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AT-A-GLANCE

AT-A-GLANCE

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 ...

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