Genetic and acquired blistering disorders of pediatric age group: An experience from Eastern India.

INTRODUCTION: Blistering or vesiculobullous disorders in pediatric population are either immunobullous or mechanobullous. Spectrum was analyzed using demographic details, clinical features, histopathology, direct immunofluorescence (DIF) and Immunofluorescence mapping (IFM). METHODOLOGY: This was a single institution based observational study in children below 18 years. The demographic details were collected using proforma containing particulars of the patient, history, complaints, and other parameters. Punch biopsy of the skin lesion was done. Biopsy samples were examined under light microscope followed by DIF using fluorescent conjugated polyclonal antibody against immunoglobulins IgG, IgM, IgA, and complement C3. The salt-split technique was also used in particular cases. IFM was done using anticytokeratin (CK) 5 & 14, antilaminin 332, anticollagen VII, and anticollagen IV antibodies. RESULTS: Out of total 50 cases, linear IgA bullous dermatosis (LABD) was the commonest. The average concordance between clinical and final diagnosis (histopathological examination + DIF) was 87.5% and discordance was 12.5%. The agreement between histopathological examination and DIF was found to be substantially significant (κ = 0.6892). IFM depicted epidermolysis bullosa simplex with reduced CK 14 expression, dystrophic epidermolysis bullosa with reduced Collagen VII expression and junctional epidermolysis bullosa with absent laminin 5 expression. CONCLUSION: The spectrum of bullous lesions in childhood was properly delineated and subcategorization of EB was done. Histopathological examination showed the hallmarks that were conclusive in most of the cases except in LABD and EB. DIF and IFM proved indispensable in those cases. Thus, DIF is not a substitute for histopathology but complementary to it.

Evaluation of filtering blebs using the 'Wuerzburg bleb classification score' compared to clinical findings.

BACKGROUND: To determine the agreement between intraocular pressure and the 'Wuerzburg bleb classification score', as well as between single items of the score and intraocular pressure. Interobserver variability was analyzed. METHODS: 57 post-trabeculectomy eyes were included. Colour photographs were used to score the filtering bleb in accordance to the Wuerzburg bleb classification score by two different examiners. At the same visit, clinical data such as intraocular pressure, best corrected visual acuity, slit lamp biomicroscopy and medical history were obtained by another examiner. RESULTS: After trabeculectomy, 42 out of 57 eyes (73.7%) reached the target pressure (≤ 21mmHg, and intraocular pressure reduction of at least 20%, without antiglaucoma medication, and without any additional intervention). Fair agreement was found between intraocular pressure and Wuerzburg bleb classification score ≥ 8 points and ≥ 7 points (kappa 0.24 and 0.27, respectively). Analyzing the subgroups of the morphological criteria, best agreement was found between occurrence of microcysts and target intraocular pressure (к 0.22-0.34). CONCLUSIONS: Evaluating filtering blebs after trabeculectomy by using the Wuerzburg bleb classification score is a good technique for predicting intraocular pressure control in eyes attaining a minimum score of seven points. The presence of microcysts on the filtering bleb predicts that the eye is likely to attain target pressure.

The prognostic value of the wuerzburg bleb classification score for the outcome of trabeculectomy.

BACKGROUND: the Wuerzburg bleb classification score (WBCS) aims at an objective and standardized assessment of the developing filtering bleb after trabeculectomy, in order to detect and treat bleb scarring at the earliest possible stage of development. The purpose of this retrospective study was to evaluate the prognostic value of the early postoperative WBCS for the long-term outcome of trabeculectomy. METHODS: the WBCS is a grading system for clinical bleb morphology. It evaluates the following parameters: vascularization, corkscrew vessels, encapsulation and microcysts. The WBCS of 113 eyes of 113 consecutive patients after trabeculectomy was determined 1 day, 1 and 2 weeks, 3, 6 and 12 months after surgery. Complete success was defined as an intra-ocular pressure (IOP) <21 mm Hg and >20% pressure reduction without glaucoma medication after 1 year. RESULTS: a complete success rate of 73.9% and a qualified success rate of 82.4% were achieved 1 year after surgery. The average total bleb score during follow-up in the success group was always higher than in the failure group, but there was no statistically significant difference at any time. The bleb average score for eyes with an IOP of ≤ 12 mm Hg after 2 weeks was significantly higher (p = 0.005) than for eyes with an IOP ≥ 13 mm Hg at the end of follow-up. CONCLUSION: patients with a higher early WBCS postoperatively had a significantly lower IOP 1 year after surgery. However, the study could not reveal a certain prognostic value of the early total bleb score using the WBCS for the long-term complete success of trabeculectomy.

Caracterización de ampollas / Characterization of blisters

Se realiza una revisión bibliográfica sobre una de las lesiones elementales de la piel más frecuentemente encontradas en la práctica médica: las ampollas; con el propósito de que constituya un referente para estudiantes y profesionales de la salud, que sirva como incentivo para que profundicen en el estudio de las mismas. Se abordan algunos aspectos de gran interés, con énfasis en los diferentes tipos de ampollas, algunas consideraciones teóricas relacionadas con la propedéutica dermatológica y las enfermedades ampollares. Se detalla sobre su reconocimiento, clasificación, y semiogénesis, pues para un correcto diagnóstico y pronóstico es indispensable un examen físico minucioso (AU)

Correlation between the indiana bleb appearance grading scale and intraocular pressure after phacotrabeculectomy.

PURPOSE: To investigate the relationship between bleb morphology, recorded using the Indiana Bleb Appearance Grading Scale (IBAGS), and intraocular pressure (IOP) after phacotrabeculectomy. METHODS: Two years postphacotrabeculectomy, a single observer compared bleb morphology to the IBAGS standard photographs in 76 eyes of 76 patients. In addition, the presence or absence of microcysts was recorded. IOP was also measured. RESULTS: On multivariate analysis increasing bleb height was associated with a low IOP (P=0.017). An increase in IBAGS height score by 1 U resulted in a reduction in IOP of 2.16 mm Hg (95% confidence interval=0.40-3.92 mm Hg). In this study, there was no association between vascularity, bleb extent or microcysts, and IOP. There were no cases of bleb leak in this series. CONCLUSIONS: Two years postphacotrabeculectomy increased bleb height, as measured by the IBAGS, was associated with a decrease in IOP, with a 1 point increase in IBAGS height score resulting in a decrease of 2.16 mm Hg. We found no association between bleb extent, vascularity, or the presence or absence of conjunctival microcysts. As there were no cases of bleb leak in this study this characteristic could not be evaluated.

Interobserver variability of the Würzburg bleb classification score.

BACKGROUND: The Würzburg bleb classification score (WBCS) serves to assess filtering blebs in a standardized fashion. The purpose of this prospective masked agreement study was to evaluate the WBCS interobserver variability. METHODS: The WBCS provides a scheme to grade clinical bleb morphology. It evaluates the following parameters: vascularity, corkscrew vessels, encapsulation, microcysts and bleb height. Thus, 113 eyes of 104 consecutive patients at various times after surgery were examined (slit lamp biomicroscopy) by 3 ophthalmologists with each observer being unaware of the findings reported by the others. To calculate the interobserver variability of the WBCS, the interobserver consistency and absolute agreement were determined with an intraclass correlation coefficient (ICC) using a 2-way random model. RESULTS: The ICC values of a single rater's judgment were: vascularity +0.62, corkscrew vessels +0.67, encapsulation +0.63, bleb height +0.53, microcysts +0.52 and total score +0.74. The ICC values of the mean of all 3 raters were: +0.83 vascularity, +0.86 corkscrew vessels, +0.84 encapsulation, +0.77 bleb height, +0.76 microcysts and +0.90 total score. CONCLUSION: The WBCS is a bleb morphology score with high levels of interobserver consistency and absolute agreement in clinical practice.

Hemorrhagic bullae represent an ominous sign for cirrhotic patients.

Hemorrhagic bullae are a clinical manifestation of many underlying diseases, especially soft-tissue infection. The aim of this study was to evaluate the characteristics and prognosis of cirrhotic patients with hemorrhagic bullae. Fifteen patients with liver cirrhosis and hemorrhagic bullae had been admitted to Chang Gung Memorial Hospital, Kaohsiung, from January to December 2003. Their clinical courses were retrospectively reviewed in detail and all of the collected data were analyzed. This study puts emphasis on the clinical presentation and outcome of these cases. The mean age of patients was 55.0 +/- 12.1 years, and 12 patients were male. Prostration and unusual extremity pain were the two leading reasons to visit our Emergency Department. The hemorrhagic bullae were located on the upper or lower extremities and in one patient, on the whole body. In this series all hemorrhagic bullae were infection-related. Although aggressive treatment was started immediately upon arrival, 14 patients died of overwhelming sepsis and 12 patients died within 48 h from the emergence of hemorrhagic bullae. Hemorrhagic bullae in cirrhotic patients usually imply a fatal infection and Gram-negative bacteria are the most common pathogen. Appropriate antimicrobial therapy and early surgical intervention are necessary to achieve survival in these patients.

New simple classification for operated bullous emphysema.

OBJECTIVES: Optimal results in bullectomy depend on both the size of the resected bulla volume and the reexpansion of the adjacent collapsed healthy pulmonary parenchyma. We hypothesized that the bigger the bulla is compared with residual volume, the greater are the possible benefits. We suggested a new prognostic classification according to bulla volume and its relationship with residual volume. METHODS: We retrospectively reviewed 121 patients with emphysematous bulla (>200 mL) who, from 1996 to 2006, underwent unilateral single (n = 64), unilateral multiple (n = 16), bilateral 1-stage (n = 9), and bilateral 2-stage (n = 32) bullectomies. Bulla volume and residual volume were measured by computed tomography and body plethysmography, respectively. Six-month postoperative decrement of residual volume values and their persistence below the baseline for 5 years were considered primary outcomes. Logistic regression was used to select significant variables. The receiver operating characteristic curve was used to identify the cutoff point for a possible classification system. RESULTS: There was no postoperative mortality. Significant postoperative improvements in respiratory function were found and correlated with bulla size. Residual volume improved in 75 patients (62%) and persisted in 20 patients (35% of the patients followed for > 5 years). Logistic regression selected bulla/residual volume ratio as the most predictive variable for both outcomes (P < .0001). The best cutoff individuated by the receiver operating characteristic curve analysis was 20% to achieve a high probability of residual volume improvement and 30% to minimize residual volume recurrence. CONCLUSIONS: Bullectomy provides good results, but more significant and long-lasting improvements are achievable with a greater ratio bulla/residual volume: scant for less than 20%, good but temporaneous for 20% to 30%, and good and long-lasting results for more than 30%.

Information loss in 2 bleb grading systems.

PURPOSE: To assess the effectiveness of transferring descriptive information from bleb photographs to 2 recently described bleb grading systems: the Moorfields Bleb Grading System (MBGS) and the Indiana Bleb Appearance Grading Scale (IBAGS). METHODS: Two experienced observers graded 51 clinical bleb photographs with a wide range of appearances using both the MBGS and IBAGS bleb grading systems in random order. Grading scores from the 2 observers were averaged, and these numbers used by a third investigator, who did not view the original photographs, to generate 102 sketched representations of the blebs. The sketches were labeled randomly, and 1 month later presented individually in random order, to mask which grading system was used as source data for each drawing. MAIN OUTCOME MEASURES: The original graders then used an arbitrary 1-5 scale to rate congruity between sketches and photographs for vascularity and morphology features, and overall agreement of the bleb sketches. RESULTS: For both the IBAGS and MBGS, interobserver agreement between the Congruity Scores (CS) of the 2 masked graders was excellent, ranging between 92% and 98% for each parameter. Overall CS results were 3.2 (good-very good) for IBAGS and 4.1 (very good-excellent) for MBGS. Vascularity CS scores from IBAGS were 3.0 (good) and those from morphology agreement averaged 3.5 (good-very good). For the MBGS, the respective results were 3.9 (good-very good) and 4.1 (very good-excellent), respectively. Photographic quality (P=0.012) and presence of a limbus-based conjunctival flap scar (P=0.012) had an influence on CS scores from IBAGS but not from MBGS. CONCLUSIONS: Both the IBAGS and MBGS produced acceptable agreement ratings between the sketches derived from grading system data and the original bleb photographs. These grading systems seem to adequately represent the blebs that are being encoded, without significant information loss from the simplification and translation process. The MBGS tended to have higher CS, and may be less influenced by photograph quality and bleb type, suggesting that bleb photographs may be best encoded for statistical analysis in clinical studies using this system.

Comparison of two clinical Bleb grading systems.

PURPOSE: To evaluate 2 recently described grading systems for clinical grading of filtering surgery blebs: the Moorfields Bleb Grading System (MBGS) and the Indiana Bleb Appearance Grading Scale (IBAGS). DESIGN: Observational comparative study. PARTICIPANTS: Twenty-four glaucoma filtering blebs in 24 eyes of 17 patients. METHODS: Three observers in a prospective agreement study compared MBGS with IBAGS during slit-lamp examination. MAIN OUTCOME MEASURES: Comparison analyses were performed, including agreement, repeatability, and intraclass correlation coefficient (ICC). RESULTS: A wide range of bleb characteristics was represented in the cohort. Acceptable levels of intrasystem agreement were found in both systems: for IBAGS, overall agreement at the 0.5- and 1.0-unit levels were 80.6% and 97.6%, respectively, and for MBGS, these were 78.4% and 97.4% for morphologic and vascularity indices. Repeatability coefficients ranged from 0.5 to 1.4 for MBGS and 0.8 to 1.2 for IBAGS. The ICC values in the MBGS ranged from 0.18 to 0.72 for single measures and 0.39 to 0.88 for average measures. For IBAGS, the single-measure ICC values were between 0.06 and 0.53, and the average-measure ICC values were between 0.16 and 0.77. The MBGS ICC values for bleb size were higher than for IBAGS. CONCLUSIONS: Both methods are reproducible clinically and had generally high levels of interobserver agreement. Both have minor deficiencies that should be amenable to improvement. The MBGS performed similarly to the IBAGS for reproducibility, had higher ICC values for morphologic features, and captured extra vascularity data with probable clinical implications.

Treatment of a heel blister caused by pressure and friction.

The treatment of heel blisters caused by pressure, friction, and shear is a complex subject. Issues such as whether to aspirate the blister, how to protect it from further pressure, friction, and shear, and which dressing to choose, need to be considered. As nurses are increasingly aware of the emphasis that has been placed on the importance of ensuring that clinical practice is based on evidence, this article attempts to shed some light on these areas of debate. It is important to highlight that during the literature search very little research was found to exist on blister care and it became clear that further research into the nursing care of a blister caused by pressure would be useful to aid nursing care and patient comfort.

Deroofing minor burn blisters--what is the evidence?

It is estimated that 150000 new patients per year will attend an A&E Department or Minor Injury Unit (MIU) with a minor burn injury (Wardrope & Edhouse 1999). There is often conflicting 'evidence' for the management and treatment of minor burns blisters and this can cause variations in the treatment provided within an A&E department resulting in confusion for staff and patients.

End of the century overview of skin blisters.

A wide spectrum of diseases of the skin are manifested as a blistering process. Blistering may occur as a secondary event associated with viral or bacterial infections of the skin, eg, herpes simplex and impetigo, or with local injury of the skin, eg, burns, ischemia, and dermatitis. In other diseases, blistering of the skin occurs as a primary event and is associated with tissue injury and fluid accumulation within a specific layer of the skin: intraepidermal, dermal-epidermal junction, or subepidermal. Blister formation in this latter group of diseases is due to either genetic mutation or an autoimmune response. Genodermatoses associated with blisters are typically manifested in the neonate, whereas the autoimmune blistering disorders are acquired and usually expressed later in life. Recent advances have uncovered the relevance of the keratinocyte cytoskeleton, the desmosome, the hemidesmosome, and extracellular matrix proteins in blister formation. A pathogenetic classification of blistering diseases is presented.

Immune-mediated subepithelial blistering diseases of mucous membranes. Pure ocular cicatricial pemphigoid is a unique clinical and immunopathological entity distinct from bullous pemphigoid and other subsets identified by antigenic specificity of autoantibodies.

BACKGROUND AND DESIGN: There is much confusion in the clinical classification of immune-mediated subepithelial blistering diseases of mucous membranes. We conducted a 6-year comprehensive study to better classify this heterogeneous disease group. Indirect immunofluorescence was performed on a salt-split-skin substrate to detect circulating antibasement membrane antibodies (n = 47). Serologic reactivity against cultured keratinocyte antigens was examined by immunoblots (n = 38) and immunoprecipitation (n = 15). The results were correlated with the clinical features and direct immunofluorescence data of the entire patient group (n = 87) without preassignment of clinical diagnoses. chi 2 Statistical analyses compared these results with those of the classic bullous pemphigoid group (n = 36). RESULTS: When compared with the bullous pemphigoid patients, a subset of patients with combined oral mucosal and skin lesions demonstrated marked similarity in direct and indirect immunofluorescence findings and in serologic reactivity to bullous pemphigoid antigens. By contrast, a subset of patients with only ocular lesions exhibited significantly lower in vivo deposits of IgG and C3, higher deposits of fibrin, virtual absence of circulating antibodies, and negative serologic reactivity to bullous pemphigoid antigens. CONCLUSIONS: Ocular patients without skin or mouth lesions, in particular those with negative indirect immunofluorescence, should be distinctively classified as ocular cicatricial pemphigoid, a unique clinical and immunopathologic entity. Patients with mucous membrane involvement who also demonstrate skin lesions and antibodies to the root of salt-split-skin substrate should be classified as anti-BP Ag mucosal pemphigoid, even though they may exhibit severe oral and/or ocular diseases. The remaining mucous membrane patients are heterogeneous. Some can be classified on the basis of autoantibodies to other basement membrane determinants, or if serum autoantibody negative, on the basis of clinical features (ie, pure oral mucosal pemphigoid or overlapping mucosal involvement).

Cytoskeletal injury and subsarcolemmal bleb formation in dog heart during in vitro total ischemia.

In previous studies a two-step hypothesis explaining the mechanism of lethal ischemic injury to cardiac myocytes has been advanced. It proposes that damage to the myocyte cytoskeleton precedes, and predisposes the cell to, mechanical injury induced by cell swelling or by ischemic contracture. This study quantitated the prevalence of breakage of the major cytoskeletal attachment between the plasmalemma and peripheral myofibers as a function of the duration (0-180 minutes) of in vitro total ischemia in dog heart papillary muscle. Breakages of Z-band, plasmalemmal attachment complexes were few before 120 minutes of ischemia, but thereafter became more prevalent; the transition between the initial rate of appearance of the breaks and the later fast rates coincided with the appearance of severe cell swelling, ischemic contracture, and ultrastructural criteria of irreversible ischemic injury. Z-band, plasmalemmal attachment complex breakage and cell swelling resulted in formation of subsarcolemmal blebs. Two major bleb types have been discerned on ultrastructural appearance using as the criteria the preservation of integrity of the plasma-lemma and subplasmalemmal leptomeres. The identification of two types of blebs suggests two independent mechanisms of injury, the first directed at Z-band attachments, and the second at the cytoskeletal structures of A- and I-band regions of the plasmalemma.