Clinical and pathological characteristics of extradigital and digital glomus tumours: a retrospective comparative study.

BACKGROUND: Most glomus tumours are located in the digits, especially in subungual areas. Less is known, however, about the clinical characteristics of extradigital glomus tumours. OBJECTIVES: The aim of this study was to characterize extradigital glomus tumours and compare their clinical, pathological and immunohistochemical characteristics with those of digital glomus tumours. METHODS: We retrospectively reviewed the charts of 110 patients with digital and 42 patients with extradigital glomus tumours, treated at a single centre from 1991 to 2010. We also investigated the immunohistochemical expression of substance P and TRPV1 in the biopsy specimens from patients with digital (n = 5) and extradigital (n = 5) glomus tumours. RESULTS: Compared with patients with digital glomus tumours, patients with extradigital tumours were significantly older (P = 0.009) and showed a significantly greater male predominance (P < 0.001), lower incidence rates of pain (P = 0.019), cold intolerance (P < 0.001) and a higher frequency of the glomangioma subtype (P < 0.001). Extradigital glomus tumours were located most frequently on the upper extremities (n = 25). The most common clinical presentations were solitary purplish papules (n = 10). Only 20% of extradigital glomus tumours were diagnosed correctly by the initial physician. Of 38 evaluable patients, 34 (89.5%) were successfully treated with simple excision without recurrence. Substance P and TRPV1 were expressed moderately to strongly in glomus cells, but there were no differences in their expression between patients with digital and extradigital glomus tumours. CONCLUSION: Extradigital glomus tumours do not have specific clinical features such as pain, leading to frequent misdiagnosis. To prevent delay in diagnosis or misdiagnosis, it is important to increase awareness of these tumours.

A case of congenital pigmented dermatofibrosarcoma protuberans (Bednar tumor) in a patient with Fanconi anemia.

Bednar tumor is a rare pigmented variation of dermatofibrosarcoma protuberans, present in 1 to 5% of all patients with dermatofibrosarcoma protuberans. No significant clinicopathologic differences exist between Bednar tumor and conventional dermatofibrosarcoma protuberans apart from the presence of scattered nonneoplastic pigmented dendritic cells in the former. Although most dermatofibrosarcoma protuberans occur in adults, they may be rarely present at birth. Fanconi anemia is a genetically heterogeneous chromosomal instability syndrome, characterized by multiple congenital anomalies, progressive bone marrow failure, and a predisposition to malignancy. We describe here a patient with Fanconi anemia who had a congenital Bednar tumor. To our knowledge, this is the first such patient described with both dermatofibrosarcoma protuberans and Fanconi anemia.

An unusual clinical presentation of tinea faciei caused by Trichophyton mentagrophytes var. erinacei.

Trichophyton mentagrophytes var. erinacei, the natural host of which is the hedgehog, has been found to cause highly inflammatory and pruritic eruptions, including tinea manuum, tinea corporis, nail infection, kerion, scalp infection, and tinea barbae. To our knowledge, however, no reports have been made of tinea faciei caused by Trichophyton mentagrophytes var. erinacei in the English language literature. We provide here the case of tinea faciei caused by Trichophyton mentagrophytes var. erinacei.

Dermatopathic lymphadenitis with generalized erythroderma in a patient with epstein-barr virus-associated hemophagocytic lymphohistiocytosis.

Here, we describe a patient with Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) who simultaneously presented with generalized erythroderma and dermatopathic lymphadenitis (DL). A 63-year-old Korean woman presented at our hospital with fever, hepatosplenomegaly, axillary lymphadenopathy, and generalized erythrodermic eruption. The bone marrow biopsy findings were consistent with the diagnosis of HLH, and EBV DNA was detected using the polymerase chain reaction. Based on serologic tests that indicated a primary EBV infection, the patient was diagnosed with EBV-associated HLH. Histopathologic analysis of enlarged lymph nodes was consistent with DL, and EBV-encoded small nuclear RNA-positive mononuclear cells were detected. We assume that activated histiocytes, lymphocytes, and proinflammatory cytokines in HLH may have important roles in the development of generalized erythroderma and DL. Disrupted epidermal/dermal junctions owing to erythroderma may also be involved in the development of DL.

Cutaneous metastasis of extraskeletal osteosarcoma arising in the mediastinum.

Cutaneous metastases have been reported in 5%-10% of patients with internal malignancy. The most common clinical features are the sudden appearance of discrete, firm, and nontender nodules in a particular area of the body. The most frequent metastatic site of extraskeletal osteosarcoma (ESOS) is the lungs. Cutaneous metastasis of ESOS is rare. To our knowledge, only one case of ESOS has been reported with skin metastasis, histopathologically classified as the osteoblastic subtype. Here, we describe a case of chondroblastic ESOS arising in the mediastinum with metastasis to the skin. Our patient presented with a scalp nodule after diagnosis of primary ESOS at the mediastinum.

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 ßf1 (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.

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.

Role of CD4CD25FOXP3 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 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.