Paraneoplastic pemphigus (PNP) is a rare, life-threatening, autoimmune, mucocutaneous blistering disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. We retrospectively analyzed 12 Korean patients with PNP who were diagnosed between 1993 and 2011. We performed analysis of the clinical features, clinical outcomes, underlying neoplasia, histological features and laboratory findings. All of the patients except one had severe mucosal involvement. Two patients had only mucosal lesions but no cutaneous involvement was observed. Erythema multiforme or lichen planus-like eruptions rather than bullous lesions were more commonly observed skin rashes. The most common histological features were interface dermatitis and apoptotic keratinocytes. There were associated hematological-related neoplasms in 11 patients, with Castleman's disease (n = 4) as the most frequent. Twelve patients were followed for 5-148 months (mean, 43.0). The prognosis depended on the nature of the underlying neoplasm. Six patients died due to respiratory failure (n = 3), postoperative septicemia (n = 1), lymphoma (n = 1) and sarcomatosis (n = 1). The 2-year survival rate was 50.0%, and the median survival period after diagnosis was 21.0 months. Immunoblotting was performed in 12 patients and autoantibodies to plakins were detected in 11 patients. The results of this study demonstrated the clinical, histological and immunological diversity of PNP. Widely accepted diagnostic criteria that account for the diversity of PNP are needed.
Although the evidence for an environmental influence on AGA remains very weak, we did find an association between hair loss severity and certain environmental factors, such as smoking and drinking. Family history with more severe hair loss and an earlier age of onset.
BackgroundCutaneous carcinosarcoma is a rare biphasic tumor comprising malignant epithelial and heterologous mesenchymal elements. Data on the clinical and histopathologic characteristics of this tumor in Asian populations are not available. The purpose of this study was to investigate the clinicopathologic and immunohistochemical features of cutaneous carcinosarcoma in the Korean population.MethodsWe retrospectively reviewed the records of 11 patients with cutaneous carcinosarcoma who were diagnosed from 2006 to 2016.ResultsThe mean patient age at diagnosis was 71.5 years (range, 43–96 years) and there was a men predilection. The most common site of cutaneous carcinosarcoma was the head and neck (8/11, 72.7%). Histopathologically, most tumors showed a characteristic morphology consisting of two types of tumor cells, varied differentiated epithelial cells (such as basal or squamous cells) and spindle cells with transition zones between the two components. These two cell types also demonstrated variable immunohistochemical characteristics.ConclusionAlthough the number of cases in this study was limited, our results provide valuable insight into the clinical and histopathologic characteristics of cutaneous carcinosarcoma in the Korean population.
Ichthyosiform eruption as a specific manifestation of mycosis fungoides is very rare and only a few such cases have currently been reported in the medical literature. A 63-year-old Korean man presented with a 4-year history of a pruritic ichthyotic eruption. There was no personal or family history of ichthyosis or atopy. The ichthyosiform skin changes involved the abdomen, arms, thighs and shins. The face, palms and soles were spared. There was no peripheral lymphadenopathy or organomegaly. The typical lesions of mycosis fungoides were not present. The results of the routine investigations were normal or negative. A skin biopsy specimen revealed the findings of early mycosis fungoides. He was successfully treated with photochemotherapy. (Ann Dermatol 21(2) 182∼184, 2009)
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The localized early-stage of Mycosis fungoides (MF) (stage IA-IIA) is usually treated with topical agents, such as nitrogen mustard, steroids, and phototherapy (UVB/PUVA) as first line therapy; response to these initial treatments is usually good. However, hyperkeratotic plantar lesions are clinically rare and have decreased responsiveness to topical agents. For such cases, physicians may consider local radiotherapy. Here, a case of an 18-year-old Korean woman who was treated with three-dimensional conformal radiotherapy (3D-CRT) for hyperkeratotic plantar lesions that were refractory to UVA-1, methotrexate, and topical steroids is reported. Complete remission was attained after radiotherapy. During the one-year follow-up period, there has been no evidence of disease recurrence and no chronic complications have been observed.
Basal cell carcinoma (BCC) primarily develops in the head and neck region, with 74-83 per cent of BCC occurring in this region. Unfortunately, most published studies on BCC were conducted in Caucasian populations, and analytic data on extra-facial BCC in Asian and Korean patients, in particular, are not readily available. Here, we report on a retrospective analysis of extra-facial BCC in Korean patients. Thirty-five patients (16 men, 19 women) diagnosed with extra-facial BCC at Chonbuk National University Hospital between January 1981 and December 2008 were evaluated. Their average age was 62.3 years and most of the patients (11 of 35, 31%) were in their fifties. The relative tumour density (RTD) was the highest in the genitalia (0.769), followed by the axilla (0.481). Other regions such as the trunk, buttocks and upper and lower extremities exhibited a much lower RTD (average: 0.1). Histopathological examinations showed that 16 tumours were nodular (46%), eight were superficial (23%) and seven were mixed (20%). Additionally, potential predisposing factors were identified in seven cases. In five patients the use of Asian medicine, including acupuncture and herbal medication, was ascertained. To the best of our knowledge, the present study is the first to analyse the clinical and histopathological characteristics of extra-facial BCC in Korean patients. Our results indicate that the incidence of extra-facial BCC is higher in the axilla and genitalia than at other locations, although these sites are frequently overlooked during routine skin examinations.
The characteristic lesion of alopecia areata is a smooth bald patch on the scalp. When there is no bald surface it is called alopecia areata incognita. To date, all cases of alopecia areata reported as so-called 'incognito' have shown a diffuse involvement of the scalp as in acute telogen effluvium. Recently, we have observed two patients who showed localised hair thinning of the scalp without bald spots. Histopathologically, the lesions were typical of alopecia areata with peribulbar lymphocytic infiltrates. The response to corticosteroid treatment and its clinical course were also compatible with alopecia areata.
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