Author: Esther Wang, Medical Student, University of Sydney, Sydney, Australia; Professor Dedee F. Murrell, Head, Department of Dermatology, St George Hospital, University of New South Wales, Sydney, Australia. DermNet New Zealand Editor-in-Chief: A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. February 2018.
IgA pemphigus is an autoimmune blistering disorder. It is also called intercellular IgA dermatosis and immunoglobulin A pemphigus among other names.
IgA pemphigus has two major subtypes.
A new classification describes 5 subtypes of IgA pemphigus:
IgA pemphigus is very rare. It most commonly affects the middle aged and elderly but it can affect any age, with a case reported in a child one month of age.
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:
Indirect immunofluorescence testing of serum can show IgA antikeratinocyte cell-surface antibodies. IgG antikeratinocyte cell-surface antibodies have also been reported.
Since few cases of IgA pemphigus have been described, the most effective treatment is unknown. Other medications worth considering include:
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