Cutaneous metastasis of uterine papillary serous carcinoma.

Uterine papillary serous carcinoma (UPSC) is an uncommon highly malignant variant of endometrial adenocarcinoma that histologically and clinically resembles papillary serous carcinoma of the ovary. This case demonstrates a very rare case of cutaneous metastasis of uterine papillary serous carcinoma. A 54-year-old Korean female developed multiple pruritic skin nodules on the pubic area 13 months later after diagnosis of uterine papillary serous carcinoma. A biopsy of the skin lesions showed papillary serous carcinoma, compatible with her primary tumor. Without clinical history, it is difficult to distinguish other types of metastatic carcinoma to the skin and primary apocrine carcinoma of the skin from metastatic uterine papillary serous carcinoma. Whereas uterine papillary serous carcinoma only rarely involves the skin, this entity should be included in the differential diagnosis of papillary adenocarcinoma in the skin.

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).

Failure to detect clonality in eosinophilic pustular folliculitis with follicular mucinosis.

Eosinophilic pustular folliculitis is characterized by an eosinophil-rich inflammatory follicular and perifollicular infiltrate primarily centred at the level of the follicular isthmus and sebaceous duct. Follicular mucinosis has been observed in lesions of eosinophilic pustular folliculitis. Clinical and histological features of eosinophilic pustular folliculitis with follicular mucinosis and alopecia mucinosa are very similar. Alopecia mucinosa may be a clonal T-cell dermatosis. A monoclonal re-arrangement of the T-cell receptor gene was detected in about half of the cases in alopecia mucinosa. To investigate T-cell clonality in a series of eosinophilic pustular folliculitis with follicular mucinosis, we performed heteroduplex analysis of re-arranged T-cell receptor gamma gene in seven cases of eosinophilic pustular folliculitis with follicular mucinosis. All cases were negative for heteroduplex-PCR analysis. The failure to demonstrate clonality may be consistent with a reactive nature of eosinophilic pustular folliculitis with follicular mucinosis.

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. CLINICAL CASE: A 40-year-old-woman presented with 5 x 4 cm, erythematous, indurated, non-movable, painful mass on the pubic area. Histopathology demonstrated diffuse tumor-cell infiltration into the subcutaneous and fascia, which was consisted of prominent epithelioid cells and scattered rhabdoid cells. A multinodular growth pattern or granulomatous appearance with central necrosis was not observed. The tumor cells showed positive reactions for vimentin, cytokeratin (AE1/AE3), and CD34. Despite the surgery, left inguinal mass with lymphadenopathy occurred one month later. We also carried out comparative genomic hybridization (CGH) with tumor cells. CGH revealed chromosomal gain of 5q32-qter, 12q24-qter, and 22q. CONCLUSION: We report a case of proximal-type of epithelioid sarcoma, which showed the chromosomal gains of 5q32-qter, 12q24-qter, and 22q by CGH.