Clinical and Epidemiological Studies of Patients with Autoimmune Bullous Diseases / 대한피부과학회지

BACKGROUND: Autoimmune bullous diseases (ABDs) are a rare significant group of dermatoses that pose great challenges to the dermatologist. So far, few epidemiological surveys for the whole spectrum of ABDs have been completed in Korea, though many epidemiological survey have focused on single ABDs. OBJECTIVE: The aim of this study was to evaluate the incidence and clinical findings of patients in the Chonnam Province of Korea with ABDs over a 5 year period. METHODS: A retrospective analysis was conducted on 52 ABDs patients from 2002 to 2006. ABDs were diagnosed chiefly by histopathologic findings, immunofluoresence (IF), and immunoblotting with epidermal and dermal human foreskin extract. For the exact diagnosis of ABDs, several additional diagnostic methods such as indirect IF with salt-split normal skin, immunoblotting (IB) with normal keratinocyte cells, enzyme-linked immunosorbent assay (ELISA) with recombinant proteins, immunoprecipitaion (IP) were also performed. RESULTS: Out of 52 patients diagnosed with ABDs during the study period, pemphigus vulgaris was observed to be the commonest ABD (38.5%) followed by bullous pemphigoid (29.8%), pemphigus foliaceus (17.8%), epidermolysis bullosa acquisita (5.8%), paraneoplastic pemphigus, pemphigoid gestationis (3.8%), and linear IgA bullous dermatoses (1.9%). CONCLUSION: This study showed the incidence of the ABDs in Chonnam-Gwangju province area were very low (52 cases over 5 years) and was stationary over this period. Pemphigus vulgaris and bullous pemphigoid were common diseases among many ABDs. The incidence of ABDs in Korea as a whole needs to be investigated.

The Efficacy of Cyclosporin in Patients with Atopic Dermatitis / 대한피부과학회지

BACKGROUND: Many studies revealed that short-term use of cyclosporin (CS) is quite effective for AD. Unfortunately, there have been very few studies on the long-term use of CS and relapse after cessation of CS in Korea. OBJECTIVE: The aim of this study was to evaluate the efficacy and side-effects of CS and relapse of diseases in moderate to severe AD patients. METHODS: CS was prescribed to 30 patients with moderate and severe AD for an average of 12 weeks. Initial dose was 4.2 mg/kg/day (maximum 300 mg/day) and dose was reduced according to their therapeutic responses. Efficacy was evaluated by the Eczema Area and Severity Index (EASI) score on each visit. We employed a measurement system in which 4 different sites (head/neck, trunk, upper extremities, lower extremities) were scored. After cessation of CS we monitored how long remission was sustained for 16 weeks. RESULTS: After an average of 1 week of CS treatment, a significant reduction in mean EASI score and symptom were observed (p<0.05). The mean EASI scores of 30 patients decreased from 29.18 to 14.31 (p<0.05). Marked improvement in the patient's assessment of pruritus was observed in the early stage of the treatment and was maintained throughout the study. Peripheral blood eosinophils significantly decreased after treatment (p<0.05). Relapse occurred within an average of 7 weeks and the EASI score after treatment was equivalent to 67% of the pre-treatment EASI score. CONCLUSION: We suggest that CS is an effective therapy in moderate to severe AD without any serious side effects.

A Case of Aplasia Cutis Congenita, Type VII

Aplasia cutis congenita (ACC) is a rare congenital defect in which localized or widespread areas of the skin are absent at birth. In the majority of cases, it is limited to the scalp especially on the vertex although other areas of the body may also be involved. Other congenital malformations can be associated with ACC. We present herein the case of a new born male with unilateral absence of skin on the extensor surface of the right lower leg. There was no associated malformation or skin disease such as blistering or nail abnormailty. According to the classification outlined by Frieden, the condition was diagnosed as type VII aplasia cutis congenita. The treatment of this large ulcer was conservative, wet dressing and prophylactic topical antibiotics. On follow up after 2 years showed that the patient was nearly cured of the ulcer and had only minimal scar formation.

A Case of Primary Cutaneous Mucinous Carcinoma on the Chest / 대한피부과학회지

Primary cutaneous mucinous carcinoma is a rare epithelial neoplasm occurring mostly in middle aged people. It arises on the head, favoring the scalp and face with preference on the eyelids, and generally follows indolent course with frequent local recurrence. We report a rare case of mucinous carcinoma which developed on the chest of a 60-year-old man. He was treated with total excision and full-thickness skin graft. This is the first such case to be reported in Korean dermatologic literature.

A Case of Angiolymphoid Hyperplasia with Eosinophilia of the Lower Eyelid

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a subcutaneous angioproliferating tumor with a characteristic inflammatory infiltrate that consists mainly of lymphocytes and eosinophils. A 24-year-old man presented with a firm single asymptomatic erythematous papule on the lower eyelid. Histopathological findings showed a proliferation of blood vessels lined with epithelioid cells and perivascular infiltration of lymphocytes and eosinophils. We report a case of ALHE occurring on an unusual site.

A Case of Primary Cutaneous Diffuse Large B-cell Lymphoma of the Leg Treated by Total Excision / 대한피부과학회지

Primary cutaneous diffuse large B-cell lymphoma of the leg is characterized by a diffuse non-epidermotropic infiltrate predominantly made up of large non-cleaved B-cells, with variable proportions of centroblast- and immunoblast-like cells. We report a case of a 90-year-old female, who presented with a solitary erythematous nodule and ulceration on the lower leg. Physical examination and staging work-up revealed no evidence of systemic involvement. After total excision and a split-thickness skin graft, the skin lesion was almost cleared. Furthermore, no other skin lesion was observed during a 1 year of follow up period.