S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology.

INTRODUCTION: Linear IgA dermatosis (LAD) is a rare subepidermal autoimmune bullous disease (AIBD) defined by predominant or exclusive immune deposits of immunoglobulin A at the basement membrane zone of skin or mucous membranes. This disorder is a rare, clinically and immunologically heterogeneous disease occurring both in children and in adults. The aim of this project is to present the main clinical features of LAD, to propose a diagnostic algorithm and provide management guidelines based primarily on experts' opinion because of the lack of large methodologically sound clinical studies. METHODS: These guidelines were initiated by the European Academy of Dermatology and Venereology (EADV) Task Force Autoimmune Bullous Diseases (AIBD). To achieve a broad consensus for these S2k consensus-based guidelines, a total of 29 experts from different countries, both European and non-European, including dermatologists, paediatric dermatologists and paediatricians were invited. All members of the guidelines committee agreed to develop consensus-based (S2k) guidelines. Prior to a first virtual consensus meeting, each of the invited authors elaborated a section of the present guidelines focusing on a selected topic, based on the relevant literature. All drafts were circulated among members of the writing group, and recommendations were discussed and voted during two hybrid consensus meetings. RESULTS: The guidelines summarizes evidence-based and expert opinion-based recommendations (S2 level) on the diagnosis and treatment of LAD. CONCLUSION: These guidelines will support dermatologists to improve their knowledge on the diagnosis and management of LAD.

Linear IgA bullous dermatosis of childhood: Retrospective single-center cohort.

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder impacting children and adults. In this single-center retrospective chart review of pediatric patients with LABD at a large tertiary referral center, we report the unifying and unique clinical features of 10 pediatric patients. Patients typically presented with the "cluster of jewels" sign (n = 6; 60%), mucous membrane involvement (n = 5; 50%) and had a mean disease duration of 38 months; six patients (60%) required inpatient admission for management of their skin disease, including all five patients who had mucous membrane involvement. Our findings suggest that pediatric LABD may be a disease with high morbidity and may be associated with severe complications when mucous membranes are involved.

IgA autoantibody may be the foremost pathogenic in three cases of linear IgA/IgG bullous dermatosis.

Linear IgA/IgG bullous dermatosis (LAGBD) is a relatively rare autoimmune bullous disease characterized by both IgA and IgG antibodies to basement membrane zone. The heterogeneity and pathogenesis of antibodies and the relationship between IgA and IgG in LAGBD have not been fully elucidated. We observed clinical, histological and immunological features of three LAGBD cases at different time points in the disease course. In our cohort, two cases showed IgA antibodies to epidermal antigens vanished when their lesions cleared after 3 months of treatment. One refractory case showed increasing antigens targeted by IgA antibodies with the progression of the disease. Collectively, the results suggest that IgA antibodies may play a major role in LAGBD. In addition, epitope spreading may be related to disease relapse and treatment refractory.

Pyoderma gangrenosum occurring 7 years after onset of linear IgA bullous dermatosis in a young woman.

We present a case of coexistence of pyoderma gangrenosum (PG) and linear IgA bullous dermatosis (LABD), with a 7-year interval between them. This is the first case of coexisting PG and LABD, to our knowledge.

A Case of Successful Management of Adult-Onset Linear IgA Bullous Disease With Sulfasalazine During the COVID-19 Pandemic.

Linear IgA bullous disease (LABD) is a rare, acquired, autoimmune, pruritic, and blistering skin condition. Dapsone is a first line treatment option, however, there are limited options if this fails, or if contraindicated. We present a case of successful management of LABD with sulfasalazine. A 46-year-old Caucasian female with LABD was commenced on high dose corticosteroids. She failed to wean, and dapsone was contraindicated due to a history of primary sclerosing cholangitis and risk of hepatitis. Following the failure of mycophenolate mofetil, sulfasalazine was trialed and successfully controlled both this patient’s LABD and ulcerative colitis. There is little literature on the use of sulfasalazine in dermatological conditions. We present sulfasalazine as an option for patients who are unable to use classically used treatments for LABD, or in those who have a dual diagnosis, as in this case, allowing for one agent to manage both conditions. Furthermore, The National Institute for Health and Care Excellence guidance mentions sulfasalazine as one of the few drugs that can be continued during the COVID-19 pandemic, and its use spared this patient from the significant immunosuppression associated with other treatment modalities.J Drugs Dermatol. 2022;21(12): doi:10.36849/JDD.6717.

Disseminated Tuberculosis in a Nigerian Adolescent with Linear IgA Bullous Dermatosis: A Case Report and Review of Literature.

Linear IgA bullous dermatosis (LABD) is an auto-immune disease affecting young children and adults, characterized by the linear deposition of IgA at the basement membrane zone with resultant complement activation and a cascade of immune reactions. There is a loss of adhesion at the dermo-epidermal junction and subsequent blister formation. It is a rare disease that has a good prognosis with adequate therapy. However, the underlying depressed immunity associated with the disease may expose them to such infections as tuberculosis. We report the case of an 11-years-old Nigerian female adolescent with LABD, diagnosed at the age of four years but defaulted on follow-up, who developed disseminated tuberculosis (pulmonary, lymph nodes, abdominal and pericardial effusion) seven years after the appearance of the initial blistering skin lesions. She commenced anti-tuberculosis drugs, steroids, and a tube pericardiostomy for the pericardial effusion. Dapsone was initiated for the LABD during the continuation phase of anti-tuberculosis therapy, with subsequent disappearance of the skin rash within two weeks.

La dermatose bulleuse linéaire à IgA (DBL) est une maladie auto-immune affectant les jeunes enfants et les adultes, caractérisée par le dépôt linéaire d'IgA dans la zone de la membrane basale, avec l'activation du complément qui en résulte et une cascade de réactions immunitaires. Il y a une perte d'adhérence à la jonction dermo-épidermique et une formation ultérieure de vésicules. C'est une maladie rare qui a un bon pronostic avec un traitement adéquat. Cependant, l'immunité déprimée sous-jacente associée à la maladie peut les exposer à des infections telles que la tuberculose. Nous rapportons le cas d'une adolescente nigériane de 11 ans atteinte de la LABD, diagnostiquée à l'âge de quatre ans mais en défaut de suivi, qui a développé une tuberculose disséminée (pulmonaire, ganglions lymphatiques, épanchement abdominal et péricardique) sept ans après l'apparition des lésions cutanées vésiculeuses initiales. Elle a commencé à recevoir des médicaments antituberculeux, des stéroïdes et une péricardiostomie par sonde pour l'épanchement péricardique. La dapsone a été initiée pour la DLB pendant la phase de continuation du traitement antituberculeux, avec une disparition de l'éruption cutanée en deux semaines. Mots clés: IgA linéaire, dermatose bulleuse, tuberculose disséminée, adolescent.

Successful treatment of a case of idiopathic linear IgA bullous dermatosis with oral sulfasalazine.

Linear IgA bullous dermatosis (LABD) is a rare acquired autoimmune chronic vesiculobullous dermatosis affecting primarily young children and older adults. We report a 17-year-old Chinese boy with a 2-month history of intense itching erythema or tense vesicles on healthy skin or on an erythematous base, with parts of lesions arising a characteristic "cluster of jewels" pattern. With the characteristics of vesicles or blisters on the skin, subepidermal blisters with neutrophilic infiltrate on histology, and linear IgA deposits on the basement membrane zone and absence of other immunoglobulins on direct immunofluorescence, LABD was dignosized. Sulfapyridine has also been reported as one of the best options of systemic therapy for LABD. Our patient successfully treated with only oral sulfasalazine (alternative medicine of sulfasalazine), which is safe and effective.

Linear IgA dermatosis of the childhood-Report of an amoxicillin-induced case.

Linear IgA dermatosis (LAD) is a rare autoimmune disorder in children. A 9-year-old boy was presented with blisters on the intact skin (face, body, arms, hands, soles, perigenital and perianal area) after amoxicillin treatment. Systemic corticosteroids and dapsone treatment for 6 weeks was successful. Clinical and immunofluorescence examinations are most important for differentiation of LAD and other drug-induced bullous dermatoses. They enable an early introduction of proper therapy.

Drug-induced linear immunoglobulin A bullous dermatosis: A French retrospective pharmacovigilance study of 69 cases.

AIMS: Linear immunoglobin A (IgA) bullous dermatosis is a rare autoimmune dermatosis considered spontaneous or drug-induced (DILAD). We assessed all DILAD cases, determined the imputability score of drugs and highlighted suspected drugs. METHODS: Data for patients with DILAD were collected retrospectively from the French Pharmacovigilance network (from 1985 to 2017) and from physicians involved in the Bullous Diseases French Study Group and the French Investigators for Skin Adverse Reactions to Drugs. Drug causality was systematically determined by the French imputability method. RESULTS: Of the 69 patients, 42% had mucous membrane involvement, 20% lesions mimicking toxic epidermal necrolysis (TEN), 21% eosinophil infiltrates and 10% keratinocytes necrosis. Direct immunofluorescence, in 80%, showed isolated linear IgA deposits. Vancomycin (VCM) was suspected in 39 cases (57%), 11 had TEN-like lesions, as compared with three without VCM suspected. Among the 33 patients with a single suspected drug, 85% had an intrinsic imputability score of I4. Among them, enoxaparin, minocycline and vibramycin were previously unpublished. For all patients, the suspect drug was withdrawn; 15 did not receive any treatment. First-line therapy for 31 patients was topical steroids. Among the 60 patients with available follow-up, 52 achieved remission, 10 without treatment. Four patients experienced relapse, four died and five had positive accidental rechallenges. CONCLUSIONS: There is no major clinical difference between DILAD and idiopathic linear IgA bullous dermatosis, but the former features a higher prevalence of patients mimicking TEN. VCM, suspected in more than half of the cases, might be responsible for more severe clinical presentations. We report three new putative drugs.

Cephalosporin-induced linear IgA dermatosis in a child: Case report and literature review.

Linear IgA dermatosis (LAD) is a rare, subepidermal blistering disease with mucocutaneous involvement. It may be idiopathic or drug induced. We describe a 4-year-old girl who presented with a vesiculobullous eruption after she had been treated with cefixime for urinary tract infection. A diagnosis of drug-induced LAD was made based on clinical, histopathological, and immunofluorescence findings. Naranjo adverse drug reaction algorithm was used to assess imputability resulting with a "probable" association. In literature, cephalosporin antibiotics are rarely reported in association with LAD. To our knowledge, this is the first case of a cefixime-induced LAD among adults and children.

Drug-induced Linear IgA Bullous Dermatosis: A Case Report and Review of the Literature.

Linear IgA bullous dermatosis (LABD) is a rare subepidermal autoimmune blistering disease characterized by linear deposition of IgA along the basement membrane zone. Although most reported cases are idiopathic, there is a subset of patients with drug-induced LABD. Various drugs have been associated with the drug-induced form of the disease. This paper reviews the literature on drugs reported to elicit linear IgA dermatosis and its specific clinical presentation. In addition, a case report of a 77-year-old male patient with linear IgA dermatosis induced by vancomycin is described. The aim of this paper is to emphasize the need to include this differential diagnosis in cases of suspected adverse drug reactions, as well as to highlight the role of drugs in LABD.

Pemphigoid variants affecting the skin.

Pemphigoid diseases are autoimmune subepidermal blistering diseases affecting the skin and mucous membranes, which are caused by autoantibodies targeting structural hemidesmosomal proteins or hemidesmosome-associated proteins. Variants of pemphigoid can be differentiated based on targeted antigens and clinical aspects. In this review, we will discuss pemphigoid variants that predominantly affect the skin, and provide clinicians with clues to diagnosis.

Linear IgA Bullous Dermatosis Preceding the Diagnosis of Primary Sclerosing Cholangitis and Ulcerative Colitis: A Case Report.

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder seen in the pediatric and adult populations that is often linked to a medication, infection, or underlying gastrointestinal, hepatobiliary, or autoimmune disease. In this study, we describe the case of a 23-year-old white man whose presentation and diagnosis of LABD ultimately led to the discovery of underlying primary sclerosing cholangitis (PSC) and ulcerative colitis (UC). His dermatitis resolved with topical steroids and dapsone, and he is undergoing systemic treatment for his UC and PSC. This exceptional case further validates the association between LABD with UC, strengthens that with PSC, and underscores the importance of alerting clinicians to consider conducting a systemic workup in addition to thorough medication history on making the diagnosis of LABD.

[Linear IgA bullous dermatosis]. / Lineare IgA-Dermatose.

Linear IgA bullous dermatosis is a rare autoimmune blistering disease that occurs in both children and adults. Strings of pearls, crowns of jewels, rosettes and urticarial plaques can occur on the whole integument with emphasis on the face (particularly perioral area) and genitalia. Pruritus is common and may be severe. The presence of IgA deposits along the basement membrane can usually be identified using direct immunofluorescence (DIF) microscopy. The histological and clinical features of this disorder may mimic those of dermatitis herpetiformis.

["Decoratively figured blisters" on the whole integument in initially diagnosed ulcerative colitis]. / "Schmuckvoll konfigurierte Blasen" am gesamten Integument bei erstdiagnostizierter Colitis ulcerosa.

The rare case of a 61-year-old patient suffering from linear IgA dermatosis is presented. The patient was previously hospitalized with chronic inflammatory bowel disease. The correct diagnosis of the disease was based on clinical and histological findings. Serological methods, such as indirect immunofluorescence, ELISA and immunoblotting are suitable for identification of the autoantibodies. In this case the detection of IgA antibodies along the basal membrane was achieved by direct immunofluorescence. Other bullous dermatoses with similar symptoms, such as an IgG-mediated bullous pemphigoid have to be excluded. The therapy of linear IgA dermatosis is ensured by steroid-containing topical agents, alongside antiseptic measures as well as systemic dapsone p.o.

Linear immunoglobulin A dermatosis mimicking toxic epidermal necrolysis: a case report of etanercept treatment.

A 65-year-old pluripathological woman attended our hospital with a cutaneous eruption of sudden appearance after vancomycin treatment. She presented targetoid lesions affecting approximately 25-30% of her body surface, large erosions with mucosal lesions and positive Nikolsky sign. Under the initial clinical suspicion of toxic epidermal necrolysis (TEN), and considering the recent literature of successful use of etanercept in these cases, she was treated with a single dose of this antitumour necrosis factor (anti-TNF) agent. Subsequently, the exanthema progression stopped and resolution of the lesions happened in a few days. Later on, histopathology revealed a subepidermal blister with dense neutrophilic infiltrate and linear deposits of immunoglobulin A (IgA) on the dermoepidermal junction, allowing us to establish the diagnosis of drug-induced linear IgA dermatosis mimicking TEN. Linear IgA dermatosis can have severe clinical manifestations, even mimicking TEN, and can have high mortality, especially in drug-induced cases. We have not found any other report of linear IgA dermatosis treated with etanercept in the English literature. Anti-TNF medications could represent useful therapeutic alternatives in this dermatosis.

Non-infectious diffuse vesiculobullous rash in a pancreas and renal transplant recipient.

Solid organ transplant patients are well established to be at risk of herpes simplex virus and varicella zoster virus infection and reactivation. We present a case of a 41-year-old woman with a history of pancreas and renal transplant who presented with what appeared to be disseminated herpes simplex virus or varicella zoster virus induced rash, but who was ultimately diagnosed and treated as linear IgA bullous dermatosis. This case alerts physicians to other non-infectious dermatoses as a cause of vesiculobullous rash in solid organ transplant patients.

Diagnostic patterns and delays in autoimmune blistering diseases of the mouth: A cross-sectional study.

OBJECTIVES: To describe the natural history and factors influencing diagnostic delays among patients with autoimmune blistering diseases of the mouth. MATERIALS AND METHODS: In this cross-sectional study, 27 newly diagnosed patients were interviewed, and professional and patient delays were calculated. Disease extent and severity scores were determined using Saraswat scoring system. RESULTS: Twenty-seven patients were interviewed and examined. Patient delay was significantly longer in patients who had desquamative gingivitis as initial presentation, in those who tried to use home remedies and over the counter medications, and in patients with less severe disease. Most patients (n = 21 [77.7%]) made more than one consultation, and the mean time needed to reach a definitive diagnosis (i.e. professional delay) was 83.2 ± 21.4 days (range from 21 to 130 days). Professional delay was significantly correlated with the number of previous consultations (r = .78) and was significantly longer in patients who had desquamative gingivitis as initial presentation. CONCLUSION: Diagnosis of oral blistering diseases is often delayed. Diagnostic delay is more common in patients presenting with desquamative gingivitis and those with less severe disease. Improving patients and healthcare professionals' awareness about oral blistering diseases might help reduce diagnostic delay.