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Linear IgA bullous dermatosis of childhood

Dermatology Online Journal, Volume 28, Issue 5 (2022)

A 4-year-old boy presented with blistering on his face and distal upper and lower extremities. Subepidermal blisters containing neutrophils and eosinophils visualized on histology supported the diagnosis of linear IgA bullous dermatosis of childhood (LABDC). The dermatosis presents with vesicles and tense blisters in an annular distribution, erythematous papules, and/or excoriated plaques. Histopathology shows subepidermal blisters with a neutrophilic infiltrate in the dermis, mainly concentrated at the tips of dermal papillae in the early stage of the disease, which can be mistaken for the pattern of neutrophilic infiltration as seen in dermatitis herpetiformis. Dapsone is the treatment of choice, which is started at a dosage of 0.5mg/kg/day. Linear IgA bullous dermatosis of childhood is a rare autoimmune disease that can be mistaken for other conditions with similar presentations but should always be considered in the differential diagnosis of children with blistering.

Cover page: Linear IgA bullous dermatosis of childhood

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Article
Peer Reviewed

A toxic epidermal necrolysis-like presentation of linear IgA bullous dermatosis treated with dapsone

Dermatology Online Journal, Volume 23, Issue 8 (2017)

Linear IgA bullous dermatosis is a rare autoimmune vesiculobullous disease characterized by linear deposition of IgA along the basement membrane zone. It is classically idiopathic, but may also arise secondary to drug exposure. A heterogeneous spectrum of clinical features has been described, including a rare, morbid variant mimicking toxic epidermal necrolysis. Herein, we present a case of vancomycin-induced linear IgA bullous dermatosis that manifested clinically as toxic epidermal necrolysis and resolved with dapsone therapy.

Cover page: A toxic epidermal necrolysis-like presentation of linear IgA bullous dermatosis treated with dapsone

Article
Peer Reviewed

Linear IgA bullous dermatosis associated with immunotherapy

Dermatology Online Journal, Volume 29, Issue 6 (2023)

Linear IgA bullous dermatosis (LABD) is a rare mucocutaneus blistering autoimmune disease caused by IgA autoantibodies. Its clinical manifestation can be indistinguishable from bullous pemphigoid (BP), a similar autoimmune bullous disease caused by IgG and IgE autoantibodies. Although BP has been reported as an adverse cutaneous effect of immunotherapy, LABD has rarely been associated with immunotherapy in the literature. We present the case of a 67-year-old woman with metastatic ovarian cancer receiving anti-PD1 and anti-CTLA4 with new onset pruritic tense bullae to the trunk, hands, elbows (in annular distribution) that occurred after immunotherapy. Skin biopsy showed subepidermal blister with abundant neutrophils on H&E histology, and linear IgA staining at the basement membrane on direct immunofluorescence consistent with the diagnosis of LABD. The condition did not improve on initial prednisone taper, but blisters rapidly resolved a few days after initiation of dapsone therapy. We favor that our patient's LABD is secondary to her immunotherapy. Our case highlights the importance of both H&E histology and direct immunofluorescence in diagnosis of blistering disorders in patients on immunotherapy to help in choosing the most effective treatment option in an attempt to avoid discontinuation of immunotherapy.

Cover page: Linear IgA bullous dermatosis associated with immunotherapy

Article
Peer Reviewed

Flame figures in linear IgA bullous dermatosis: a novel histopathologic finding

Dermatology Online Journal, Volume 23, Issue 11 (2017)

Background: Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease usually with a neutrophil rich inflammatory infiltrate, and characterized by linear IgA deposition at the basement membrane zone (BMZ), and neutrophil predominant dermal inflammation. We report a case of LABD with numerous eosinophils and flame figure formation, a unique histopathologic finding not previously reported. A 69-year-old woman presented with a rapidly progressive, intensely pruritic rash over forearms, breasts, axillae, hips, and thighs. Thelesions were comprised of annular vesicles and bullae with hemorrhagic crusts and erosions. The clinical differential diagnosis included bullous pemphigoid(BP), LABD, and epidermolysis bullosa aquisita (EBA).

Results: A biopsy from a bullous plaque on the wrist revealed a subepidermal blister with neutrophils and numerous eosinophils with flame figure formation.Direct immunofluorescent (DIF) microscopy revealed linear deposition of IgA at the BMZ.

Conclusions: Although unusual, the combined findings supported a diagnosis of LABD. Increased eosinophils may be associated with drug-induced LABD and may explain the numerous eosinophils in our case. It is important to be aware of this finding as the pathology may easily be misdiagnosed as BP, or possibly bullousWells syndrome. This case emphasizes that combined clinical, pathologic, and DIF findings are essential in the diagnosis of bullous dermatoses.

Cover page: Flame figures in linear IgA bullous dermatosis: a novel histopathologic finding

Article
Peer Reviewed

Sublamina densa-type linear IgA bullous dermatosis with IgA autoantibodies specific for type VII collagen: a case report and clinicopathological review of 32 cases

Dermatology Online Journal, Volume 23, Issue 11 (2017)

Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disorder characterized by linear deposits of IgA at the basement membrane zone(BMZ) and/or by circulating IgA anti-BMZ antibodies. Comparing with other immuno-bullous diseases, LABD represents a heterogeneous disease entitywith diversity of pathogenic IgA autoantibodies to different hemidesmosomal antigens and an association with malignancies and occasional drug use. We herein present an 82-year-old Japanese man with LABD, whose indirect immunofluorescence using 1M NaCl-split skin showed positive staining for IgA at the dermal side alone. Fluorescence overlay antigen mapping using laser scanning confocal microscopy (FOAM-LSCM) was employed to examine the in vivo bound patient’s IgA, which was specific for type VII collagen (COL7), a prominent antigen of the sublamina densa. One year later, he developed malignant lymphoma, suggesting the diagnosis of paraneoplastic LABD. We reviewed 32 cases of sublamina-densa type LABD with anti-COL7 IgA antibodies thus far reported in the literature to compare the clinicopathological characteristics of this rare disease variant and emphasize that COL7 is the main autoantigen in sublamina densa disease.

Cover page: Sublamina densa-type linear IgA bullous dermatosis with IgA autoantibodies specific for type VII collagen: a case report and clinicopathological review of 32 cases

Article
Peer Reviewed

Linear IgA bullous dermatosis associated with ulcerative proctitis: treatment challenge

Dermatology Online Journal, Volume 24, Issue 7 (2018)

Linear IgA bullous dermatosis is a rare bullous disease in children and adults that can be associated with autoimmune conditions, malignancies, infections, or medication exposure. The definitive diagnosis relies on the biopsy. A 58-year-old man presented to our clinic with a pruritic vesicular and bullous eruption. Histology showed the classic findings of a subepidermal blister with neutrophilic infiltrate and linear IgA deposition along the dermal-epidermal junction. Upon further evaluation, he was diagnosed with ulcerative proctitis. His therapy was complicated owing to side effects and lack of response to the standard treatment options. Dapsone, a first-line therapy, caused symptomatic methemoglobinemia whereas niacinamide with doxycycline were not effective. He required intravenous and oral steroids to reach improvement followed by transitioning to methotrexate.

Cover page: Linear IgA bullous dermatosis associated with ulcerative proctitis: treatment challenge

Article
Peer Reviewed

Vancomycin-induced linear IgA bullous dermatosis: associations

Dermatology Online Journal, Volume 22, Issue 4 (2016)

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease. LABD is considered mostly idiopathic, butsome cases have been reported to be drug-induced, mainly associated with vancomycin (VCM).We present two cases of LABD possibly associated with VCM used for cardiac surgery prophylaxis; in the presented cases, the eruptions occurred only after VCM withdrawal, therefore leaving a question about the relationship between VCM and LABD in these cases.We reviewed previous reports of VCM-induced LABD and analyzed the following parameters: gender, age, recent medical history, concurrent medication, latency period, progression after withdrawal, time to resolution, treatment, and rechallenge.Results: The causal relationship between VCM and LABD was often unclear; patients frequently had concurrent medication and symptoms frequently began and/or progressed after VCM withdrawal. Among the 46 reviewed patients in addition to our two cases (n=48), 20 (42%) had recent history of cardiac procedure, cardiac infection, congestive heart failure, or aortic aneurism.Conclusion: Further investigation is needed to ascertain the association between LABD, VCM, and heart disease.

Cover page: Vancomycin-induced linear IgA bullous dermatosis: associations

Article
Peer Reviewed

Trimethoprim-sulfamethoxazole-induced linear IgA bullous disease presenting as toxic epidermal necrolysis

Dermatology Online Journal, Volume 23, Issue 8 (2017)

BACKGROUND: Linear IgA bullous dermatosis (LABD) is an autoimmune blistering skin disorder characterized by linear IgA deposits along the dermoepidermal junction. Usually idiopathic, LABD can be drug-induced.

OBJECTIVE: To report the atypical characteristics of a case of trimethoprim-sulfamethoxazole-induced LABD presenting as toxic epidermal necrolysis (TEN).

METHODS: A 63-year-old woman treated with trimethoprim-sulfamethoxazole for Pneumocystis jirovecii infection developed a generalized maculopapular rash with herpetiform lesions, rosette-like lesions, and tense bullae with Nikolsky sign.

RESULTS: Anti-basement membrane zone antibodies were negative, but immunoblot revealed a 160 kDa band corresponding to subepidermal class IgA desmoglein 1. Skin biopsy specimens revealed a subepidermal bulla and direct immunofluorescence showed linear IgA deposition along the basement membrane zone. A diagnosis of toxic epidermal necrolysis was excluded and replaced by trimethoprim-sulfamethoxazole-induced LABD.

CONCLUSION: We report a case of trimethoprim-sulfamethoxazole-induced LABD with a 160 kDa IgA desmoglein 1 found by immunoblotting analysis, probably by epitope spreading.

Cover page: Trimethoprim-sulfamethoxazole-induced linear IgA bullous disease presenting as toxic epidermal necrolysis

Article
Peer Reviewed

Generalized linear IgA dermatosis with palmar involvement

Dermatology Online Journal, Volume 21, Issue 9 (2015)

Linear IgA bullous dermatosis (LABD) is a sub-epidermal blistering disorder characterized by deposition of IgA along the basement membrane zone (BMZ) as detected by immunofluorescence microscopy. The diagnosis is made by clinicopathologic correlation with immunofluorescence confirmation. Differentiation from other bullous dermatoses is important because therapeutic measures differ. Prompt initiation of the appropriate therapies can have a major impact on outcomes. We present three cases with prominent palmar involvement to alert the clinician of this potential physical exam finding and to consider LABD in the right context.

Cover page: Generalized linear IgA dermatosis with palmar involvement

Article
Peer Reviewed

Diffuse vesiculobullous eruption with systemic findings

Dermatology Online Journal, Volume 24, Issue 5 (2018)

Drug induced linear IgA bullous dermatosis (LABD) is a rare blistering disease that has been shown to be associated with the use of various medications. Although rarely seen together, some of the medications associated with LABD can lead to the syndrome drug reaction with eosinophilia and systemic symptoms (DRESS), which presents with fever, cutaneous eruption, and multi-organ involvement. We present a patient who developed fever and a generalized vesiculobullous eruption after recently starting amlodipine and meloxicam. Initial laboratory tests demonstrated elevated liver function tests, leukocystosis, and eosinophilia. Histopathologic examination of the punch biopsy revealed a bulla with sub-epidermal split and numerous neutrophils. Direct immunofluorescence demonstrated broad deposition of IgA along the dermal-epidermal junction. These findings were consistent with an overlap between LABD and DRESS. Drug induced LABD and DRESS are independently both rare diseases. It is even more uncommon to see the two concurrently in the same patient. In this patient, these two conditions were thought to be triggered by either amlodipine or meloxicam. Given the high mortality rate associated with DRESS, it is important to recognize the presentation and initiate the appropriate treatment plan as soon as possible.

Cover page: Diffuse vesiculobullous eruption with systemic findings