Folliculotropic Mycosis Fungoides in 20 Korean Cases: Clinical and Histopathologic Features and Response to Ultraviolet A-1 and/or Photodynamic Therapy

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a variant of mycosis fungoides (MF) that is characterized clinically by variable types of skin eruptions, including plaques, acneiform lesions, and alopecic patches. Histopathologically, FMF is characterized by folliculotropic infiltrates. OBJECTIVE: This study was conducted to scrutinize the clinical and histopathologic features of FMF in Koreans and the responses to phototherapy. METHODS: Twenty Koreans diagnosed with MF who had histopathologic evidence of folliculotropism were enrolled. RESULTS: Eighteen patients had head-and-neck-region infiltration, while five had solitary lesion. In all patients, the atypical lymphocytic infiltrate had a perifollicular distribution. Twelve patients were treated with ultraviolet A (UVA)-1. Eleven of these 12 patients with early-stage FMF experienced >80% improvement (8: complete remission; 3: partial remission). Four patients, including 2 who relapsed after UVA-1, were treated with photodynamic therapy (PDT), reaching complete remission after PDT. CONCLUSION: As FMF has variable clinical presentations, skin biopsy is required to confirm the diagnosis. And both UVA-1 and methyl aminolevulinate-PDT are clinically effective in treatment of early-stage FMF.

The Clinical Profile of Patients with Psoriasis in Korea: A Nationwide Cross-Sectional Study (EPI-PSODE)

BACKGROUND: Psoriasis is an immune-mediated, chronic inflammatory disease affecting multiple aspects of patients' lives. Its epidemiology varies regionally; however, nationwide epidemiologic data on psoriasis depicting profile of Korean patients has not been available to date. OBJECTIVE: To understand nationwide epidemiologic characteristics and clinical features of adult patients with psoriasis visited university hospitals in Korea. METHODS: This multicenter, non-interventional, cross-sectional study recruited 1,278 adult patients with psoriasis across 25 centers in Korea in 2013. Various clinical data including PASI, BSA, DLQI, SF-36 and PASE were collected. RESULTS: A total of 1,260 patients completed the study (male:female=1.47:1). The mean age was 47.0 years with a distribution mostly in the 50s (24.9%). Early onset (<40 years) of psoriasis accounted for 53.9% of patients. The mean disease duration was 109.2 months; mean body mass index was 23.9 kg/m²; and 12.7% of patients had a family history of psoriasis. Plaque and guttate types of psoriasis accounted for 85.8% and 8.4%, respectively. Patients with PASI ≥10 accounted for 24.9%; patients with body surface area ≥10 were 45.9%. Patients with DLQI ≥6 accounted for 78.8%. Between PASI <10 and PASI ≥10 groups, significant difference was noted in age at diagnosis, disease duration, blood pressure, waist circumference of female, and treatment experiences with phototherapy, systemic agents, and biologics. CONCLUSION: This was the first nationwide epidemiologic study of patients with psoriasis in Korea and provides an overview of the epidemiologic characteristics and clinical profiles of this patient population.

Acquired acrodermatitis enteropathica caused by zinc deficiency in a heavy drinker: A case report

Acrodematitis enteropathica is an uncommon disease due to zinc deficiency which is characterized by perioral and acral dermatitis, alopecia, and diarrhea. This disease typically presents in infants, either due to an autosomal recessive genetic disorder or after cessation of breastfeeding. Acquired adult-onset acrodermatitis enteropathica has rarely been reported so far. We report a case of a 38-year-old man who had a history of chronic alcohol intake and presented with severe acrodermatitis enteropathica. The patient was successfully managed with zinc supplement and supportive care.

Pityriasis Lichenoides-like Mycosis Fungoides: Clinical and Histologic Features and Response to Phototherapy

BACKGROUND: Pityriasis lichenoides (PL)-like skin lesions rarely appear as a specific manifestation of mycosis fungoides (MF). OBJECTIVE: We investigated the clinicopathological features, immunophenotypes, and treatments of PL-like MF. METHODS: This study included 15 patients with PL-like lesions selected from a population of 316 patients diagnosed with MF at one institution. RESULTS: The patients were between 4 and 59 years of age. Four patients were older than 20 years of age. All of the patients had early-stage MF. In all patients, the atypical lymphocytic infiltrate had a perivascular distribution with epidermotropism. The CD4/CD8 ratio was <1 in 12 patients. Thirteen patients were treated with either narrowband ultraviolet B (NBUVB) or psoralen+ultraviolet A (PUVA), and all of them had complete responses. CONCLUSION: PL-like MF appears to have a favorable prognosis and occurrence of this variant in adults is uncommon. MF should be suspected in the case of a PL-like skin eruption. Therefore, biopsy is required to confirm the diagnosis of PL-like MF, and NBUVB is a clinically effective treatment.

Ultraviolet A1 Phototherapy of Mycosis Fungoides

No abstract available.

Ultraviolet A1 Phototherapy of Mycosis Fungoides

No abstract available.

Narrowband Ultraviolet B Phototherapy of Early Stage Mycosis Fungoides in Korean Patients

BACKGROUND: Narrowband UVB (NBUVB) is currently used to treat early mycosis fungoides (MF). There are a number of reports on the efficacy and safety of NBUVB in Caucasians, but little data is available for Asians. OBJECTIVE: This study was designed to evaluate the effectiveness and safety of NBUVB for early stage MF in Korean patients. METHODS: We enrolled 14 patients (12 men, 2 women; age range, 10~64 years) with clinically and histologically proven MF. Three patients were stage IA, and the others were stage IB. The patients received NBUVB phototherapy three times a week. The starting dose was 70% of the minimal erythema dose and was increased in 20 percent increments if the previous treatment did not cause erythema. Clinical response, total number of treatments, total cumulative dose, duration of remission and side effects were investigated. RESULTS: Eleven of 14 patients (78.6%) achieved complete remission within a mean of 15.36+/-5.71 weeks (range, 5~27 weeks), 31.0+/-7.4 treatments (range, 16~39 treatments) and a mean cumulative UVB dose of 31.31+/-12.16 J/cm2 (range, 11.4~46.8 J/cm2). Three of the 14 patients (21.4%) achieved a partial remission. After discontinuation of treatment, 6 of 11 patients (54.5%) with complete remission relapsed after a mean of 8.5+/-4.09 months. No serious adverse effects were observed except for hyperpigmentation (7/14, 50%). CONCLUSION: Our data suggest that NBUVB therapy is safe and effective for the treatment of early stage MF in Korean patients.

Efficacy of Ultraviolet A1 Phototherapy in Recalcitrant Skin Diseases

BACKGROUND: Ultraviolet (UV) radiation has been used for decades to treat a variety of skin diseases. UVA1 was used initially as an effective treatment for acute exacerbated atopic dermatitis. Since then, UVA1 has been attempted for recalcitrant skin diseases. OBJECTIVE: This study examined the efficacy of UVA1 phototherapy in three recalcitrant skin diseases. METHODS: This retrospective study reviewed the efficacy and follow-up of 26 patients with atopic dermatitis (AD), mycosis fungoides (MF) and localized scleroderma (LS). SUPUVASUN 3000 (Mutzhas Co., Munich, Germany) and SELLAMED 3000 (Sellas Medizinische Gerate GmbH, Gevelsberg, Germany) were the UVA1 equipment used. Irradiation was performed in accordance with the disease. Low-dose (20 J/cm2), medium-dose (65 J/cm2) and high-dose regimens (100 J/cm2) of UVA1 therapy were employed. The frequency of the therapy ranged from 3 to 5 times weekly. The therapeutic effectiveness was assessed according to the clinical examination before and after the last treatment. RESULTS: In patients with AD, complete and partial remission was achieved in four (80%) and one (20%) patient, respectively. In patients with MF, complete and partial remission was observed in thirteen (86.7%) and two (13.3%) patients, respectively. In patients with LS, complete and partial remission was observed in three (50%) and three (50%) patients, respectively. CONCLUSION: UVA1 phototherapy is an effective treatment modality for acute exacerbated AD, MF and LS.

A Case of Necrolytic Migratory erythema Induced by a Pancreatic Insufficiency / 대한피부과학회지

Necrolytic migratory erythema is a cutaneous reaction pattern with specific histopathologic features that is typically associated with a functioning pancreatic islet cell neoplasm such as a glucagonoma. However, cases without. glucagonoma have also been reported, such as, liver cirrhosis, chronic pancreatitis, celiac sprue etc. Other clinical features include anemia, glossitis and weight loss. We report a case of neccrolytic migratory erythema induced by a pancreatic insufficiency without glucagonoma. A 43-year-old male was seen at our department because of rnultiple erythematous, desquamative and erosive patches on the whole body for 1 month. He also had weight loss(25Kg) and stomatitis. Seven years previously he had a Whipple's procedure for a pancreatic head rupture. The laboratory data showed a low protein and zinc level, and high glucagon level. Histopathologic findings of the case showed epidermal edema and pallor, and superficial epiderrnal necrosis. The patient improved progressively after intravenous infusion of amino acids with a pancreatic enzyme supplementation.