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Skin - nontumor
Blistering disorders
Linear IgA disease
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 2 November 2009
Last major update: November 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Definition
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● Children or adults with acquired autoimmune disorder of pruritic subepidermal bullae or vesicles with linear deposits of IgA along the basement membrane
● Idiopathic or drug induced
Sites
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● Thighs, buttocks, lower trunk, genital area, scalp, perioral face
Etiology
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● Unclear, appears to be immune mediated
● Target antigen of the IgA autoantibodies is the 120 kd secretion portion of the BP180 antigen, but other antigens have been reported
● Often occurs after administration of antibiotics, predominantly vancomycin, rarely after amoxicillin-clavulanic (Pediatr Dermatol 2007;24:E40); also nonsteroidal anti-inflammatory agents, diuretics, rarely interferon alpha-2A (Clin Exp Dermatol 2009;34:e123)
● Not associated with HLA DR3, B8
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)
Prognostic factors
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● Childhood cases are usually self-healing
● Adult cases follow a more prolonged course and rarely are refractory
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
Microscopic 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 (but not IgG) 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
Videos
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● #1
Differential Diagnosis
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● Dermatitis herpetiformis: similar morphology, IgA band is usually granular, but rarely is linear/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 > Blistering disorders > Linear IgA disease
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