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Introduction: Linear IgA bullous dermatosis (LABD) is a rare autoimmune disease. Although dapsone is the initial treatment, other immunomodulators are used in resistant cases or when dapsone is unavailable. Case report: A 12-year-old Mexican child, with no relevant medical history, developed in May 2023 a disseminated dermatosis affecting all body segments, including mucous membranes, characterized by erythematous patches and plaques evolving into the formation of serous and serosanguinous blisters and vesicles, distributed in a “string of pearls” pattern. LABD was suspected and confirmed by skin biopsy, which showed a subepidermal blister with neutrophilic infiltration and linear Immunoglobulin A deposits at the dermo-epidermal junction by direct immunofluorescence. Treatment with prednisone (2 mg/kg/day) and cyclosporine (5 mg/kg/day) resulted in improvement and lesion remission within 2 weeks. Both drugs needed to be discontinued for 3 months due to intermittent blistering. Cyclosporine was continued as maintenance therapy at a dose of 4 mg/kg/day for 8 months. Conclusions: The report highlights the use of cyclosporine as an alternative immunomodulator for DAAL, an immunosuppressive agent used in autoimmune disorders. Few cases, including this one, have described complete remission and control of the dermatosis with cyclosporine, accompanied by prednisone at the start of treatment. [ABSTRACT FROM AUTHOR]

(1) Background: Linear immunoglobulin A bullous dermatosis (LABD) is a rare autoimmune, subepidermal blistering disease, characterized by linear IgA deposits along the epidermal basement membrane. LABD is idiopathic and is associated with medication and systemic autoimmune diseases. (2) Methods: We investigated the demographic characteristics, disease course, causative agents, and associated diseases in Korean patients with LABD. The Korean Health Insurance Review and Assessment Service database was used to obtain data. We identified 670 LABD cases between 2010 and 2022. (3) Results: The annual incidence of LABD was 1.3 per 100,000 persons, with a higher prevalence in individuals ≥60 years old. The patients were treated with dapsone for 30.7 ± 56.7 days, had 1.3 ± 0.7 hospital visits, and were hospitalized for 19.8 ± 19.7 days. Risk factors, including malignancy, commonly preceded LABD. Antibiotic use, specifically vancomycin and third-generation cephalosporins, was a risk factor. The mean age of LABD diagnosis was 55.9 ± 21.7 years. (4) Conclusion: This is the first published study to assess a nationwide cohort for LABD. The incidence of LABD was higher than that in other studies. Most case reports have linked LABD with the administration of specific antibiotics; however, this study shows there were more associations with other conditions. [ABSTRACT FROM AUTHOR]

Linear immunoglobulin A bullous dermatosis is a rare autoimmune disease with a good response to dapsone. We present the case of a female patient who was diagnosed with bullous pemphigoid and initially treated with systemic steroids; however, a poor response to the medication led to treatment discontinuation and loss to follow-up. The patient had lost vision in the left eye 2 years before this study and recently experienced decreased vision in the right eye.

Bullous pemphigoid (BP) and linear immunoglobulin A bullous dermatosis (LABD) are common immunobullous diseases affecting both adults and children, with minor differences in clinical presentation. The only test for a definitive diagnosis is direct immunofluorescence (DIF) study, with linear deposits of immunoglobulin G (IgG) (mainly) at the dermoepidermal junction (DEJ) being diagnosed as BP and linear deposits of IgA at DEJ, diagnosed as LABD. Mixed immunobullous disease is the term given for immunobullous diseases with equal IgA/IgG deposits at the DEJ. Through this case report, a mixed immunobullous disease with clinical diagnosis of LABD and DIF findings of BP is being discussed. Furthermore, the presence of a BP-LABD spectrum of disease that can progress in either direction and present with features of both diseases is discussed.

Bullous pemphigoid (BP) and linear immunoglobulin A bullous dermatosis (LABD) are common immunobullous diseases affecting both adults and children, with minor differences in clinical presentation. The only test for a definitive diagnosis is direct immunofluorescence (DIF) study, with linear deposits of immunoglobulin G (IgG) (mainly) at the dermoepidermal junction (DEJ) being diagnosed as BP and linear deposits of IgA at DEJ, diagnosed as LABD. Mixed immunobullous disease is the term given for immunobullous diseases with equal IgA/IgG deposits at the DEJ. Through this case report, a mixed immunobullous disease with clinical diagnosis of LABD and DIF findings of BP is being discussed. Furthermore, the presence of a BP-LABD spectrum of disease that can progress in either direction and present with features of both diseases is discussed. [ABSTRACT FROM AUTHOR]

Linear immunoglobulin A bullous dermatosis is a rare autoimmune disease with a good response to dapsone. We present the case of a female patient who was diagnosed with bullous pemphigoid and initially treated with systemic steroids; however, a poor response to the medication led to treatment discontinuation and loss to follow-up. The patient had lost vision in the left eye 2 years before this study and recently experienced decreased vision in the right eye. [ABSTRACT FROM AUTHOR]

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is an autoimmune mucocutaneous disease characterized by subepidermal blistering induced by IgA autoantibodies against several autoantigens in the basal membranous zone of the skin and mucosal tissue. Although diaminodiphenyl sulfone (DDS), also known as dapsone, is generally recognized as the first-line therapy for LABD, DDS can induce several severe side effects. We present a Japanese case of LABD with DDS-induced hemolytic anemia and alopecia. In the present case, the DDS-induced hemolytic anemia and hair loss made the DDS monotherapy difficult. When DDS is used in LABD patients with iron deficiency anemia (IDA), hemolytic anemia is concealed by IDA. It is thus necessary to carefully and frequently examine the laboratory data to find the signs of DDS-induced hemolytic anemia. Even though there is no literature on DDS-induced alopecia, alopecia was reported as one of the side effects of DDS in an FDA report, and, in our case, hair loss was improved after reducing its dosage. We have to recognize that alopecia is one of the side effects of DDS and that careful management is needed in order not to overlook the adverse side effects of DDS when treating LABD patients.

There are a number of diseases that manifest both on the skin and the oral mucosa, and therefore the importance for dermatologists in clinical practice to be aware of these associations is paramount. In the following continuing medical education series, we outline orocutaneous disease associations with both immunologic and inflammatory etiologies. [ABSTRACT FROM AUTHOR]

Bronchiolitis obliterans is a small-airway obstructive lung disease for which immunologically mediated pathogenesis is supposed. Frequent association of bronchiolitis obliterans with paraneoplastic pemphigus is well known, but its association with other autoimmune bullous diseases has not been reported except for a case of anti-laminin-332-type mucous membrane pemphigoid in a patient with chronic graft-versus-host disease. We report a case of non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans in a patient without transplantation. Although the patient's serum contained immunoglobulin (Ig)A antibodies to the 180-kDa bullous pemphigoid antigen/type XVII collagen and IgG antibodies to laminin-332, diagnosis of either linear IgA bullous dermatosis or mucous membrane pemphigoid could not be made because of the failure to detect linear IgA deposition at the basement membrane zone by direct immunofluorescence and the lack of mucous membrane lesions. Physicians should be aware that autoimmune bullous diseases other than paraneoplastic pemphigus can also associate with this rare but potentially fatal lung disease. [ABSTRACT FROM AUTHOR]

Pemphigus herpetiformis ( PH) is a rare variant of pemphigus characterized by erythemas and vesicles, tending to present with annular-shaped lesions. Immunologically, immunoglobulin (Ig)G deposition at the keratinocyte cell surfaces is observed. Linear IgA bullous dermatosis ( LABD) is a rare subepidermal blistering disease with linear IgA deposits at the epidermal basement membrane zone ( BMZ). The annular-shaped skin lesions in PH mimic clinical manifestation of other autoimmune bullous diseases, including LABD, although PH and LABD have different immunological and histopathological features. Herein, we report the first case of a shift from LABD to PH. A 70-year-old Japanese man presented annular erythemas surrounded by vesicles on the trunk and extremities. Histopathological examination revealed subepidermal bullae and eosinophilic spongiosis. Direct immunofluorescence demonstrated linear IgA deposits at the epidermal BMZ. Immunoblot analyses of normal human epidermal and dermal extracts, supernatant of HaCaT cells, recombinant proteins of BP180 NC16a and C-terminal domains, and purified laminin-332 showed no reactivity for either IgG or IgA. IgG chemiluminescent enzyme immunoassays for desmogleins 1 and 3, and BP180 were all negative. These findings led to the diagnosis of sole LABD. Although oral prednisolone temporarily improved the skin lesions, annular erythema without vesicles remained. A new skin biopsy revealed subcorneal pustules with eosinophils, but no subepidermal bullae. Direct immunofluorescence revealed IgG and C3 deposition at the keratinocyte cell surfaces. IgG enzyme-linked immunosorbent assay for mammalian desmocollins 1-3 revealed desmocollin 1 reactivity. Based on these findings, we made a diagnosis of sole PH. [ABSTRACT FROM AUTHOR]

Background: Linear immunoglobulin A bullous dermatosis (LABD) is a rare autoimmune subepidermal bullous disease. It is defined by continuous linear deposition of IgA in the basement membrane zone on direct immunofluorescence microscopy. The clinical presentations of LABD may mimic other diseases, and data in Taiwanese populations are still lacking. The current study aims to examine LABD status in Taiwan. Methods: We reviewed the database at our institute from 1995 to 2008. The gold standard for the diagnosis of LABD is based on continuous linear depositions of IgA in the basement membrane zone on direct immunofluorescence. Results: A total of 16 LABD patients were identified. Mean age at diagnosis was 55 years, and most (> 80%) occurred after the fourth decade. The trunk was most commonly involved (76%). However, in contrast to previous reports, the mucosal involvement was rare in our series (18%). Initial impressions were dermatitis herpetiformis in 8 patients (50%), bullous pemphigoid in 4 patients (25%), and vasculitis, varicella, and pemphigus vulgaris in the remaining 4 patients (25%). Four patients reported a history of drug ingestion shortly before the onset of the disease, and all recovered after discontinuing the offending drugs. One of them had griseofulvin-associated LABD, a case not reported previously. The other three drugs were rifampin, vancomycin and gemcitabine. Among the various regimens, dapsone (100 mg) twice a day achieved the best treatment response in the five treated patients. Conclusion: The rare and diverse presentations of LABD highlight the importance of our study results in aiding clinical diagnosis and planning treatment strategies.

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is an autoimmune mucocutaneous disease characterized by subepidermal blistering induced by IgA autoantibodies against several autoantigens in the basal membranous zone of the skin and mucosal tissue. Although diaminodiphenyl sulfone (DDS), also known as dapsone, is generally recognized as the first-line therapy for LABD, DDS can induce several severe side effects. We present a Japanese case of LABD with DDS-induced hemolytic anemia and alopecia. In the present case, the DDS-induced hemolytic anemia and hair loss made the DDS monotherapy difficult. When DDS is used in LABD patients with iron deficiency anemia (IDA), hemolytic anemia is concealed by IDA. It is thus necessary to carefully and frequently examine the laboratory data to find the signs of DDS-induced hemolytic anemia. Even though there is no literature on DDS-induced alopecia, alopecia was reported as one of the side effects of DDS in an FDA report, and, in our case, hair loss was improved after reducing its dosage. We have to recognize that alopecia is one of the side effects of DDS and that careful management is needed in order not to overlook the adverse side effects of DDS when treating LABD patients. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Linear immunoglobulin A bullous dermatosis (LABD) is a rare, heterogeneous, autoimmune blistering disorder. Although the main characteristics of the disease seem to be well identified, international accepted diagnostic criteria are lacking. Several authors suggested their own criteria, but they are often not complete or even in contrast with clinical and immunopathological findings from the literature, while others are too selective. In this review, the current views and the problems with the definition of reliable diagnostic criteria for LABD will be discussed. © 2013 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Abstract: Background: Linear immunoglobulin A bullous dermatosis (LABD) is a rare autoimmune subepidermal bullous disease. It is defined by continuous linear deposition of IgA in the basement membrane zone on direct immunofluorescence microscopy. The clinical presentations of LABD may mimic other diseases, and data in Taiwanese populations are still lacking. The current study aims to examine LABD status in Taiwan. Methods: We reviewed the database at our institute from 1995 to 2008. The gold standard for the diagnosis of LABD is based on continuous linear depositions of IgA in the basement membrane zone on direct immunofluorescence. Results: A total of 16 LABD patients were identified. Mean age at diagnosis was 55 years, and most (> 80%) occurred after the fourth decade. The trunk was most commonly involved (76%). However, in contrast to previous reports, the mucosal involvement was rare in our series (18%). Initial impressions were dermatitis herpetiformis in 8 patients (50%), bullous pemphigoid in 4 patients (25%), and vasculitis, varicella, and pemphigus vulgaris in the remaining 4 patients (25%). Four patients reported a history of drug ingestion shortly before the onset of the disease, and all recovered after discontinuing the offending drugs. One of them had griseofulvin-associated LABD, a case not reported previously. The other three drugs were rifampin, vancomycin and gemcitabine. Among the various regimens, dapsone (100 mg) twice a day achieved the best treatment response in the five treated patients. Conclusion: The rare and diverse presentations of LABD highlight the importance of our study results in aiding clinical diagnosis and planning treatment strategies. [ABSTRACT FROM AUTHOR]

We report a case involving a 62-year-old woman with in vivo-bound immunoglobulin (Ig)G and IgA antibodies in both the intercellular space ( ICS) and basement membrane zone ( BMZ). Her clinical and histopathological features were identical with those of pemphigus vulgaris, while the immunopathological findings suggested IgG/IgA pemphigus. Direct immunofluorescence ( IF) showed in vivo-bound IgG and IgA antibodies in the ICS and BMZ, whereas indirect IF showed circulating IgG but not IgA antibodies in the ICS and BMZ. The anti- ICS IgG bound to desmoglein-3, while the anti- BMZ antibodies bound to the epidermal side of 1 mol/L NaCl-split skin. To the best of our knowledge, only two similar cases have been reported so far. Furthermore, we also examined IgG subclass distribution of the in vivo-bound and circulating anti- ICS and BMZ antibodies, and found that IgG1, IgG2 and IgG4 bound to ICS of the lesional skins, while IgG1 and IgG3 bound to the BMZ. The circulating anti- ICS antibodies belonged to IgG1 and IgG4, while the circulating anti- BMZ antibodies to IgG1, IgG2 and IgG4. We report a case involving a 62-year-old woman with in vivo-bound immunoglobulin (Ig)G and IgA antibodies in both the intercellular space ( ICS) and basement membrane zone ( BMZ). Her clinical and histopathological features were identical with those of pemphigus vulgaris, while the immunopathological findings suggested IgG/IgA pemphigus. Direct immunofluorescence ( IF) showed in vivo-bound IgG and IgA antibodies in the ICS and BMZ, whereas indirect IF showed circulating IgG but not IgA antibodies in the ICS and BMZ. The anti- ICS IgG bound to desmoglein-3, while the anti- BMZ antibodies bound to the epidermal side of 1 mol/L NaCl-split skin. To the best of our knowledge, only two similar cases have been reported so far. Furthermore, we also examined IgG subclass distribution of the in vivo-bound and circulating anti- ICS and BMZ antibodies, and found that IgG1, IgG2 and IgG4 bound to ICS of the lesional skins, while IgG1 and IgG3 bound to the BMZ. The circulating anti- ICS antibodies belonged to IgG1 and IgG4, while the circulating anti- BMZ antibodies to IgG1, IgG2 and IgG4. [ABSTRACT FROM AUTHOR]