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Linear Immunoglobulin A Dermatosis

  • Rare blistering disease with onset typically after fourth decade of life.

  • Linear band of immunoglobulin (Ig) A at the dermal–epidermal basement membrane.

  • Clinical presentations may mimic dermatitis herpetiformis, bullous pemphigoid, and cicatricial pemphigoid.

  • May occur in association with many drugs, including vancomycin.

  • May occur in association with inflammatory bowel diseases, but is only rarely associated with gluten-sensitive enteropathy.

  • Rarely seen in association with malignancy, specifically lymphoid malignancy.

  • Histology shows subepidermal collection of neutrophils at the basement membrane, often collecting in papillary tips with subepidermal blisters.

  • Patients have relatively low titers of circulating IgA autoantibodies, most frequently against portions of BPAG2 (type XVII collagen), or rarely against BPAG1, LAD 285, type VII collagen, and others.

  • Most patients respond dramatically to treatment with dapsone; some require adjunctive systemic corticosteroids.

  • Prognosis is variable with both spontaneous remissions and longstanding disease.

Chronic Bullous Disease of Childhood

  • Rare blistering disorder of childhood presenting predominantly in children younger than 5 years of age.

  • Linear IgA at the dermal–epidermal basement membrane.

  • Clinical presentation of tense bullae, often in perineum and perioral regions, giving a “cluster-of-jewels” appearance. New lesions sometimes appear around the periphery of previous lesions with a collarette of blisters.

  • Histology shows subepidermal collection of neutrophils at the basement membrane, similar to linear IgA bullous dermatosis.

  • Most patients respond dramatically to treatment with dapsone.

  • Spontaneous remissions, often within 2 years, are frequent.

Linear immunoglobulin (Ig) A dermatosis is a rare, immune-mediated, blistering skin disease that is defined by the presence of homogeneous linear deposits of IgA at the cutaneous basement membrane (Fig. 58-1). Although in the original description of patients with linear IgA dermatosis it was considered to be a manifestation of dermatitis herpetiformis (DH), it has now been clearly separated from DH on the basis of its immunopathology, immunogenetics, and lack of consistent association with a gluten-sensitive enteropathy.1-4 Patients with linear IgA dermatosis can present with lesions suggestive of epidermolysis bullosa acquisita (EBA), DH, bullous pemphigoid (BP), lichen planus, prurigo nodularis, or cicatricial pemphigoid.1-6

Figure 58-1

Direct immunofluorescence of normal-appearing perilesional skin from a patient with linear immunoglobulin A dermatosis. A homogeneous band of immunoglobulin A is present at the dermal–epidermal junction.

Drug-induced linear IgA was initially described in association with vancomycin and is now associated with a wide variety of drugs.7-15 Drug-induced linear IgA differs somewhat from classic linear IgA in clinical presentation with a wider variety of clinical presentations, including morbilliform, erythema multiforme–like, and toxic epidermal necrolysis–like.7-11,16-18

Chronic bullous disease of childhood (CBDC) is a rare blistering disease that occurs predominantly in children younger than 5 years of age and has an identical pattern of homogeneous linear IgA deposits at the epidermal basement membrane.19,20 Studies demonstrate that in some patients CBDC and linear IgA dermatosis represent ...

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