Skin-nontumor / Clinical dermatology
Blistering disorders
Linear IgA disease

Author: Mowafak Hamodat, M.D., MB.CH.B, MSc., FRCPC (see Authors page)

Revised: 15 June 2016, last major update July 2011

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PubMed Search: Linear IgA disease

Cite this page: Linear IgA disease. PathologyOutlines.com website. http://pathologyoutlines.com/topic/skinnontumorlineariga.html. Accessed December 7th, 2016.
Definition / General
  • 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
  • Thighs, buttocks, lower trunk, genital area, scalp, perioral face
  • Mucous membrane involvement is importance because of associated scarring
Etiology
  • 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
  • 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
Treatment
Clinical Images

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

Micro Description
  • Subepidermal blisters with neutrophilic infiltration
  • Identical to dermatitis herpetiformis
Micro Images

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Subepidermal blisters with neutrophils

Neutrophilic microabscess

(B) and (C), respectively: Vancomycin associated cases

Linear IgA basement membrane staining

Positive Stains
  • 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)
Differential Diagnosis
  • 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