• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
• /
• v.21 no.2
• /
• pp.154-164
• /
• 2008

Epidermolysis Bullosa(EB) is rare, chronic mechanobullous disease, which easily produce bulla by minor trauma or spontaneously. There are three major forms of Epidermolysis bullosa - Epidermolysis bullosa simplex, Junctional epidermolysis bullosa, Dystrophic epidermolysis bullosa. This report is on the 21-year-old female patient who has Dystrophic epidermolysis bullosa(Recessive Dystrophic epidermolysis bullosa-mitis). The Korean medical treatments such as acupuncture and herbal medication were used, with light therapy : Carbon Are, which is known for effect of dermal resuscitation. As a result, Carbon Arc and Korean medical treatment's effect of dermal resuscitation is proved in this study. But more cases are necessary for establishing more effective treatment methods.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
• /
• v.20 no.1 s.32
• /
• pp.265-284
• /
• 2007

Introduction : There are three major forms of Epidermolysis Bullosa(EB) - EB Simplex(EBS), Junctional EB(JEB) and Dystrophic EB(DEB). DEB is divided into two groups that Dominant DEB(DDEB) and Recessive DEB(RDEB). RDEB has two types that Hallopeau-Siemens(generalized type) and mitis(localized type). Objects : This study was carried out to observe the progress of Recessive Dystrophic Epidermolysis Bullosa-mitis(RDEB-mitis) disease and find effective remedy. Methods: Both diagnosis and treatment were divided into three groups voluntarily. l. diagnosis - dermatic symptoms, mucosal symptoms, general symptoms 2. treatment - herbal medications, moxibustion, medicines used extemally(外用藥) Results : After treating 9 months, the condition of the patient grew better and the grades of severity of ROEB-mitis disease were lower at each diagnosis item. Conclusions : As above results, oriental medical treatments were very effective for this disease. But more case studies are necessary for establishing more effective diagnosis and treatment methods.

• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.8 no.2
• /
• pp.109-112
• /
• 2012

Epidermolysis bullosa (EB) is an inherited skin disease characterized by blister formation of the skin following just minor trauma or spontaneously. The main classification of EB is based on the localization of the blistering. In epidermolysis bullosa simplex, the blister formation is intraepidermal; in epidermolysis bullosa junctionalis the blister formation in the epidermal-dermal junction; in the dystrophic forms of EB blister formation is intradermal. Oral manifestations of recessive dystrophic EB are characterized by rampant caries, crowding, microstomia, ankyloglossia, vestibular obliteration. Dental therapy may be well provided to patient with recessive dystrophic EB by using general anesthesia especially in case of poor cooperation. A two years old girl with recessive dystrophic EB visited our clinic for rampant caries. She was hospitalized for severe anemia and fever, and we provided dental therapy under general anesthesia. Extraction of incisors and stainless steel crown restoration of primary molars were performed.

• Archives of Reconstructive Microsurgery
• /
• v.19 no.2
• /
• pp.97-100
• /
• 2010

Purpose: Epidermolysis bullosa is a rare genetic disease, characterized by the presence of extremely fragile skin and formation of recurrent blister resulting from even a minor mechanical injury. Squamous cell carcinoma (SCC) is recognized as a complication of the chronic scarring associated with dystrophic epidermolysis bullosa (DEB). When a soft tissue defect happens in a patient with epidermolysis bullosa, it is difficult to cover it with a skin graft or a flap. We describe the successful use of a pedicled deep inferior epigastric perforator flap for the reconstruction of SCC associated with DEB in the groin. Methods: A 29-year-old man diagnosed with DEB at birth sustained an ulcer increasing in the right groin for the last 7 months. Under general anesthesia, the mass lesion and lymph nodes were removed and the resulting defect was covered with a pedicled deep inferior epigastric perforator flap. Results: The flap survived completely and his postoperative course was uneventful. Histopathological examination revealed a SCC in the right groin and malignant tumor cells in the removed lymph nodes as well. Additional positron emission tomogram showed a malignant lesion in the ileocecal area with regional lymph node metastasis. The patient was referred to an oncologist for chemotheraphy, but the patient refused to take it. During a 4-month follow-up period, there was no recurrence in the right groin. Conclusion: We suggest that perforator flaps can be considered as a reliable alternative for the reconstruction of soft tissue defects in a patient with DEB.

• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.8 no.2
• /
• pp.122-126
• /
• 2012

Epidermolysis bullosa (EB) is a genetic disorder, characterized by blisters on skin and mucosal surfaces even upon light mechanical damage. EB is caused by genetic mutations in at least seven proteins on the basement membrane zone, which is the boundary between the epidermis and the dermis. There are many types of EB differing in clinical and genetic aspects, and the prognosis varies depending on the EB type. There are largely three types of EB, categorized by the electron-microscopic location of the blisters. The blisters form within the epidermis in epidermolysis bullosa simplex, in the lamina lucida in junctional epidermolysis bullosa, and just beneath the basal lamina in dystrophic epidermolysis bullosa. To date, there is no medication or treatment that cures EB or completely prevents the blisters, so generally symptomatic treatment is performed. EB patients must always be cautious, for blisters can form at the slightest injuries, and the patients must be dealt with gently. Injuries and infections have to be prevented and treated, and deficient nutrients must be supplied during dental treatment period. Some patients may experience pain when swallowing food or dental treatment due to blisters and resulting scars in the mouth, pharynx, and esophagus. Recently, two pediatric patients were diagnosed with EB at Pusan National University Hospital and visited the Department of Pediatric Dentistry for oral care and dental treatment. The treatment results are reported here.