Iga-mediated Epidermolysis Bullosa Acquisita Two Cases and Review of the Literature

Actas Dermo-Sifiliográficas (English Edition)

ISSN: 1578-2190

The Official publication of the Spanish Academy of Dermatology and Venereology (AEDV). Actas Dermo-Sifiliográficas, founded in 1909, is the oldest monthly medical journals published in Spain. In the year 2006 has been indexed in the Medlinedatabase, and has get a vehicle for expressing the most current Spanish medicine and modern. All articles are subjected to a rigorous process of revision in pairs, and careful editing for literary and scientific way. Together with the classic Original and Clinical Case Study sections, we also include Reviews, Example Diagnoses, and Book Reviews. Dermo-Sifiliográficas is an essential publication for anyone who needs to exist current on all aspects of Spanish and globe dermatology.

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Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering illness acquired by autoantibodies to type VII collagen. The clinical presentation is variable, with skin and mucosal lesions that can cause meaning dysfunction. Different treatment options exist, merely the results are oft unsatisfactory.

Objective

To review all the cases of epidermolysis bullosa acquisita (EBA) diagnosed at our hospital over a 26-year flow.

Materials and methods

Nosotros performed a retrospective review of the clinical, histologic, and immunologic features of EBA in 9 patients.

Results

Hateful age at presentation was 37 years and 66.67% of the patients were women. EBA occurred in association with malignant tumors, inflammatory bowel illness, and autoimmune disorders. The most common variant was inflammatory EBA (vi of the nine cases). In all 9 patients, histology revealed a subepidermal cicatrice and straight immunofluorescence showed linear deposits of immunoglobulin G and C3 in the basement membrane zone. Indirect immunofluorescence performed on salt-split skin substrate was positive in 6 patients and showed a dermal pattern in all cases. Five patients were tested for autoantibodies to type VII collagen using enzyme-linked immunosorbent assay, with positive results in 2 cases. Immunoblotting using recombinant noncollagenous domains (NC1) of type Seven collagen was positive in all 6 cases in which it was performed. Response to treatment was variable.

Conclusions

EBA is a rare disease with a variable clinical presentation that can be confused with that of other subepidermal baking diseases. Correct diagnosis requires a high level of clinical suspicion and the use of all bachelor diagnostic tests. Thorough evaluation of cutaneous and mucosal involvement and prompt initiation of appropriate treatment will ensure the detection and prevention of dysfunction and treatment-related complications.

Keywords:

Epidermolysis bullosa acquisita

Autoimmune subepidermal bullous disease

Resumen

Introducción

La epidermolisis ampollosa adquirida es una enfermedad ampollosa subepidérmica autoinmune causada por autoanticuerpos contra el colágeno 7. Su clínica es heterogénea con afectación de piel y mucosas pudiendo generar secuelas invalidantes. Existen diversas opciones terapéuticas frecuentemente insatisfactorias.

Objetivo

Revisar los casos de epidermolisis ampollosa adquirida diagnosticados durante un periodo de 26 años.

Material y métodos

Estudio retrospectivo de las características clínicas eastward inmunopatológicas de 9 pacientes con dicho diagnóstico

Resultados

La mediana de edad de presentación fue de 37 años, el 66.67% de pacientes fueron mujeres. Asociaciones: neoplasias malignas, enfermedad inflamatoria intestinal y procesos autoinmunes. La variante inflamatoria fue la más frecuente (6/nine). La histología mostró constantemente una ampolla subepidérmica y la inmunofluorescencia directa la presencia de depósitos lineales de IgG y C3 en membrana basal. La inmunofluorescencia indirecta fue positiva en 6 pacientes, mostrando en todos ellos un patrón dérmico en piel separada. En 5 pacientes se determinaron los anticuerpos contra el colágeno VII por Enzyme-Linked Immuno Sorbent Assay de los cuales 2 fueron positivos, eastward Inmunoblot con NC1 recombinante en 6 casos, positivo en todos ellos. La respuesta terapéutica fue variable.

Conclusiones

Se trata de una enfermedad rara, de clínica heterogénea que puede inducir a confusión con otras enfermedades ampollosas subepidérmicas. Se requiere un alto índice de sospecha y el empleo de todos los métodos disponibles para establecer su diagnóstico. La correcta evaluación de la afectación cutáneo-mucosa y la instauración precoz de la terapéutica adecuada permitirá la detección de sus secuelas y de las complicaciones del tratamiento.

Palabras clave:

Epidermólisis ampollosa adquirida

Enfermedad ampollosa subepidérmica autoinmune

Full Text

Introduction

Epidermolysis bullosa acquisita (EBA) is a chronic subepidermal bullous disease of the cutaneous and mucosal tissues.i–3 EBA was originally described by Roenigk and colleaguesone in 1971 equally a mechanobullous disorder associated with peel fragility. Although similar to congenital epidermolysis bullosa, EBA appears in adults with no family history. In 1973 Kushnirukiv reported that direct immunofluorescence (DIF) revealed immunoglobulin (Ig) G and C3 deposition in the basement membrane of tissue samples from patients with EBA. In 1981 Yaoita and colleaguesv used immunoelectron microscopy techniques to demonstrate the location of deposits in the sublamina densa in EBA and in the lamina lucida in bullous pemphigoid. In 1984 Woodley and colleaguessix identified a 290-kDa poly peptide as the target antigen in EBA; this poly peptide was afterwards identified every bit type Vii collagen.vii An inflammatory variant of this disorder was then described; clinical characteristics were similar to those of bullous pemphigoid and other pemphigoid subtypes, mimicking mucosal pemphigoid or Brunsting-Perry cicatricial pemphigoid.8–10 Features of both weather condition may be nowadays at one time or the clinical picture may shift toward ane or the other during the class of illness.11

EBA is an uncommon affliction with an estimated incidence of 0.2 cases per meg inhabitants.2,3 The low prevalence has meant that no racial predilection has been established, although black patients of African descent were reported to be overrepresented in one patient serial12 and higher prevalence has also been described for Korean populations.xiii Historic period at onset is usually betwixt 40 and 50 years, although EBA has been found in patients of advanced age and in children.14,15 Vertical manual from a mother with EBA to an infant has also been reported.16

The etiology is unknown merely it seems clear that autoantibodies to type Vii collagen play a office in the pathogenesis of this disease. The relevance of these antibodies has been demonstrated by the evolution of blisters in an infant equally a effect of the transfer of antibodies from maternal claret to the fetus.sixteen Ex vivo evidence comes from the induction of dermal-epidermal separation, with neutrophil recruitment, in frozen sections of human skin exposed to sera from patients with EBA.17 Experimental in vivo models of EBA accept been based on passive immunization (transfer to mice of IgG antibodies to type VII human or rabbit collagen)xviii or active immunization (of sure mice strains with immunodominant fragments in the NC1 domain of type VII collagen).nineteen

The classic mechanobullous form of EBA is characterized histologically by the presence of subepidermal blisters with scant inflammatory infiltrate. Inflammatory EBA is marked past a more intense infiltrate containing neutrophils in abundance and occasionally eosinophils. DIF reveals linear, predominantly IgG deposition along the dermal-epidermal basement membrane. C3, IgA, or IgM deposition of variable intensity is observed in some cases.4,5,20 Indirect immunofluorescence (IIF) of sera from some patients may detect the presence of circulating IgG autoantibodies that target the dermal-epidermal basement membrane. It is possible to utilize different substrates, such every bit human skin or monkey esophageal tissue,5 but the best results are obtained using human skin separated in a 1.0-Grand sodium chloride (NaCl) solution. The use of common salt-split skin enables detection of circulating IgG autoantibodies that demark to the dermal (base) side of the separation, a finding that distinguishes EBA from bullous pemphigoid, in which these autoantibodies bind to the epidermal (roof) side. Although this is a relatively unproblematic way to differentiate these 2 diseases, it will not distinguish EBA from other autoimmune subepidermal bullous diseases with a dermal pattern, such as bullous systemic lupus erythematosus (BLSE) or various forms of pemphigoid associated with anti-laminin 332, anti-laminin γ1 or anti-laminin-p 105).21 When IIF is negative, DIF can be used after inducing separations in biopsy fabric (with the 1.0-Yard NaCl solution); alternatively, a suction-induced cicatrice tin can be biopsied for DIF. DIF will also pinpoint the location of IgG deposits in the dermal portion of the separation in EBA. However, like IIF, this technique will not differentiate between EBA and other diseases with a dermal blueprint.22 The diagnostic value of the pattern of autoantibody deposition in the membrane has recently been described. When IgG deposits class above the sublamina densa (in bullous pemphigoid and anti-lamina 332 pemphigoid), an northward-serrated pattern is typical; when deposits are in the sublamina densa (every bit in EBA), a u-serrated pattern forms; for this technique sections must be of less than fourμm.23

Immunoblotting of serum from patients with EBA using dermal extracts identifies protein bands of approximately 290 kDa and 145 kDa, corresponding to complete (dimeric) type VII collagen or i of its regions (monomeric). Most antigen epitopes of collagen Seven are located in the NC1 domain (145 kDa). Therefore, recombinant NC1 protein produced in the laboratory can also be used in immunoblot analysis or in classic enzyme-linked immunosorbent assay (ELISA). ELISA using recombinant NC1 is considerably more than sensitive and specific than IIF on a split-skin substrate or immunoblotting with dermal extracts; ELISA as well quantifies autoantibodies.24 A recently desribed technique using complete type VII collagen (containing both the NC1 and NC2 domains) has been shown to be slightly more than sensitive than the technique using merely NC1.25

Immunoelectron microscopy is the gold standard diagnostic method because information technology identifies the location of immunoglobulin deposition in the sublamina densa in EBA and in the lamina lucida and hemidesmosomes in bullous pemphigoid.5,20 Nevertheless, this is a circuitous technique that is unavailable in many centers.

EBA can be diagnosed with a off-white caste of certainty past correlating clinical findings with the results of the various diagnostic tools. However, information technology cannot be distinguished from BSLE in this way, since pathogenic autoantibodies to type VII collagen are nowadays in both diseases.26 For a diagnosis of BSLE, the American College of Rheumatology (ACR) criteria must be met. BSLE lesions are mainly located in skin areas exposed to sunlight, and this disease is less refractory to treatment than EBA.27

Various therapies for EBA have been tried and accept often proven ineffective; in any case, response is unpredictable. Few big patient series have been published and no randomized clinical trials have been performed considering the disease is then rare.3,28

Nosotros analyzed the demographic, clinical, and immunopathologic characteristics of EBA as well as response to therapy in a series of patients diagnosed between 1985 and 2011 in our infirmary.

Patients and Methods

This retrospective study included patients diagnosed with EBA in Hospital Clínic de Barcelona between 1985 and 2011. Diagnosis was based on clinical, histologic, and immunopathologic evidence. We analyzed all demographic, clinical, immunopathologic, and therapeutic data on record.

For IIF studies we used common salt-split human being skin prepared conventionally by incubation in a 1.0K NaCl solution; patient serum samples for studies were obtained during the active phase of disease.21 For DIF nosotros used the method described by Gammon and colleagues.22 Immunoblotting was performed using dermal extracts and, in some cases, recombinant proteins of the NC1 domain of type 7 collagen were used. ELISA was performed on domain-specific recombinant proteins (His-hCVII-NC1 and NC2-His-hCVII-NC1 NC2-H) of type Vii collagen, as described elsewhere.25

Results

We identified 9 patients diagnosed with EBA between 1985 and 2011. The mean age was 37 years; 3 were men and 6 women (male:female ratio, 1:ii). The median time betwixt onset and diagnosis was 7 months (range, 1–72 months).

Most of the patients (6 out of 9) presented with the inflammatory form (Fig. 1). The classic mechanobullous form (Fig. ii) was seen in patients of more than advanced age: all patients with this form were over 72 years old. In 1 patient (Instance v, Tabular array 1), the clinical phenotype inverse from bullous pemphigoid to Brunsting-Perry pemphigoid. Disease was confined to the skin in merely ii patients. All of the vii patients with mucosal involvement had oral lesions; additionally, 2 had lesions on the genitals, two had conjunctival interest, and 1 had lesions on the pharynx, larynx and esophagus.

Malignant disease was a frequent association, with 5 tumors in 3 patients. 2 tumors were in the lung, 1 was on the cervix, ane in the breast, and 1 in the peel (melanoma). Other associations were inflammatory bowel disease (ane instance of Crohn disease and 1 of ulcerative colitis) and autoimmune diseases or a finding of circulating antibodies (1 hemolytic anemia, one optic neuritis, 1 thyroiditis, 4 positive for antinuclear antibodies [ANA]) (Table one).

Histology showed a subepidermal blister with scant inflammatory infiltrate for all three patients with archetype EBA. In the 6 patients with inflammatory EBA, histology detected an inflammatory infiltrate with abundant neutrophils and eosinophils, and occasional microabscesses in the papillary dermis. Eosinophilic spongiosis was observed in ii cases. DIF consistently showed linear IgG and C3 deposits of variable intensity along the basement membrane(Fig. 3). Other conjugates were detected in 5 patients (56%) (Tabular array ane). IIF was performed with human salt-split peel and the serum samples from 8 patients; in half dozen of these patients (75%) the result was positive and a dermal design was detected (Fig. iv). For 1 patient (case half dozen, Table 1), IIF performed in another centre merely detected the presence of ANA; serum was not available to us for IIF in salt-split homo skin. Results of DIF on salt-split pare showed an exclusively dermal IgG degradation pattern for all half dozen patients in whom this test was performed; this group included 2 patients with negative IIF results and a patient for whom IIF was not performed.

Type Vii collagen ELISA was performed in 5 cases and was positive in two (40%). Immunoblot analysis was performed in 6 cases and was positive in 5 (83%) (Fig. 5). These results are summarized in Table 1. ANA were detected in iv patients (44%). In 1 case (patient 6, Table 1), this was an isolated finding. In 2 other cases (patients 3 and seven, Table 1) autoimmune diseases were also found. Although patient 8 (Table 1) was positive for ANA and anti-DNA, the ACR criteria for a diagnosis of BSLE were never met at whatever fourth dimension during the course of disease. This patient was unresponsive to high doses of corticosteroids and dapsone just showed a good response to intravenous immunoglobulin (IVIG) therapy and colchicine. These outcomes suggested a diagnosis of EBA rather than BSLE even though the two processes have like immunologic features and overlap to a certain extent.

After diagnosis, a diversity of treatments were tried; response was variable in about patients and disease was difficult to command in two cases (patients 3 and five, Tabular array 2). Patient 3 started handling with prednisone at a daily dose of 1mg/kg, achieving partial response. Colchicine at a dose of 1mg every 12h was added, followed by dapsone (100mg/d), to little consequence. Loftier-dose IVIG therapy (2g/kg) was therefore started. At that place was still no response, only after prednisone and colchicine were added to the regimen, partial response was finally achieved. During the course of affliction, pulmonary adenocarcinoma was detected; metastasis developed and the patient died. The case of patient 5 was the almost refractory to therapy. Various treatments were tried and withdrawn, either because of agin events or lack of response. In December 2007, rituximab was started (375mg/chiliad2/wk for 4 weeks). There was proficient response to this regimen in the skin, but gingival erosion remained a problem. At the time of writing, the patient was in complete remission on low-dose corticosteroids, dapsone, and colchicine. Nosotros emphasize the excellent response to IVIG therapy in a patient with the classic grade of EBA (patient 1, Table two) considering lesions had been refractory to all the treatments that had been tried previously.

Discussion

EBA is an uncommon disease that mainly affects adults.1–3 The median age at diagnosis in our series was 37 years; women predominated, equally in the 4 previously published studies.29–32

The classic mechanobullous form of EBA is the one that was first reported. This class has quite characteristic clinical features that facilitate diagnosis, undoubtedly explaining why about of the initial publications were nearly this phenotype. Since the inflammatory diversity was described, more cases of this form have emerged. It is possible that greater agreement of EBA and appropriate use of the diagnostic techniques now bachelor take enabled the diagnosis of cases that might previously take been thought to exist bullous pemphigoid. The inflammatory grade was more frequent in our series, equally in other recently published ones. All the same the classic grade predominated in our older patients, an ascertainment that was not described in other series.29–32 We cannot explain this finding and the small number of patients precludes drawing conclusions. Information technology is well known that EBA is oft institute in clan with other diseases. The about conspicuously established association is probably with inflammatory bowel disease, particularly Crohn affliction.1,3 The presence of type 7 collagen in the intestinal epithelium and the phenomenon of epitope spreading could explicate this association; this hypothesis is supported by the presence of circulating antibodies that target this collagen in approximately 68% of patients with Crohn disease even when no skin signs are present.33,34 In our series the incidence of tumors was higher in association with inflammatory bowel illness. Single cases of EBA associated with tumors have been reported, but the clan has not been reported in any patient series until now. The course of disease in our patients did non seem to suggest a paraneoplastic procedure. In fact, the three tumors that had been diagnosed before the onset of EBA were in remission. In patient iii, diagnosis was delayed 72 months from onset and the tumors were detected at the same time; she did not receive immunosuppressants that might have triggered development of the tumor. In patient two, the tumor presented after complete remission of EBA, which had been treated only with colchicine.

The degree of clinical inflammation correlated with the intensity of the inflammatory infiltrate, consistent with the literature on histologic findings in this illness. DIF did non let us to differentiate EBA from bullous pemphigoid, even though we often observed that IgG deposits were more intense than C3 deposits in our EBA patients (in contrast with the more than intense C3 deposition reported in pemphigoid35). The diagnostic method with the highest sensitivity was DIF with salt-split skin, which showed a dermal blueprint in all cases where it was used. IIF with salt-carve up skin was negative in simply two patients. The immune-deposit patterns revealed by DIF—either northward-serrated or u-serrated—could not be assessed in our series, possibly because of technical problems related to the thickness of sections. We must point out that the results of the tests done in patient 5 did not lead to a definitive diagnosis of EBA. Nosotros cannot rule out that this slowly progressing example, which was refractory to treatment, was not IgA-mediated EBA because the appropriate ELISA was not done for this patient, who had associated Crohn disease and mucosal involvement and was negative for anti-BP 180 antibody. IgG deposition in the basement membrane, however, was much more intense than either IgA or C3 deposition.

Response to therapy was highly variable and not ever satisfactory. We underline the excellent results obtained with IVIG infusion in a woman with the classic course of EBA who had non responded to several previous treatments (Table two). The approach to EBA treatment we would suggest based on our experience would exist to commencement prednisone (or an equivalent) at 0.5mg/kg in association with colchicine (ane–2mg/d) and/or dapsone (25–100mg/d). If the patient responds well enough to allow the prednisone dose to be tapered to 5mg (or discontinued altogether), the corticosteroid therapy can exist maintained. If the prednisone dose cannot exist reduced (despite the addition of up to 100mg of dapsone and twomg of colchicine) we propose calculation IVIG therapy. Rituximab currently seems to be more than effective at a lower price, and this drug could exist a good choice in cases that are refractory to other treatments. However, our experience with rituximab in EBA is express to a single patient.

In summary, we present the kickoff Spanish series of EBA patients. Consequent with the only other 4 series published29–32 (analyzing 38, 30, fourteen, and 12 patients, respectively), nosotros found that inflammatory EBA predominated. In EBA, clinical suspicion must be high and optimal use of available diagnostic tools will be necessary for reaching a house diagnosis. Lehman and colleagues36 proposed a diagnostic algorithm for autoimmune bullous diseases that took into consideration the cost of the diverse tests so that they can be used rationally. After diagnosis, the extent of illness and the possibility of associated conditions must exist carefully assessed, and advisable treatment should be started so that interest of the center and mucosae of the respiratory and digestive tracts can exist prevented. Considering this disease is highly variable in presentation, information technology is difficult to evaluate response to a detail drug.

This retrospective study included a small number of patients. Prospective multicenter studies are needed to improve our understanding of the mechanisms involved in EBA, to assess the efficacy of the drug treatments available, and to provide a basis for tailoring handling.

Ethical Disclosures Protection of human and animal subjects

The authors declare that no experiments were performed on humans or animals for this investigation.

Confidentiality of information

The authors declare that they have followed the protocols of their hospitals concerning the publication of patient data, and that all the patients included in this report were accordingly informed and gave their written informed consent.

Right to privacy and informed consent

The authors declare that no private patient data are disclosed in this article.

Conflicts of Involvement

The authors declare that they have no conflicts of interest.

Acknowledgments

Nosotros thank Dr C. Sitaru, who performed the blazon 7 collagen ELISA assays, and Drs J. Herrero and A. Guilabert, who performed the immunoblotting analyses.

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Epidermolysis bullosa acquisita: Concise review and practical considerations.

Int J Dermatol, 48 (2009), pp. 227-233

Delight cite this article as: Barreiro-Capurro A, Mascaró-Galy JM, Iranzo P. Estudio retrospectivo de las características clínicas, histológicas e inmunológicas en una serie de de 9 pacientes con epidermólisis ampollosa adquirida. Actas Dermosifiliogr. 2013;104:904–914.

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Source: https://www.actasdermo.org/en-estudio-retrospectivo-las-caracteristicas-clinicas-articulo-resumen-S1578219013002412

Iga-mediated Epidermolysis Bullosa Acquisita Two Cases and Review of the Literature

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