Quality of Life and Economic Burden in Recessive Dystrophic Epidermolysis Bullosa

BACKGROUND: Patients with recessive dystrophic epidermolysis bullosa (RDEB) exhibit blisters and erosions since birth, causing pain, pruritus and various complications. RDEB affects quality of life (QoL) in physical, emotional and social aspects. Furthermore, interminable dressing changes and supportive therapies impose a significant economic burden on the patient's family. OBJECTIVE: We assessed the QoL and economic burden in patients with RDEB. METHODS: Sixteen patients with RDEB were surveyed to assess the QoL and economic burden. Patients answered questionnaires consisting of a visual analogue scale (VAS) on pain and pruritus, Skindex-29, Quality of Life in EB questionnaire (QOLEB), and the economic burden due to EB. RESULTS: Thirteen patients with RDEB completed the questionnaire. Female patients presented higher VAS, QOLEB and total Skindex-29 scores than male patients. Patients with RDEB showed severe levels of pruritus, which was more intolerable than pain. Mean VAS score on pain in RDEB was higher than in oral lichen planus and post-herpetic neuralgia. VAS score on pruritus was similar to those in chronic urticaria, atopic dermatitis, and prurigo nodularis. Compared with other dermatologic conditions, patients with RDEB were profoundly affected in all three scales of skindex-29. Mean "medical cost" in a month was $257.54 (USD) (+/-169.39) and mean "dressing cost" was $358.41 (USD) (+/-312.55), which was negatively related to patient age. CONCLUSION: RDEB had a profound impact on QoL and economic burden. Compared with other dermatologic diseases, RDEB showed severe symptoms and QoL was seriously impaired. Most patients sustained economic burdens, especially on preparing dressing materials. Younger patients experienced more economic burdens.

A Multicenter Collaborative Study by the Korean Society of Vitiligo about Patients' Occupations and the Provoking Factors of Vitiligo

BACKGROUND: It was previously thought that persons with genetic predispositions to vitiligo develop the condition after exposure to various precipitating environmental factors. However, in many cases, the aggravating factors of vitiligo have not been clearly identified. OBJECTIVE: To identify the aggravating factors of vitiligo in the working environment and daily life. METHODS: A total of 489 vitiligo patients were recruited from 10 institutions in South Korea; patients were provided with a questionnaire about environmental factors and behavior patterns in the workplace and in daily life, and their association with vitiligo. RESULTS: Ninety-five of the 470 enrolled patients (20.2%) answered that environmental risk factors in daily life and in the workplace affected the development of vitiligo. The most frequently attributed causes were trauma and burn (13.6%), followed by sunlight (12.8%), stress (12.8%), cleaning products/disinfectant/chemicals (4.9%), and hair dye (2.1%). CONCLUSION: Vitiligo of the hand and foot was associated with frequent exposure to aggravating materials and overexposure to sunlight, along with frequent trauma of these areas, all of which could be considered important risk factors of vitiligo. The development of vitiligo could potentially be controlled through the early detection of aggravating factors.

Cryopreserved Autologous Fat Injections as a Filler Agent for Facial Augmentation: Are They Still Safe?

No abstract available.

Transient Bullous Dermolysis of the Newborn / 대한피부과학회지

Transient bullous dermolysis of the newborn (TBDN) is a rare subtype of the dystrophic epidermolysis bullosa characterized by blistering at birth which improves spontaneously during early life. Electron microscopy showed sublamina densa separation with dilated rough endoplasmic reticulum and electron dense inclusions. Immunofluorescence mapping using anti-type VII collagen antibody showed widespread intraepidermal type VII collagens which are a characteristic finding of TBDN. Here, we report two cases of TBDN presenting typical clinical manifestations, electron microscopy findings, and immunofluorescence mapping results. The skin lesions of both patients healed spontaneously 2~3 months later.

Livedoid Vasculopathy with Hyperhomocysteinemia due to MTHFR Mutation / 대한피부과학회지

Livedoid vasculopathy is a hyalinizing vascular disease characterized by thrombosis and ulceration of the lower extremities. It can be caused by an alteration in control of coagulation with the formation of thrombi within dermal blood vessels. We report a case of livedoid vasculopathy with hyperhomocysteinemia due to MTHFR mutation, which is treated by folic acid and which also showed very unusual clinical manifestations. A 38-year-old male visited the department of dermatology with a 1 year history of purplish-brown purpura with punched-out ulcers on both lower legs. He had a history of homocysteinemia due to methylene tetrahydrofolate reductase (MTHFR) mutation. The histopathologic findings of the lesional skin revealed dense superficial and deep perivascular and perifollicular infiltrates of lymphocytes and fibrin deposition within the vessels in the dermis. On the basis of clinical and pathological findings, livedoid vasculopathy with hyperhomocysteinemia due to MTHFR mutation was diagnosed and improved by the treatment of 1 mg of folic acid daily.

Verification of the HACCP System in School Foodservice Operations: Focus on the Microbiological Quality of Foods in Heating Process and After-Heating Process

The objective of this study was to evaluate and improve the microbiological quality of HACCP application in school foodservice operations. The microbiological quality of foods and utensils were evaluated two times at each critical control point (CCP) with 3M petrifilm in five Daegu elementary schools. Two processes were evaluated: Heating process and after-heating process. The CCPs of the heating process were receiving, cooking and serving temperatures. The CCPs of the after-heating process were personal hygiene, cross contamination avoidance and serving temperature. After the first experiment, 31 employees of five schools were classroom educated, trained on-site, and pre- and post-tested on HACCP-based sanitation with the goal of improving the microbiological quality of the foodservice. Scores representing knowledge of holding, thawing, washing, food temperature, sanitizing and food-borne illness increased after education. In the heating process, internal food temperatures in the first and second experiments were higher than 74 degrees C, the holding temperature in the first experiment was less than 60 degrees C. In the second experiment, the serving temperature improved to a satisfactory level. The microbiological quality in the second experiment improved by decreasing the time from cooking to serving. In the after-heating process, the ingredients were boiled before being cut in the first experiment. In the second experiment, ingredients were cut before being boiled, improving microbiological quality. Also in the second experiment, cooking just before serving food improved its microbiological quality through time-temperature control. These results strongly suggest it is essential to measure microbiological quality regularly and to educate employees on HACCP continuously, especially time-temperature control and cross contamination avoidance in order to improve foodservice quality.