IgA Pemphigus Clinical Presentation

Updated: Jun 18, 2021
  • Author: Jose A Plaza, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Patients affected with IgA pemphigus usually have subacute onset of disease. In more than one half of reported patients, pruritus is present. The systematic review by Kridin showed that the most common presenting symptoms are vesicles (80.8%), pustules (75%), circinate plaques (63.6%), and pruritus (65.6%). [7] Further, the trunk was the most common area of involvement (83.5%), followed by the extremities (80.2%). Intertriginous involvement was noted in 39.3% of cases, head/neck involvement in 29.8%, and mucosal involvement in 13.2% of cases.

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Physical Examination

IgA pemphigus is a vesiculopustular skin disease. In general, lesions form within erythematous plaques but also can form in skin without plaques. The initial, clear, fluid-filled blisters associated with IgA pemphigus fill with neutrophils and transform into pustules.

In some IgA pemphigus patients, neutrophils settle at the lower part of the blister, forming a hypopyon pattern. Lesions usually became flaccid after an initial tense appearance. Some lesions become crusted and form a herpetiform pattern. [36]

In most IgA pemphigus patients, the trunk and proximal extremities primarily are involved. In some IgA pemphigus patients, scalp and postauricular areas are involved extensively. In some IgA pemphigus patients, intertriginous areas (axillary and inframammary areas) are involved. Mucous membranes, palms, and soles usually are spared. In one patient with IgA pemphigus, involvement of perianal skin and oral mucosa was observed. [37]

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