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Skin-nontumor / Clinical Dermatology

Blistering disorders

Linear IgA disease


Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC (see Reviewers page)
Revised: 26 August 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● Children or adults with acquired (drug induced or idiopathic) autoimmune disorder of pruritic subepidermal bullae or vesicles with linear deposits of IgA along the basement membrane

Sites
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● Thighs, buttocks, lower trunk, genital area, scalp, perioral face
● Mucous membrane involvement is importance because of associated scarring

Etiology
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● Unclear, appears to be immune mediated
● Target antigen of IgA autoantibodies is 120 kd secretory portion of BP180 antigen, but other antigens have also been reported
● Often occurs after administration of antibiotics, predominantly vancomycin or penicillin, rarely after amoxicillin-clavulanic acid (Pediatr Dermatol 2007;24:E40)
● Also after administration of nonsteroidal anti-inflammatory agents, diuretics, rarely interferon alpha-2A
● Not associated with HLA DR3, B8 (Clin Exp Dermatol 2009;34:e123)

Clinical features
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● Small tense blisters or annular bullous plaques ("cluster of jewels" or “string of beads”) with erosions and crusting
● Vancomycin associated cases may present with morbilliform (resembling measles) eruption without blistering (Arch Dermatol 2008;144:774)
● In Tunisia, is most frequent childhood bullous dermatosis; usually spares mucosa; responds rapidly to dapsone with long stabilization; erythromycin and oxacillin may be alternative therapy (Pediatr Dermatol 2009;26:28)
● Healing may be associated with hyper- or hypo- pigmentation
● Childhood cases are usually self-healing; symptoms may last over 5 years, and occasionally extend into adult life
● Adult cases follow a more prolonged course and rarely are refractory
● Associated with internal malignancy, including lymphoma and bladder carcinoma

Case reports
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● 5 year old girl with lesions developing after an insect bite (Minerva Pediatr 2008;60:351)
● 16 year old girl with IgA nephropathy 6 years after skin disease (Pediatr Dermatol 2008;25:339)
● 73 year old man with vesicles after vancomycin therapy (Dermatol Online J 2006;12:12)

Treatment
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● Withdrawal of offending drug, if applicable
● Dapsone or dapsone plus corticosteroids (eMedicine, DermQuest)
● Dexamethasone, 2 mg/day, in one report (J Dermatol 2008;35:737)
● Possibly enteric-coated mycophenolate sodium for refractory cases (J Dermatolog Treat 2008;19:364)

Clinical images
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Annular bullous plaques ("cluster of jewels") with erosions and crusting, pre- and post-therapy


Various images      Bullous lesions of hand and face

Micro description
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● Subepidermal blisters with neutrophilic infiltration
● Identical to dermatitis herpetiformis

Micro images
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Subepidermal blisters with neutrophils

                                                       
Neutrophilic microabscess     Neutrophils and eosinophils       Neutrophils at junction

    
Vancomycin associated cases

       
Linear IgA basement membrane staining

Positive stains
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● Linear deposition of IgA along the basement membrane zone of epidermis
● IgA band is linear, at basement membrane, compared to dermatitis herpetiformis, which has granular band at dermal papillae
● IgG in 25% of cases (Br J Dermatol 1997;116:293)
● IgM and C3 are occasionally present (Int J Dermatol 1985;24:569)

Videos
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Video #1, #2

Differential diagnosis
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Dermatitis herpetiformis: similar morphology but IgA band is usually granular; band is at dermal papillae; serum has anti-endomysial or anti-tissue transglutaminase IgA antibodies, symptoms improve on gluten-free diet (Dermatol Online J 2008;14:21)
Bullous lupus erythematosus: systemic symptoms; papillary dermal microabscesses, mucin in reticular dermis

End of Skin-nontumor / Clinical Dermatology > Blistering disorders > Linear IgA disease


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