Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia

Arakaki, Osao; Yamamoto, Yuichi; Awazawa, Ryoko; Nonaka, Kimiko; Taira, Kaoru; Asato, Yutaka; Hagiwara, Keisuke; Oyama, Bungo; Ishii, Norito; Hashimoto, Takashi; Uezato, Hiroshi

doi:10.1111/j.1346-8138.2008.00499.x

Cited by 14 publications

(9 citation statements)

References 46 publications

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“…Only a few cases of AHA coexisting with bullous pemphigoid, mucous membrane pemphigoid, pemphigus vulgaris, linear IgA bullous dermatosis (LABD) and epidermolysis bullosa acquisita have been reported. 2,5,[10][11][12][13][14][15] Coexistence of pemphigoid and AHA is believed to be caused by homologous structural sequences between plasma fVIII and hemidesmosomal antigen BPAG2. Antibodies against plasma fVIII are likely to interact with the central collagen-like domain of BPAG2; thus, a decrease in the level of fVIII inhibitor may improve cutaneous lesions of pemphigoid.…”

Section: Discussionmentioning

confidence: 99%

“…3,4 In 35-40% of cases, AHA accom-panies autoimmune diseases, including bullous dermatoses (Table 1). 4,5 Unlike congenital hemophilia A that occurs in men, AHA affects both sexes and has different manifestations. In congenital hemophilia, spontaneous hemarthroses are typical, whereas in the acquired form, extensive bleedings into soft tissues (skin, mucous membranes, muscles) and life-threatening external hemorrhages are observed.…”

Section: Introductionmentioning

confidence: 99%

“…The secondary form occurs in neoplastic and lymphoproliferative diseases, as well as in drug reactions and among puerperal women . In 35–40% of cases, AHA accompanies autoimmune diseases, including bullous dermatoses (Table ) …”

Section: Introductionmentioning

confidence: 99%

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Coexistence of pemphigus foliaceus and acquired hemophilia A: A case report

Filipczak¹,

Michalska-Jakubus²,

Górska-Kosicka

et al. 2015

The Journal of Dermatology

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Pemphigus foliaceus (PF) is an autoimmune bullous dermatosis with anti-desmoglein-1 autoantibodies. Acquired hemophilia A (AHA) is a rare coagulation disorder with a high mortality rate, caused by anti-factor VIII immunoglobulin G antibodies leading to spontaneous severe hemorrhages into skin, muscles or soft tissues. This coagulopathy may be associated with malignancies, drug reactions and autoimmune disorders including bullous dermatoses. Herein, we demonstrate a first report of AHA in the course of pemphigus foliaceus. A 55-year-old woman presenting with extensive, erosive, crusted, scaly skin lesions was diagnosed with PF based on histopathological and immunofluorescent examination, confirmed by the presence of anti-desmoglein-1 antibodies on enzyme-linked immunoassay. She developed extensive internal hemorrhages and prolonged external bleeding after laparotomy. Based on coagulation tests, AHA was diagnosed. Simultaneous remission of pemphigus and coagulopathy occurred with immunosuppressants and recombinant activated factor VII.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Coexistence of pemphigus foliaceus and acquired hemophilia A: A case report

Filipczak¹,

Michalska-Jakubus²,

Górska-Kosicka

et al. 2015

The Journal of Dermatology

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“…Association between LAD and malignancy has been described. Tumors that have been reported in patients with LAD include: B-cell lymphoma, chronic lymphocytic leukemia and carcinoma of the bladder, thyroid and esophagus; single case reports of ocular melanoma [17][18][19][20][21] and renal cell carcinoma [22][23] have also been described. Van der Waal et al reported a case of a patient with a metastasized renal cell carcinoma who developed an extensive blistering eruption similar to our case.…”

Section: Discussionmentioning

confidence: 99%

Drug - Induced Linear Iga Bullous Dermatosis Simulating Toxic Epidermal Necrolysis

Nasr¹,

Ammoury²,

Chouairy³

2014

LMJ

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Linear IgA bullous dermatosis (LAD) is an autoimmune subepidermal blistering disorder. LAD may be either idiopathic or drug related; the most common drug being vancomycin. The clinical presentations of both idiopathic and drug-related LAD are variable and may mimic other blistering disorders. We report a case of a 76-year-old man known to have a renal cell carcinoma who presented a vancomycininduced LAD that clinically mimicked toxic epidermal necrolysis (TEN).

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“…1 Idiopathic, systemic disease-related and drugrelated types of this disorder have been described so far. 1,2 We report the first case of LABD possibly associated with mefenamic acid, which is well known to induce commonly fixed drug eruptions.…”

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confidence: 99%

Linear immunoglobulin A bullous dermatosis possibly induced by mefenamic acid

Jin

Nakano

Akasaka

et al. 2010

The Journal of Dermatology

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Dear Editor, Linear immunoglobulin A bullous dermatosis (LABD) is a relatively rare autoimmune blistering disease characterized clinically by the presence of small tense blisters and immunologically by the presence of immunoglobulin (Ig)A at the dermal-epidermal junction.1 Idiopathic, systemic disease-related and drugrelated types of this disorder have been described so far. 1,2 We report the first case of LABD possibly associated with mefenamic acid, which is well known to induce commonly fixed drug eruptions.A 69-year-old Japanese woman presented with multiple blisters and erosions on her trunk, hip and thighs. She first noticed the skin eruption after taking mefenamic acid for 7 days for treatment of a common cold and the skin lesions were only a few blisters at the beginning. She visited a dermatology practitioner and a diagnosis of fixed drug eruption by mefenamic acids was made. Thus, she was given an oral antihistaminic drug and topical corticosteroid ointment. However, the eruption exacerbated and she was then referred to our clinic. Physical examination revealed some tense bullae and edematous erythema with vesiculobullae and erosions arranged annularly on her trunk, hip, thighs and upper extremities (Fig 1a,b). Mucosal lesions were not found in mucous membranes and there were no scars and milia. History taking revealed that the patient had experienced a similar eruption after taking mefenamic acids approximately 7 years before visiting us. Histological examination showed a subepidermal blister with a dense inflammatory infiltrate of lymphocytes, neutrophils and eosinophils in the edematous superficial dermis. Acantholysis and papillary abscess were not seen. All the laboratory data obtained were within normal limits.Direct immunofluorescent study revealed linear deposits of IgA and IgG at the basement membrane zone (BMZ) (Fig 1c,d). Indirect immunofluorescence found anti-BMZ antibodies: IgA (titer 1:160) and IgG (titer 1:160). Also, indirect immunofluorescence on 1 mol⁄ L NaCl-split human skin sections demonstrated that both IgA and IgG antibodies reacted with the epidermal side of the split (data not shown). Immunoblot analysis with human epidermal extracts failed to detect a clear band (data not shown). Enzyme-linked immunosorbent assays (ELISA) for both BP180 and BP230 were negative.We made a diagnosis of LABD based on clinical observations and immunological results. Also, we suspected mefenamic acid as a causative drug. We started treatment of 25 mg ⁄ day prednisone and the patient showed good response when we tapered prednisone.Linear IgA bullous dermatosis is an acquired immune-mediated subepidermal blistering disease characterized by circulating IgA autoantibodies, and now the soluble 120-kDa (LAD-1) ⁄ 97-kDa (LABD97) ectodomain of BP180 is the major target of the IgA antibodies. This condition is currently classified as a pemphigoid disease, which also includes bullous pemphigoid, pemphigoid gestationis and mucous membrane pemphigoid.3 It is well known that LABD sometimes shows an antigenic het...

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Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia

Cited by 14 publications

References 46 publications

Coexistence of pemphigus foliaceus and acquired hemophilia A: A case report

Coexistence of pemphigus foliaceus and acquired hemophilia A: A case report

Drug - Induced Linear Iga Bullous Dermatosis Simulating Toxic Epidermal Necrolysis

Linear immunoglobulin A bullous dermatosis possibly induced by mefenamic acid

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