International bullous diseases group: consensus on diagnostic criteria for epidermolysis bullosa acquisita

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a complex autoimmune bullous disease disease with variable clinical presentations and multiple possible diagnostic tests, making an international consensus on the diagnosis of EBA essential. ----- OBJECTIVES: To obtain an international consensus on the clinical and diagnostic criteria for EBA. ----- METHODS: The International Bullous Diseases Group (IBDG) met three times to discuss the clinical and diagnostic criteria for EBA. For the final voting exercise, 22 experts from 14 different countries voted on 50 different items. When > 30% disagreed with a proposal, a discussion was held and re-voting carried out. ----- RESULTS: In total, 48 of 50 proposals achieved consensus after discussion. This included nine diagnostic criteria, which are summarized in a flow chart. The IBDG was unable to determine one procedure that would be applicable worldwide. A limitation of the study is that differential diagnosis of bullous systemic lupus erythematosus has not been addressed. ----- CONCLUSIONS: This first international consensus conference established generally agreed-upon clinical and laboratory criteria defining the clinical classification of and diagnostic testing for EBA. Holding these voting exercises in person with the possibility of discussion prior to voting has advantages in reaching consensus over Delphi exercises with remote voting

Ed Herzog and Fiancée in the Snow

Ed Herzog, a housemate of Bob Jones, poses with his German fiancée on base in Frankfurt, Germany. The photograph was taken during the only big snowfall that winter. Jones was a professor of physics at Linfield College from 1955-1987.https://digitalcommons.linfield.edu/lca_bobjones_photos/1013/thumbnail.jp

Determinants of skin problems of the stump in lower-limb amputees

Objective: To identify determinants of skin problems in lower-limb amputees. Design: Survey, using a questionnaire. Setting: Not applicable. Participants: Lower-limb amputees (N=2039) who either obtained their prosthesis through the Orthopedische Instrument Makerij (a group of orthopedic workshops in the Netherlands) or were a member of the (Dutch) National Society of Amputees (Landelijke Vereniging van Geamputeerden) were invited to participate. In total, 872 lower-limb amputees agreed to participate. Intervention: Amputees filled in the questionnaire to assess characteristics of the amputation and prosthesis, level of activity, slump and prosthesis hygiene, and skin problems. Stepwise backward logistic regression was performed to analyze determinants of skin problems. Main Outcome Measure: Skin problems in the month prior to completing the questionnaire. Results: A total of 816 questionnaires were received. Eventually 805 questionnaires were suitable for statistical analysis, Protective determinants were (in order of magnitude of association) older age, male sex, and amputation because of peripheral arterial disease and/or diabetes. Provocative determinants were (in order of magnitude of association) use of antibacterial soap, smoking, and washing the stump 4 times a week or more often. In total, 63% of the participants (95% confidence interval, 60%-67%) reported 1 or more skin problems. Conclusions: The provocative determinants identified in this study-use of antibacterial soap, smoking, and stump washing frequency-have to be studied for their clinical relevance

Skin problems in lower limb amputees: A systematic review

Purpose. Skin problems of the stump in lower limb amputees are relative common in daily rehabilitation practice, possibly impeding prosthetic use. This impediment may have great impact in daily life. Our objective was to review literature systematically concerning incidence and prevalence of skin disorders of the stump in lower limb amputees. Method. A literature search was performed in several medical databases (MEDLINE, CINAHL, EMBASE, RECAL) using database specific search strategies. Reference lists in the identified publications were used as threads for retrieving more publications missed in the searches. Only clinical studies and patient surveys were eligible for further assessment. Results. 545 publications were initially found. After selection, 28 publications were assessed for research methodology. Only one publication fulfilled the selection criteria. The prevalence of skin problems in a series of 45 lower leg amputees of 65 years and older was 16%. Conclusions. Prevalence and incidence of skin problems of the stump in lower limb amputees are mainly unknown

Evaporative Water Loss and Epidermis Regeneration in Partial-Thickness Wounds Dressed with a Fluid-Retaining Versus a Clot-Inducing Wound Covering in Guinea Pigs

The effects of a new high vapour permeable poly-(ether urethane) (PEU) wound covering on evaporative water loss (EWL) and epidermis regeneration in partial-thickness wounds in guinea pigs were investigated and compared with an occlusive wound covering (OpSite®) and air-exposed controls. It was also assessed whether the EWL reflected the phases of epidermis regeneration. In PEU-covered wounds, the initially raised EWL of 100 gm-2h-1 decreased in four days to 30 gm-2h-1, the same level as in occluded wounds. In control wounds this level was only reached after seven days, reflecting slower epidermis regeneration. The results showed that triphasic EWL behaviour corresponded to the sequence of wound healing. There was (1) a constant raised EWL until epidermal resurfacing is complete, (2) a fast EWL decrease during parakeratotic keratinization, and (3) a gradual normalization of the EWL during maturation of the stratum corneum. In wounds covered with OpSite this phasic EWL behaviour did not occur, owing to forced reduction because of the relatively impermeable covering. It was concluded that the PEU wound covering has an ideal water vapour permeance of 22.2 gm-2h-1 kPa-1, as can be seen in the accelerated epidermis regeneration and fast EWL reduction in partial thickness wounds in guinea pig

Skin problems of the stump in lower limb amputees: 1. a clinical study

Use of a prosthesis in lower limb amputees can lead to skin problems of the amputation stump. However, little is known about the epidemiology and type of problems experienced. We conducted a cross-sectional survey consisting of a questionnaire and a clinical assessment of the amputation stump. The aims of the study were to estimate the prevalence of skin problems of the amputation stump, to evaluate the impact of these skin problems and to evaluate differences between clinically observed skin problems and skin problems reported by the amputee. Participants (n=124) were recruited from among lower limb amputees who visited an orthopaedic workshop. The prevalence of skin problems was 36%. Problems identified were: reduction in prosthesis use, and reduction in walking distance without a break. Significantly more skin problems were reported than observed (p=0.011). Cold skin and excessive perspiration in particular were significantly more reported than observed

Skin problems in lower limb amputees: an overview by case reports

The stump in lower limb amputees is prone to skin problems because it is exposed to several unnatural conditions (shear and stress forces and increased humidity) when a prosthesis is used. This study reviews the literature on case reports of lower limb amputees with skin problems on the stump. In total, 56 reports comprising 76 cases were identified in the literature. The main disorders are acroangiodermatitis, allergic contact dermatitis, bullous diseases, epidermal hyperplasia, hyperhidrosis, infections, malignancies and ulcerations

Restrictions in oral functions caused by oral manifestations of epidermolysis bullosa

Several forms of epidermolysis bullosa (EB) present oral manifestations. Blistering of the (peri) oral mucosa affects the opening of the mouth, the mobility of the tongue and lips, thereby restricting oral functions. We describe the prevalence and characteristics of oral manifestations of EB in relation to loss of oral functions in a cross-sectional study of different types of EB patients using standardized measurement techniques. Twenty-two patients were included. The mobility of the mandible, lips and tongue was measured, the mandibular function impairment questionnaire (MFIQ) was filled out and additional questions regarding hindrance of EB during oral hygiene and intelligibility of speech (being understood) were asked in structured interviews. The median age was 11.8 yrs. Mobility of the mandible, tongue and lip was restricted, oral hygiene procedures were hindered in most patients. A data comparison was made between the recessive dystrophic EB (RDEB) and junctional EB (JEB) groups. Mandibular function was impaired in both groups but more severely in the RDEB-population. Intelligibility in both groups was almost unaffected. Restrictions in mobility of the mouth, tongue and lips are frequently present in EB patients. These are most severe in the RDEB group and support the clinical relevance of optimizing symptomatic treatment

LAMB3 mutations in generalized atrophic benign epidermolysis bullosa: consequences at the mRNA and protein level.

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