Home » Topics A–Z » IgA pemphigus
Author: Esther Wang, Medical Student, University of Sydney, Sydney, NSW, Australia; Professor Dedee Murrell, Head, Department of Dermatology, St George Hospital, University of New South Wales, Sydney, NSW, Australia. DermNet New Zealand Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell/Maria McGivern. February 2018.
IgA pemphigus (or immunoglobulin A pemphigus) is an autoimmune blistering disorder. It is also called intercellular IgA dermatosis among other names.
IgA pemphigus has two major subtypes:
However, a new classification describes five subtypes of IgA pemphigus; these being:
IgA pemphigus is very rare. It most commonly affects the middle aged and old people but it can affect people at any age, with a case reported in a 1-month-old child.
The clinical features of IgA pemphigus include blisters, pustules, erythema, erosions and vegetating lesions.
IgA pemphigus appears to favour the trunk, upper and lower extremities, axillae, and groin. Mucosal involvement is very rare.
The exact cause of IgA pemphigus is unclear.
Other conditions that should be considered in the differential diagnosis include:
An autoimmune blistering disease or autoinflammatory pustular disease may be suspected clinically from the clinical features, often after the eruption has failed to clear with antibiotics.
The diagnosis of IgA pemphigus requires a skin biopsy for histology and direct immunofluorescent testing.
Indirect immunofluorescence testing of serum can show IgA anti-keratinocyte cell-surface antibodies. Immunoglobulin G (IgG) anti-keratinocyte cell-surface antibodies have also been reported.
The main medications used to treat IgA pemphigus are systemic steroids and dapsone.
Since few cases of IgA pemphigus have been described, the most effective treatment is unknown. Other medications worth considering include:
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