No abstract available.
Epidermolysis Bullosa* ; Epidermolysis Bullosa, Junctional*

No abstract available.
Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa*

No abstract available.
Epidermolysis Bullosa Dystrophica* ; Epidermolysis Bullosa*

No abstract available.
Epidermolysis Bullosa Dystrophica* ; Epidermolysis Bullosa*

Dystrophic epidermolysis bullosa is a rare, chronic non-inflammatory bullous disease with a hereditary pattern of occurrence, and this disease easily produces bullae that heal with scarring and milium formation. A 13-month-old female baby was brought to the department of dermatology with multiple variable sized bullae and erythematous to dark brownish patches and crusts on both her feet. The histopathologic findings showed subepidermal non-inflammatory blisters and the electron microscopic findings showed vacuolization of the sublamina densa and broken anchoring fibrils. On the basis of the clinical and microscopic findings, she was diagnosed as having dominant dystrophic epidermolysis bullosa. We report here on a case of dominant dystrophic epidermolysis bullosa with the clinical, histological and electron microscopic findings, as well as the family history of the patient.
Blister ; Cicatrix ; Dermatology ; Electrons ; Epidermolysis Bullosa Dystrophica ; Epidermolysis Bullosa Simplex ; Female ; Foot ; Humans ; Infant

BACKGROUND: In epidermolysis bullosa acquisita, it has been recognized that there exists heterogeneity in the clinical and serologic/immunopathologic features. OBJECTIVE: We examined patients with epidermolysis bullosa acquisita to see if there were any associated clinical and serological features which may predict disease activity or prognosis in the disease. METHODS: Clinical and some serologic features were compared. between 2 groups of patients with epidermolysis bullosa acquisita; one with complete remission of the symptoms and signs of the disease for more than 2 years and the other group with persistent disease activities of longer than 5 years.
Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Humans ; Population Characteristics ; Prognosis

Introduction: Epidermolysis Bullosa Pruriginosa (EBP) is a rare subtype of the inherited Dystrophic ~ Epidermolysis Bullosa spectrum of diseases and results from a gene mutation in COL7AL Though predominantly an autosomal dominant disease, autosomal recessive and even sporadic have been reported. Case Summary: Case Summary:We report a case of a 12-year-old Filipino male presenting with a chronic history of numerous scratching-induced blisters predominantly distributed on the extensor aspect of his arms and legs without concomitant oral lesions, nail dystrophy, or hair findings, and without a family history of similar lesions. Histopathologic assessment, Direct Immunofluorescence (DIF), and Indirect Immunofiuorescence (IIF) showed a subepidermal split with scant inflammatory infiltrates, no immunofluorescence, and absent userrated linear immunofluorescence at the dermal-side of the Salt Split Skin slide, respectively, which were all consistent with EBP. Enzyme-Linked Immunosorbent Assay (ELISA) for Anti-Collagen VII antibodies was slightly elevated, which may suggest an alternative diagnosis of Epidermolysis Bullosa Acquisita (EBA). This slight elevation may be due to the mutated Collagen Vil protein becoming antigenic and therefore provoking an immune response. To conclusively distinguish EBP from EBA, a COL7AI gene mutation analysis was recommended. With a diagnosis of EBP cannot totally rule out EBA, the patient was initially managed with dapsone monotherapy, counseled regarding behavioral modification to reduce scratching and trauma, advised wound care and close monitoring for the development of oropharyngeal lesions, and recommended for COL7A1 genetic mutation analysis. Conclusion This report demonstrates a case of EBP with elevated Anti-Collagen VII antibodies. The diistinction between EBP and EBA is important because this changes the management: EBP is largely supportive, while EBA may benefit from immunosuppressive therapy.
Epidermolysis Bullosa Pruriginosa ; Enzyme-Linked Immunosorbent Assay ; Epidermolysis Bullosa Acquisita

OBJECTIVE: We investigate the presence and the quality of pain in patients with dystrophic epidermolysis bullosa (DEB), and its correlation with the level of anxiety and depression. METHODS: We collected data from 27 DEB patients and 26 healthy individuals. DEB patients and controls completed 1 scale for the quality of pain, and 1 scale for anxiety and depression. Pain was assessed with the short form of the McGill Pain Questionnaire, whereas anxiety and depression were assessed with the Hamilton rating scale for anxiety and depression. RESULTS: DEB patients and healthy control individuals were homogeneous for age and gender (p>0.05). A statistically significant difference in the two groups was seen for sensory pain rating scale (p<0.001), affective pain rating scale (p=0.029), total pain rating scale (p<0.001), visual analogue scale (p=0.012) and present pain intensity (p=0.001), but not for anxiety (p=0.169) and depression (p=0.530). The characteristics of pain that showed a significant difference between DEB patients and healthy controls were shooting, splitting, tender and throbbing (p<0.05). In DEB patients pain was not correlated with anxiety or depression (p>0.05), whereas a slight correlation between pain and anxiety was found in healthy controls (p<0.05). No difference was found between quality of pain and anxiety-depression in DEB patients (p>0.05), but was between the DEB dominant and the recessive form of DEB (p=0.025). CONCLUSION: The perception of pain in DEB patients appears greater than in healthy individuals, with splitting and tender characteristics being the most significant ones, but was not associated with anxious and/or depressive symptoms.
Anxiety* ; Depression* ; Epidermolysis Bullosa ; Epidermolysis Bullosa Dystrophica* ; Humans ; Pain Measurement

We have experienced a case of congenital pyloric atresia associated with epidermolysis bullosa in a premature newborn who was born at the gestation period of 33+3 week. She showed a few blisters on left ankle at birth and the easy formation of blisters involving the area of trauma or friction with depigmentation after healing. The histologic finding of the lesion showed junctional epidermolysis bullosa. Abdominal roentgenographic finding on day 2 showed single bubble sign. That suggested pyloric atresia. It was confirmed by upper gasrtointestinal series radiography and corrected by surgery, gastrojejunostomy on day 16. She discharged on day 50. The severity of the formation of blisters decreased but the poor weight agin became the main problem. The brief review of literatures was made.
Ankle ; Blister ; Epidermolysis Bullosa* ; Epidermolysis Bullosa, Junctional ; Friction ; Gastric Bypass ; Humans ; Infant, Newborn ; Parturition ; Pregnancy ; Radiography

The Dowling-Meara type of epidermolysis bullosa simplex(EBS) is genetic disease that is transmitted as an autosomal dominant trait and is charscterized clinically by pherpetiform clustering of blisters and palmo-plantar keratoderma. The disease usually presents at with or in early infancy. Although serious and hemorrhagic Wers may occur on any part of the body, the lesions heal without scaning in general. The disease shows a tendency to improve by progressian of age and it usually follows a relatively benign course. Microecopically, there are intraepidermal bli.ter s forming as a result of cytolysis of basal cells. In addition, the is a highly characteristic clumping of tonofilaments of keratinocytes in the lower epidermis, which is not seen in any other form of EBS. We report a case of Dowling-Meara type of EBS that is first destribed in Korean medical literatures.
Blister ; Epidermis ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Intermediate Filaments ; Keratinocytes