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IgA pemphigus – Occurrence of anti‐Desmocollin 1 and anti‐Desmoglein 1 antibody reactivity in an individual patient
Author(s) -
Kopp Tamara,
Sitaru Cassian,
Pieczkowski Friederike,
Schneeberger Achim,
Födinger Dagmar,
Zillikens Detlef,
Stingl Georg,
Karlhofer Franz M.
Publication year - 2006
Publication title -
jddg: journal der deutschen dermatologischen gesellschaft
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.463
H-Index - 60
eISSN - 1610-0387
pISSN - 1610-0379
DOI - 10.1111/j.1610-0387.2006.06166.x
Subject(s) - desmoglein 1 , desmoglein 3 , desmoglein , autoantibody , pemphigus , immunofluorescence , pemphigus vulgaris , keratinocyte , direct fluorescent antibody , immunology , antibody , pathology , medicine , biology , in vitro , biochemistry
Summary Background : IgA pemphigus is a rare pustular autoimmune disease with exclusive IgA anti‐keratinocyte cell surface antibody reactivity. Two subtypes have been discerned: in the subcorneal pustular dermatosis type, desmocollin 1 has been identified as a targeted autoantigen, while in few cases of the intraepidermal neutrophilic type, IgA anti‐desmoglein 1 or IgA anti‐desmoglein 3 reactivity has been demonstrated. Patients and Methods : A 48‐year‐old white male presented with generalized large confluent pustules. Skin pathology was assessed by histology and direct immunofluorescence analysis. IgG/IgA autoantibodies against desmoglein 1/3 and desmocollin 1 were measured by ELISA and indirect immunofluorescence using desmocollin 1 cDNA‐transfected COS7 cells, respectively. Results : Histopathology revealed subcorneal pustules and direct immunofluorescence microscopy exclusively showed in vivo bound IgA with an intercellular pattern in the epidermis. Desmocollin 1 was identified as a target of IgA autoantibodies by indirect immunofluorescence microscopy utilizing desmocollin 1 cDNA‐transfected COS7 cells. In addition, IgA anti‐desmoglein 1 reactivity was demonstrated by ELISA. Neither IgA anti‐desmoglein 3 nor IgG anti‐desmoglein 1/3 autoantibodies were present. Conclusions : Both desmocollin 1 and desmoglein 1 were autoantigens in this patient with IgA pemphigus and a distinct clinical presentation. To our knowledge, this is the first IgA pemphigus case with dual autoantibody reactivity.