Linear IgA Dermatosis Clinical Presentation

Updated: Jun 21, 2018
  • Author: Mark Tye Haeberle, MD; Chief Editor: William D James, MD  more...
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Presentation

History

Some patients note a prolonged period of prodromal itching or transient pruritus or burning before lesions appear. Patients with ocular manifestations may complain of pain, grittiness, or discharge.

Bullae may be chronic, or lesions may appear acutely, as seen in drug-induced disease. Rash latency in vancomycin-induced cases of linear IgA dermatosis ranges from 1-13 days after the first dose. Review of medication exposures and delineation of the drug timeline are crucial in identifying potential inciting agents.

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Physical Examination

The classic primary lesions of linear IgA dermatosis are clear and/or hemorrhagic round or oval vesicles or bullae on normal, erythematous, or urticarial skin. Cutaneous manifestations may also include erythematous plaques, blanching macules and papules, or targetoid erythema multiforme–like lesions. The diagnosis is not dependent on the presence of vesicles and/or bullae and a morbilliform variant has been described. [14]

Bullae may be discrete or arranged in a herpetiform pattern, often described as the cluster of jewels sign. Alternatively, vesicles and bullae may be seen at the edge of annular or polycyclic lesions, the appearance of which has been described as the string of beads sign (see the images below). Rarely, linear IgA dermatosis may exhibit an isomorphic phenomenon. [15]

Annular lesions demonstrating the string of beads Annular lesions demonstrating the string of beads sign.
Bullous lesions of the palmar surface in an elderl Bullous lesions of the palmar surface in an elderly man with vancomycin-induced linear immunoglobulin A (IgA) dermatosis.

The distribution of linear IgA dermatosis differs between adults and children. Lesions in children are typically localized to the lower abdomen and anogenital areas, with frequent involvement of the perineum (see the image below). Other sites of involvement include the feet, hands, and face, particularly the perioral area. In adults, the trunk and the limbs are most commonly affected. In adults, involvement of the perineum and the perioral area is less common than in children. Lesions in both children and adults may be distributed symmetrically or asymmetrically. Dermatitis herpetiformis–like involvement of the extensor surfaces of the knees and the elbows is seen infrequently. Crusts, excoriations, erosions, or ulcers may be present.

Bullous lesions on the genital area in a child wit Bullous lesions on the genital area in a child with linear IgA dermatosis.

Oral manifestations are common in children and adults with linear IgA dermatosis. Oral lesions include vesicles, ulcerations, erythematous patches, erosions, desquamative gingivitis, or erosive cheilitis, and they may precede skin lesions. [16]

Both children and adults frequently complain of ocular symptoms, such as grittiness, burning, or discharge. Ophthalmologic findings even in the absence of ocular complaints may include subconjunctival fibrosis, shrinkage of the fornices, symblepharon formation, and cicatricial entropion with trichiasis (see the image below). [17]

Persons with linear immunoglobulin A (IgA) dermato Persons with linear immunoglobulin A (IgA) dermatosis may present with prominent ocular signs and symptoms.
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