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@#INTRODUCTION: Linear IgA bullous disease (LABD) is a rare autoimmune blistering disease characterized by subepithelial bullae and linear IgA deposition along the basement membrane zone of the epidermis. Lesions present as pruritic papulovesicles and tense bullae which may coalesce forming annular or polycyclic urticarial plaques with blistering on the edge of the lesions forming the classic “string of pearls” sign. Lesions may affect the face, trunk, and extensor extremities. Incidence rates range from 0.5 to 2.3 cases per million individuals per year. Due to its rare occurrence, there are only a fewdocumented reports on cases of LABD, particularly in the Filipino population. CASE REPORT: A 33 year-old Filipino female consulted because of a 3-week history of severely pruritic vesicles and crusts on the face, trunk, and arms. Patient noted no gastrointestinal symptoms on consultation. Skin punch biopsy revealed subepidermal blisters with collection of neutrophils at the dermal papillae. Direct immunofluorescence showed strong (+2) deposits of linear IgA at the dermo- epidermal junction in perilesional skin thus validating the diagnosis. The patient’s serum was negative for IgA anti-tissue transglutaminase and IgA antiendomysial antibodies. Patient was treated with topical corticosteroids and Dapsone 50 mgs OD with remarkable improvement. CONCLUSION: Linear IgA bullous disease has very few reported cases especially in the Philippine setting. Dapsone is considered the first-line treatment. The disease may persist for a decade or longer, and relapses may occur. Careful history-taking accompanied by the histological, immunofluorescence, and serological findings must be done to ensure proper treatment and good prognosis.
Subject(s)
Dermatitis Herpetiformis , Linear IgA Bullous Dermatosis , Immunoglobulin AABSTRACT
Objective To evaluate the value of indirect immunofluorescence (IIF) on three different substrates including normal human skin (NS),monkey esophagus (ME) and salt-split human skin (SS) in the diagnosis of autoimmune subepidermal bullous diseases.Methods A total of 56 patients with autoimmune subepidermal bullous diseases,including 47 with bullous pemphigoid (BP),6 with epidermolysis bullosa acquisita (EBA),2 with linear IgA bullous dermatosis,and 1 with anti-P200 pemphigoid,were diagnosed in and enrolled from Department of Dermatology,Institute of Dermatology,Chinese Academy of Medical Sciences between January 2015 and December 2016.Seventy patients with pemphigus,15 patients with chronic eczema and 15 healthy adults served as controls.Blood samples collected from these patients and controls were subjected to IIF on three different substrates including NS,ME and SS,and the fluorescence deposition was observed.The sensitivities and specificities of IIF in the diagnosis of different subepidermal bullous diseases were compared.Statistical analysis was carried out with SPSS 13.0 software by using chi-square test for the comparison of enumeration data.Results IIF on NS or ME in the serum of patients with BP showed linear deposition of fluorescent material along the basement membrane zone.IIF on SS showed linear deposition of fluorescent material in the epidermis in the patients with BP,but in the dermis in the patients with EBA and anti-P200 pemphigoid.The sensitivities of IIF on NS,ME or SS in the diagnosis of subepidermal bullous diseases were 73.2％,60.7％ and 94.6％ respectively,and the specificities were 98.0％,100％ and 97.1％ respectively.There were significant differences among the sensitivities (x2 =18.2,P ＜ 0.05),but no significant difference was observed among the specificities (P ＞ 0.05).The diagnostic sensitivity of IIF on SS was significantly higher than that of IIF on NS or ME(x2 =8.0,16.7,both P ＜ 0.05).Conclusion In the diagnosis of autoimmune subepidermal bullous diseases,IIF on SS is superior to IIF on ME or NS.
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@#Linear IgA bullous dermatosis, also known as chronic bullous disease of childhood when present in the pediatric age group, is a rare blistering disease more predominantly seen in females less than five years old. This case describes a 2-year old girl who presented with scattered, tense vesicles and bullae on an erythematous base forming the classic “cluster of jewels” appearance. This clinical picture is often mistaken as bullous impetigo, commonly seen in children, delaying diagnosis and prompt treatment. Histopathologic examination showed subepidermal blistering with a predominantly neutrophilic inflammatory infiltrate. The direct immunofluorescence studies revealed a linear band of IgA deposition in the basement membrane zone consistent with the diagnosis of CBDC. The patient was started on colchicine and oral prednisone at 1 mg/kg/day and complete resolution was achieved within two weeks of therapy.
Subject(s)
ColchicineABSTRACT
Background: Treatments for autoimmune blistering disease carry significant risks of medical complications and can affect the patient's quality of life. Recently, the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire was developed in Australia. Objective: The objective of this study was to evaluate the reliability and validity of the Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire in Chinese patients with autoimmune blistering diseases. Methods: The Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire was produced by forward-backward translation and cross-cultural adaptation of the original English version. Autoimmune blistering disease patients recruited in the study self-administered the Chinese Treatment of Autoimmune Bullous Disease Quality of Life questionnaire, the Dermatology Life Quality Index and the 36-item Short-Form Health Survey. Reliability of the Chinese Treatment of Autoimmune Bullous Disease Quality of Life was evaluated using internal consistency and test-retest (days 0 and 7) methods. Validity was analyzed by face, content, construct, convergent and discriminant validity measures. Results: A total of 86 autoimmune blistering disease patients were recruited for the study. Cronbach's alpha coefficient was 0.883 and the intraclass correlation coefficient was 0.871. Face and content validities were satisfactory. Convergent validity testing revealed correlation coefficients of 0.664 for the Treatment of Autoimmune Bullous Disease Quality of Life and Dermatology Life Quality Index and –0.577 for the Treatment of Autoimmune Bullous Disease Quality of Life and 36-item Short-Form Health Survey. With respect to discriminant validity, no significant differences were observed in the Treatment of Autoimmune Bullous Disease Quality of Life scores of men and women (t = 0.251, P = 0.802), inpatients and outpatients (t = 0.447, P = 0.656), patients on steroids and steroid-sparing medications (t = 0.672, P = 0.503) and patients with different autoimmune blistering disease subtypes (F = 0.030, P = 0.971). Limitations: Illiterate patients were excluded from the study. The patients were from a single hospital and most of their conditions were in a relatively stable status. Conclusion: The Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire is a reliable and valid instrument to measure treatment burden and to serve as an end point in clinical trials in Chinese autoimmune blistering disease patients.
ABSTRACT
Background: Treatments for autoimmune blistering disease carry significant risks of medical complications and can affect the patient's quality of life. Recently, the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire was developed in Australia. Objective: The objective of this study was to evaluate the reliability and validity of the Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire in Chinese patients with autoimmune blistering diseases. Methods: The Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire was produced by forward-backward translation and cross-cultural adaptation of the original English version. Autoimmune blistering disease patients recruited in the study self-administered the Chinese Treatment of Autoimmune Bullous Disease Quality of Life questionnaire, the Dermatology Life Quality Index and the 36-item Short-Form Health Survey. Reliability of the Chinese Treatment of Autoimmune Bullous Disease Quality of Life was evaluated using internal consistency and test-retest (days 0 and 7) methods. Validity was analyzed by face, content, construct, convergent and discriminant validity measures. Results: A total of 86 autoimmune blistering disease patients were recruited for the study. Cronbach's alpha coefficient was 0.883 and the intraclass correlation coefficient was 0.871. Face and content validities were satisfactory. Convergent validity testing revealed correlation coefficients of 0.664 for the Treatment of Autoimmune Bullous Disease Quality of Life and Dermatology Life Quality Index and –0.577 for the Treatment of Autoimmune Bullous Disease Quality of Life and 36-item Short-Form Health Survey. With respect to discriminant validity, no significant differences were observed in the Treatment of Autoimmune Bullous Disease Quality of Life scores of men and women (t = 0.251, P = 0.802), inpatients and outpatients (t = 0.447, P = 0.656), patients on steroids and steroid-sparing medications (t = 0.672, P = 0.503) and patients with different autoimmune blistering disease subtypes (F = 0.030, P = 0.971). Limitations: Illiterate patients were excluded from the study. The patients were from a single hospital and most of their conditions were in a relatively stable status. Conclusion: The Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire is a reliable and valid instrument to measure treatment burden and to serve as an end point in clinical trials in Chinese autoimmune blistering disease patients.
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Resumen: La Epidermólisis ampollosa es una enfermedad hereditaria, crónica, incurable y de baja prevalencia. Se caracteriza por la aparición de ampollas luego de traumatismos mínimos, de manifestación predominantemente cutánea y de difícil manejo. Interfiere en la calidad de vida del paciente requiriendo un abordaje terapéutico multi-disciplinario. Presentamos a continuación un caso clínico, con el objetivo de describir manifestaciones clínicas, el enfoque diagnóstico y las posibilidades terapéuticas actuales para el abordaje integral de los pacientes que padecen esta enfermedad.
Abstract: Epidermolysis bullosa is an hereditary, chronic,disease, incurable and with very low prevalence. It is characterized by blisters after minor trauma, with predominantly cutaneous manifestation and difficult manage. Interferes with the quality of life of patients requiring a multi-disciplinary therapeutic approach. We present a clinical case, in order to describe clinical manifestations, current diagnostic approach and therapeutic possibilities for the comprehensive management of patients with this disease.
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BACKGROUND: Linear IgA bullous dermatosis (LABD) is an autoimmune, chronic bullous disease characterized by sub-epithelial bullae with linear IgA deposits along the basement membrane. LABD primarily affects young children and adults. There has been no study on LABD in Korea to date. OBJECTIVE: The purpose of this study was to evaluate the clinical features, laboratory examinations, treatments, and outcomes of Korean LABD patients. Patient characteristics including age at disease onset, gender, medical associations, medications, immunofluorescence findings, disease duration, treatment, and outcome were analyzed. METHODS: A retrospective analysis was conducted on 16 LABD patients diagnosed at Gangnam Severance Hospital between 1999 and 2014. RESULTS: A total of 16 LABD patients were included in the study, 5 children and 11 adults. The mean ages at disease onset in children and adults were 3.2 and 41 years, respectively. Eighty percent of children with LABD showed complete remission. In adults, partial remission was achieved in 36.4%, and complete remission in 54.5% of patients. Two patients were diagnosed with drug-induced LABD, and 2 with ulcerative colitis-associated LABD. CONCLUSION: Our report differed from previous reports in that all 5 children with LABD were male, and the incidence in adults was higher in females than males. Most patients responded well to dapsone and oral prednisolone. Since LABD is rare and can be misdiagnosed as impetigo or bullous pemphigoid, diagnosis by immunofluorescence microscopy is necessary for proper treatment to attain disease remission.
Subject(s)
Adult , Child , Female , Humans , Male , Basement Membrane , Dapsone , Diagnosis , Fluorescent Antibody Technique , Immunoglobulin A , Impetigo , Incidence , Korea , Linear IgA Bullous Dermatosis , Microscopy, Fluorescence , Pemphigoid, Bullous , Prednisolone , Retrospective Studies , UlcerABSTRACT
Chronic bullous disease of childhood (CBDC) is an autoimmune blistering disease that is characterized by Immunoglobulin A (IgA) deposits at the basement membrane zone. IgA autoantibodies (aAbs) from the serum of patients with CBDC react with antigens of 97 kDa (LABD97) and 120 kDa (LAD-1), and both of which are fragments of the extracellular domain of bullous pemphigoid 180 (BP180, type XVII collagen). The CBDC sera reacts with the immunodominant NC16a domain of BP180, which is the major region recognized by IgG aAbs in patients with bullous pemphigoid. A five-year-old boy presented with multiple pruritic tense blisters on the umbilical and inguinal areas for six weeks. The direct immunofluorescence of the perilesional area demonstrated linear deposits of IgA at the basement membrane zone. Using immunoblotting and an enzyme linked immunosorbent assay (ELISA), we identified the IgA aAbs reactive to antigens with a molecular weight of 120 kDa (LAD-1), which is a fragment of the extracellular domain of BP180.
Subject(s)
Humans , Autoantibodies , Basement Membrane , Blister , Enzyme-Linked Immunosorbent Assay , Fluorescent Antibody Technique, Direct , Immunoblotting , Immunoglobulin A , Immunoglobulin G , Linear IgA Bullous Dermatosis , Molecular Weight , Pemphigoid, Bullous , Transcutaneous Electric Nerve StimulationABSTRACT
La Dermatosis IgA lineal es una enfermedad vesiculoampollar subepidérmica autoinmune caracterizada por anticuerpos IgA en la unión dermoepidérmica. Es una enfermedad poco frecuente, siendo la mayoría de los casos idiopáticos, pero con reporte de casos por medicamentos, infecciones virales, enfermedades autoinmunes y tumores malignos. Se presentará un caso clínico de Dermatosis IgA lineal causada por Diclofenaco.
Linear IgA dermatosis is an autoimmune subepidermal vesiculobullous disease characterized by IgA antibodies at the dermo-epidermal junction. It is an uncommon disease, with most cases idiopathic, but with case reports caused by drug, viral infections, autoimmune diseases and malignant tumors. A clinical case of Linear IgA dermatosis caused by diclofenac is presented.
Subject(s)
Humans , Adult , Female , Anti-Inflammatory Agents, Non-Steroidal/adverse effects , Linear IgA Bullous Dermatosis/chemically induced , Linear IgA Bullous Dermatosis/drug therapy , Diclofenac/adverse effects , Autoimmune Diseases , Linear IgA Bullous Dermatosis/pathology , Fluorescent Antibody Technique, DirectABSTRACT
Edema blisters is an acute bullous disease that occurs following acute lower leg edema in patients with heart, renal and liver disease. Most of the affected patients are elderly, immobile and have been hospitalized for a long time. These clinical situations are commonly observed, but this can be misdiagnosed as other bullous diseases, especially friction blisters and bullous pemphigoid. We herein report a case of acute edema blisters in a 74-year-old female who presented with a tense bullae based on an edematous lower leg.
Subject(s)
Aged , Female , Humans , Blister , Edema , Friction , Heart , Leg , Liver Diseases , Pemphigoid, Bullous , Transcutaneous Electric Nerve StimulationABSTRACT
Pemphigus foliaceous (PF) is an autoimmune blistering disease resulting from acquired immunoglobulin G autoantibodies against desmoglein 1 of the skin, which is one of the adhesion molecules of keratinocytes. Clinically patients with PF develop crusted and scaly erosions mainly over the seborrhoeic distribution i.e. the face, scalp and upper trunk. Mild cases of PF may be localized but in some cases it may progress to erythrodermic exfoliative dermatitis. There is however no mucosal involvement in PF in contrast to pemphigus vulgaris and paraneoplastic pemphigus. Light microscopy of lesional biopsy shows subcorneal acantholysis. Direct immunofluorescence study of perilesional skin reveals presence of intraepithelial intercellular deposit of IgG and C3. We describe 2 cases of PF in the presence of thymoma, a relatively rare association, which could further support the fact of thymoma associated autoimmune disease.
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BACKGROUND: Autoimmune bullous diseases (ABDs) are a rare significant group of dermatoses that pose great challenges to the dermatologist. So far, few epidemiological surveys for the whole spectrum of ABDs have been completed in Korea, though many epidemiological survey have focused on single ABDs. OBJECTIVE: The aim of this study was to evaluate the incidence and clinical findings of patients in the Chonnam Province of Korea with ABDs over a 5 year period. METHODS: A retrospective analysis was conducted on 52 ABDs patients from 2002 to 2006. ABDs were diagnosed chiefly by histopathologic findings, immunofluoresence (IF), and immunoblotting with epidermal and dermal human foreskin extract. For the exact diagnosis of ABDs, several additional diagnostic methods such as indirect IF with salt-split normal skin, immunoblotting (IB) with normal keratinocyte cells, enzyme-linked immunosorbent assay (ELISA) with recombinant proteins, immunoprecipitaion (IP) were also performed. RESULTS: Out of 52 patients diagnosed with ABDs during the study period, pemphigus vulgaris was observed to be the commonest ABD (38.5%) followed by bullous pemphigoid (29.8%), pemphigus foliaceus (17.8%), epidermolysis bullosa acquisita (5.8%), paraneoplastic pemphigus, pemphigoid gestationis (3.8%), and linear IgA bullous dermatoses (1.9%). CONCLUSION: This study showed the incidence of the ABDs in Chonnam-Gwangju province area were very low (52 cases over 5 years) and was stationary over this period. Pemphigus vulgaris and bullous pemphigoid were common diseases among many ABDs. The incidence of ABDs in Korea as a whole needs to be investigated.
Subject(s)
Female , Humans , Enzyme-Linked Immunosorbent Assay , Epidemiologic Studies , Epidermolysis Bullosa Acquisita , Foreskin , Immunoblotting , Immunoglobulin A , Incidence , Keratinocytes , Korea , Pemphigoid Gestationis , Pemphigoid, Bullous , Pemphigus , Recombinant Proteins , Retrospective Studies , Skin , Skin Diseases , Skin Diseases, VesiculobullousABSTRACT
Anti-p200 pemphigoid is a newly defined autoimmune subepidermal blistering disease, which is characterized by the presence of IgG autoantibodies to the dermal side of 1M NaCl split skin as well as by the reactivity of these antibodies to a novel 200-kDa antigen on immunoblot analysis of a dermal extract. We describe a 49-year-old Korean male who presented with a bullous eruption on the whole body, which clinically resembled bullous pemphigoid or epidermolysis bullosa acquisita. A histopathological examination of a lesional skin biopsy specimen showed an area of dermal-epidermal separation and mixed dermal inflammatory infiltrates consisting of lymphocytes, neutrophils, and eosinophils. Direct immunofluorescence showed a linear deposition of IgG and C3 along the basement membrane zone. Indirect immunofluorescence demonstrated circulating IgG autoantibodies directed against the dermal side of the 1M NaCl split skin. Immunoblot analysis of dermal extracts revealed the patient's sera recognized the 200-kDa antigen. This is the first Korean case of an anti-p200 pemphigoid who showed good response to the treatment with systemic corticosteroids and dapsone.
Subject(s)
Humans , Male , Middle Aged , Autoantibodies/immunology , Pemphigoid, Bullous/diagnosisABSTRACT
We herein present two cases of generalized pustular psoriasis(GPP) followed by acquired bullous diseases during antipsoriatic management. Although there were several reports de-scribing the coexistence of psoriasis vulgaris and autoimmune bullous diseases such as bullous pemphigoid or pemphigus vulgaris(PV), a coexistence of GPP and bullous disease was sparcely reported. In one patient, we could define atypical autoantigen which was distinct from the other known antigens in documented cases of bullous diseases. The other case was compatible with PV. The psoriatic lesions and bullous eruptions of the two patients cleared in several weeks after administration of cyclosporine.
Subject(s)
Humans , Cyclosporine , Pemphigoid, Bullous , Pemphigus , PsoriasisABSTRACT
BACKGROUND: Immunofluorescent test (IF) is the mainstay of diagnosis for autoimmune blistering diseases. The salt split skin (SSS) techniques, which artificially create a blister through the lamina lucida can often clarify the diagnosis in most cases. The titer of antibodies in the sera of patients is in many instances proportional to the severity of the disease. So, indirect immunofluorescent test can afford a useful aid in their management. METHODS: Skin antigen was prepared from normal human skin which was treated with 1.0 M sodium chloride solution and sectioned at 4-6 m on cryostat. The skin sections were incubated with the patients' sera in varying dilutions, washed, and then incubated with fluorescein tagged anti-human IgG and IgM. The sections were washed again and viewed under epi-fluorescence microscopy. We tested the sera from patients with discoid lupus erythematosus (dLE, n=1), pemphigus vulgaris (PV, n=3), bullous pemphigoid (BP, n=2), photosensitive dermatitis (PSD, n=1), systemic lupus erythematosus (SLE, n=11) and normal individuals (n=6). RESULTS: Anti-skin antibody (ASA) was positive in 17 out of 18 patients by the SSS technique and negative in one photosensitive dermatitis and all of the healthy normal controls. Among the 17 ASA-positive patients, 3 patients with PV had anti-intercellular substance (ICS) antibodies, 13 patients who are 11 patients with SLE and 2 with BP had anti-basement membrane zone (BMZ) antibodies and one patient with dLE had both of the antibodies. We found three types of anti-BMZ antibody IIF patterns along split sites, i.e. dermal type (1 of 11 SLE), epidermal type (5 of 11 SLE, 2 of BP) and combined type (5 of 11 SLE, 1 dLE). All of the SLE patients had IgM anti-BMZ, by contrast 2 BP patients had IgG anti-BMZ. CONCLUSIONS: Our study suggests that indirect IF test for circulating ASA using SSS is very useful for diagnosing autoimmune bullous disease and distinguishing the type of ASA.