIONS This study used a retrospective design and included a small sample size. CONCLUSION Although the clinicopathological features were similar regardless of subgroup, survival outcomes and prognostic factors differed depending on the primary tumor site of cutaneous ENKTL.

Prurigo pigmentosa: clinicopathological study and analysis of 50 cases in Korea.

Prurigo pigmentosa is a recurrent dermatosis with severe pruritus and several peculiar clinical features. Its exact etiology and pathogenesis are unclear. The aim of this study was to investigate the clinical features and chronological changes in the histopathology of prurigo pigmentosa in Korean patients and to assess the etiology of this condition. We reviewed the medical records, clinical photographs and biopsy specimens from 50 patients diagnosed with prurigo pigmentosa. Mean age at diagnosis was 23.7 years (range, 15–61 years). Prurigo pigmentosa started as urticarial papules or plaques, changing first to papulovesicles and then to reticulated brownish macules. The most frequent sites were the back and chest, especially depressed areas such as the central back and inter‐mammary area. Dietary change was suspected as a cause of prurigo pigmentosa in 17 patients. Histopathologically, early‐stage lesions had dermatitis herpetiformis‐like features; fully‐developed lesions displayed impetigo‐like or acute, generalized, exanthematous, pustulosis‐like features; and late lesions presented with post‐inflammatory hyperpigmentation‐like features. Oral minocycline, with or without dapsone, was effective in inhibiting the appearance of new lesions, but did not prevent recurrence. Prurigo pigmentosa is not rare in Korea, is apparently associated with dietary modification and preferentially involves the depressed regions of the trunk.

Subcutaneous panniculitis-like T-cell lymphoma: a clinical and pathologic study of 14 korean patients.

Background Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a distinctive skin lymphoma characterized by neoplastic T-cell infiltration of the subcutaneous tissue, mimicking panniculitis. Objective To describe the clinical and pathologic features of SPTL in Korean patients. Methods Fourteen SPTL patients evaluated over 15 years were retrospectively reviewed. Results The mean patient age was 35 years (range: 7~73 years), with male predominance (2.5:1). Most patients presented with either nodules or plaques, occurring most commonly on the trunk, with two patients (14%) having hemophagocytic syndrome. Histopathologically, all patients showed infiltrates of small-to-medium pleomorphic cells mimicking panniculitis, with some also showing rimming, bean-bag cells, and fat necrosis. Most patients were positive for CD3 (14/14), CD8 (12/13), TIA-1 (9/9) and βf1 (5/5), but were negative for CD4 (11/12), CD20 (8/8), CD56 (14/14) and Epstein-Barr virus (8/8). Ten patients (71%) received chemotherapy and 2 (14%) died due to the disease, with an average survival time of 4 months. Survival analysis did not reveal any significant prognostic factors. Conclusion This is the first series of patients with SPTL in Korea. Due to its indolent clinical course and relatively high survival rate, SPTL should be differentiated from cutaneous γδ T-cell lymphoma.

Treatment of Elastosis Perforans Serpiginosa with the Pinhole Method Using a Carbon Dioxide Laser

Elastosis perforans serpiginosa (EPS) is a rare perforating disorder characterized by transepidermal elimination of abnormal elastic fibers. EPS skin lesions present as hyperkeratotic papules with a serpiginous or arcuate distribution, usually occurring on the neck or back. These lesions may trigger emotional distress in the patient if they appear on a conspicuous site, such as the neck. We report the treatment of EPS on the neck in a 37-year-old woman using the pinhole method with a carbon dioxide (CO2) laser.

Metaplastic Ossification in a Cutaneous Pyogenic Granuloma: A Case Report

Cutaneous ossification may occur in association with a variety of cutaneous neoplasms and inflammatory conditions, such as pilomatricomas, basal cell carcinomas, nevi, chondroid syringomas, venous stasis, and scars. However, it has rarely been reported in pyogenic granuloma, a relatively common benign vascular tumor of the skin and mucous membranes. We herein presented a rare case of cutaneous pyogenic granuloma with ectopic ossification on the big toe of a 37‐year‐old man, with high recurrence despite repeated CO2 laser ablations. We propose the hypothesis that vascular endothelial growth factor (VEGF) and bone morphogenetic proteins (BMPs) play pathologic roles in the development of ectopic bone formation in pyogenic granuloma.

Proximal-type epithelioid sarcoma: case report and result of comparative genomic hybridization

Background:  Epithelioid sarcoma is a rare mesenchymal neoplasm. Recently, a more aggressive, so‐called ‘proximal type’ epithelioid sarcoma has been described.