Subject(s)
Epidermolysis Bullosa Dystrophica , Quality of Life , Severity of Illness Index , Surveys and Questionnaires , Adolescent , Adult , Child , Child, Preschool , Epidermolysis Bullosa Dystrophica/psychology , Female , Humans , Male , Middle Aged , Prospective Studies , Reproducibility of Results , Romania , Young AdultABSTRACT
Individuals with epidermolysis bullosa (EB) may present with a broad spectrum of growth impairment and multiorgan disorders, with compromised nutritional status and quality of life. The provision of nutrients through a gastrostomy tube may minimize EB-related malnourishment but may also result in skin injuries and infections. In this systematic review we consider the current evidence about the effectiveness of gastrostomy in restoring nutritional status and improving quality of life in patients with EB. Seven studies (n = 146) met selection criteria and patients ranged in age from 6 weeks to 33 years of age. Although it is not a risk-free procedure, the placement of a gastrostomy tube is a feasible and safe alternative to provide nutritional support and to improve the quality of life of patients.
Subject(s)
Epidermolysis Bullosa Dystrophica/surgery , Gastrostomy , Nutritional Status , Quality of Life , Adolescent , Adult , Child , Child, Preschool , Enteral Nutrition/methods , Epidemiologic Methods , Epidermolysis Bullosa Dystrophica/psychology , Female , Humans , Infant , Male , Malnutrition/prevention & control , Malnutrition/psychology , Young AdultABSTRACT
The psychological aspect in patients with dystrophic epidermolysis bullosa (DEB) is poorly documented. We sought to determine the role of DEB in anxiety, depression and self-esteem. We conducted a cross-sectional study, collecting data from 27 DEB patients and 26 healthy individuals. DEB patients and healthy controls completed three different psychometric scales for anxiety and depression and one scale for self-esteem. DEB patients and healthy controls were homogeneous for age and sex (P > 0.05), but not for employment, marital status and economic level (P < 0.05). Median values of all psychometric battery scales were not statistically significant between DEB patients and healthy controls, except for Goldberg scale for anxiety (P = 0.003) and depression (P = 0.037) and slightly significant for Zung Scale for anxiety (P = 0.048) with no difference between DEB patients with dominant versus recessive form in all scales (P > 0.05). Among DEB patients, only employment showed a significant difference in all scales (P < 0.05) but Hamilton for depression, whereas self-esteem seemed to be affected by marriage (P = 0.04) and education (P = 0.016). DEB patients apparently are not more anxious and/or depressed and do not have less self-esteem than healthy individuals.
Subject(s)
Anxiety/etiology , Depression/etiology , Epidermolysis Bullosa Dystrophica/psychology , Self Concept , Adolescent , Adult , Case-Control Studies , Cross-Sectional Studies , Epidermolysis Bullosa Dystrophica/complications , Female , Humans , Male , Middle Aged , Psychometrics , Young AdultABSTRACT
Epidermolysis bullosa (EB) consists of a group of genetic hereditary disorders in which patients frequently present fragile skin and mucosa that form blisters following minor trauma. More than 20 subtypes of EB have been recognized in the literature. Specific genetic mutations are well characterized for most the different EB subtypes and variants. The most common oral manifestations of EB are painful blisters affecting all the oral surfaces. Dental treatment for patients with EB consists of palliative therapy for its oral manifestations along with typical restorative and periodontal procedures. The aim of this article is to describe two dental clinical treatments of recessive dystrophic EB cases and their specific clinical manifestations. The psychological intervention required during the dental treatment of these patients is also presented.
Subject(s)
Epidermolysis Bullosa Dystrophica/therapy , Mouth Diseases/therapy , Adolescent , Anti-Infective Agents, Local/therapeutic use , Blister/psychology , Blister/therapy , Child, Preschool , Chlorhexidine/therapeutic use , Dental Plaque/therapy , Dental Restoration, Permanent , Epidermolysis Bullosa Dystrophica/psychology , Female , Follow-Up Studies , Gingivitis/therapy , Humans , Jaw, Edentulous, Partially/rehabilitation , Male , Mouth Diseases/psychology , Oral Ulcer/therapy , Palliative Care , Patient Care Planning , Periodontal Diseases/psychology , Periodontal Diseases/therapy , Radiography, Panoramic , Tooth Extraction , ToothbrushingABSTRACT
Epidermolysis bullosa (EB) consists of a group of genetic hereditary disorders in which patients frequently present fragile skin and mucosa that form blisters following minor trauma. More than 20 subtypes of EB have been recognized in the literature. Specific genetic mutations are well characterized for most the different EB subtypes and variants. The most common oral manifestations of EB are painful blisters affecting all the oral surfaces. Dental treatment for patients with EB consists of palliative therapy for its oral manifestations along with typical restorative and periodontal procedures. The aim of this article is to describe two dental clinical treatments of recessive dystrophic EB cases and their specific clinical manifestations. The psychological intervention required during the dental treatment of these patients is also presented.
Subject(s)
Adolescent , Child, Preschool , Female , Humans , Male , Epidermolysis Bullosa Dystrophica/therapy , Mouth Diseases/therapy , Anti-Infective Agents, Local/therapeutic use , Blister/psychology , Blister/therapy , Chlorhexidine/therapeutic use , Dental Restoration, Permanent , Dental Plaque/therapy , Epidermolysis Bullosa Dystrophica/psychology , Follow-Up Studies , Gingivitis/therapy , Jaw, Edentulous, Partially/rehabilitation , Mouth Diseases/psychology , Oral Ulcer/therapy , Palliative Care , Patient Care Planning , Periodontal Diseases/psychology , Periodontal Diseases/therapy , Radiography, Panoramic , Tooth Extraction , ToothbrushingABSTRACT
Sam Hall was diagnosed with epidermolysis bullosa as a baby. Here she describes growing up with the condition and the continual dressing changes it brings, and how she has not let it prevent her from leading an active and exciting life.
Subject(s)
Adaptation, Psychological , Attitude to Health , Epidermolysis Bullosa Dystrophica/psychology , Activities of Daily Living , Adolescent , Bandages , Carboxymethylcellulose Sodium/therapeutic use , Epidermolysis Bullosa Dystrophica/diagnosis , Epidermolysis Bullosa Dystrophica/prevention & control , Female , Humans , Parents/psychology , Psychology, Adolescent , Quality of Life , Silicones/therapeutic use , Skin Care/methods , Skin Care/psychologyABSTRACT
Epidermolysis bullosa hereditaria dystrophica mutilans Hallopeau-Siemens is an autosomal recessive, chronically mutilating disease. Causal therapy is not known. In a five-year-old boy suffering from this disease and having the right hand completely clumped to a fist, syndactyly and contraction release was performed. The wounds healed under the care of the silicone-coated polyamide net dressing Mepitel. Under intensive postoperative physio- and ergotherapeutic care, a major part of his previous hand function could be restored. Complete epithelialization occurred within four weeks. Twelve months after the procedure, the area of the previous lesions was marked by an unexpected mechanical stability. It was very impressive to see the improvement in his psychological well-being and his sense of self-esteem during this period of partial recovery of his hand function.
