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 betaf1 (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 gammadelta T-cell lymphoma.

A Case of Tumor of the Follicular Infundibulum with Sebaceous Differentiation

Tumor of the follicular infundibulum (TFI) is an uncommon benign adnexal tumor that usually presents as a solitary keratotic papule on the face or scalp of elderly patients. Histopathologically, it typically manifests as a plate-like fenestrated proliferation of monomorphic pale-staining cells. A 76-year-old male presented with about a 2 cm, well-defined, yellowish to brownish, slightly elevated, twisted and bent, interrupted, cord-like plaque on his left lower abdomen. Microscopic examination revealed a sharply demarcated plate-like proliferation of pale cells localized in the papillary dermis with multiple connections to the overlying epidermis. The histopathological features were compatible with TFI, except for foci of sebaceous differentiation. There has been one previous case report of TFI with sebaceous differentiation in the English medical literature. Herein, we report on a singular case of TFI with sebaceous differentiation.

A Case of Erythrodermic Mycosis Fungoides Treated with Gemcitabine / 대한피부과학회지

Erythrodermic mycosis fungoides (MF) denotes a condition in which erythroderma occurs in patients with otherwise typical clinical features of MF. Gemcitabine is a novel treatment regiment for MF. A 16-year-old boy presented in the hospital with skin lesions that showed generalized erythema with scaling, several plaques, and nodules with ulceration on the extremities and trunk. He was diagnosed with erythrodermic MF. His lesions did not improve with methotrexate (7.5 mg/week) and PUVA for 6 months, so his treatment regimen was changed to systemic gemcitabine therapy (1,000 mg/m2 per week). After 6 cycles of gemcitabine, his skin lesions disappeared. We report a case of erythrodermic MF treated by gemcitabine.

Cutaneous Metastasis of Choriocarcinoma / 대한피부과학회지

Choriocarcinoma is one of the malignant tumors of trophoblastic cells characterized by secretion of human chorionic gonadotrophin (hCG). Choriocarcinoma often metastasizes to the lung, liver, and brain but skin metastasis is rare. To our knowledge, only one case of cutaneous metastasis was reported in Korea. A 35-year-old female presented with hemoptysis for 1 month. Biopsy from lung nodules revealed metastatic choriocarcinoma. She was subsequently referred to our clinic for evaluation of a rapidly growing, reddish nodule with bleeding tendency on the scalp. Histologic examination demonstrated biphasic pattern of cytotrophoblasts and hCG-positive syncytiotrophoblasts with multiple foci of hemorrhage - typical histologic features of choriocarcinoma. The patient was diagnosed with metastatic choriocarcinoma. We herein report a case of choriocarcinoma with cutaneous metastasis.

Role of CD4(+)CD25(high+)FOXP3(+) Regulatory T Cells in Psoriasis

BACKGROUND: CD4(+)CD25(high+)regulatory T cells (Tregs) are considered to be of vital importance for maintaining immunologic self-tolerance and preventing autoimmune diseases. These cells have been found to be deficient in skin lesions and in the peripheral blood of patients with psoriasis. OBJECTIVE: To investigate the role of Tregs in the pathogenesis of psoriasis and to evaluate the changes in Tregs in relation to the severity and the clinical course of psoriasis. METHODS: Immunohistochemistry (CD3, 4, 8, 79 and FOXP3) was performed in 22 psoriatic patients compared to 5 normal controls. Flow cytometry (CD3, 4, 8, 25 and FOXP3) was performed in 18 psoriatic patients and 8 normal volunteers and reverse transcriptase polymerase chain reaction (foxp3 mRNA) was performed in 8 psoriasis patients. RESULTS: An increase in the FOXP3(+) cell fraction was detected in the lesional psoriatic skin irrespective of the severity of psoriasis as compared with the normal skin. However, a decrease in FOXP3(+) cells was observed in the samples obtained from psoriasis of 'acute course'. FOXP3(+) Treg populations in the blood of the 'acute course' psoriasis was not different compared to that of 'chronic course' psoriasis and normal controls. CONCLUSION: The deficiency of FOXP3(+) Tregs in the lesional psoriatic skin might be responsible for the exacerbation of psoriasis.

A Case of Cutaneous and Systemic Plasmacytosis with an Unusual Clinical Presentation / 대한피부과학회지

Cutaneous and systemic plasmacytosis is a rare disorder that's characterized by a cutaneous plasma cell infiltrate accompanied by polyclonal hypergammaglobulinemia. Clinically, it appears as multiple red-brown plaques that mainly occur on the trunk, and this is sometimes associated with variable extracutaneous involvement. All the cases of cutaneous and systemic plasmacytosis reported in Korea have shown the typical skin manifestations on the trunk of the patients. Herein, we report on a case of cutaneous and systemic plasmacytosis and the patient presented with multiple erythematous to brownish patches on the face, neck and both axillary areas.

A Case of Rudimentary Meningocele / 대한피부과학회지

Rudimentary menigocele is a developmental anomaly in which menigothelial elements are present in the skin and subcutaneous tissue. A 9-year-old boy presented with a well-circumscribed, central atrophic and alopecic patch on the midline of the occipital area, and this lesion had been present at birth. Histological examination showed dense collagen bundles with pseudovascular spaces lined by oval, spindle shaped cells in the dermis and the subcutaneous layer. Collagen bodies and psammoma body were also detected. On the immunohistochemistry, these oval, spindle shaped cells were positive for vimentin, but negative for CD31, EMA and S100 protein.

A Case of Incontinentia Pigmenti Associated with Multiorgan Abnormalities

Incontinentia pigmenti is a systemic disorder affecting the skin, teeth, eyes, nervous tissue, hair, nails, musculoskeletal system, and heart. We describe an 11-month-old girl with incontinentia pigmenti associated with a ventricular septal defect, left hemiatrophy, hemangiomas, an abnormal labial frenum, and spastic cerebral palsy manifested as left hemiplegia and developmental delay. We believe this patient illustrates that incontinentia pigmenti is a systemic disorder necessitating a multidisciplinary approach to management.

A Case of Low-Fat, Fibrous Spindle Cell Lipoma / 대한피부과학회지

Spindle cell lipoma is a benign tumor composed of ropey collagen, myxoid stroma, CD34-positive spindled cells, and mature fat. The variable proportion of these elements among different cases of the neoplasm makes diagnosis difficult. Furthermore, numerous variants have been reported, including pseudoangiomatous spindle cell lipoma, angiomatous spindle cell lipoma, fibrous spindle cell lipoma, and low-fat or fat-free spindle cell lipoma. Here we report a case of low-fat, fibrous spindle cell lipoma in which fat is noted to be present in <5% of the tumor, whereas fibrous collagenous stroma is predominant.