IgA pemphigus--occurrence of anti-desmocollin 1 and anti-desmoglein 1 antibody reactivity in an individual patient.

Abstract

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.

• Authors: Tamara Kopp, Cassian Sitaru, Friederike Pieczkowski, Achim Schneeberger, Dagmar Födinger, Detlef Zillikens, Georg Stingl, Franz M Karlhofer
• Journal: Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG (J Dtsch Dermatol Ges) Vol. 4 Issue 12 Pg. 1045-50 (Dec 2006) ISSN: 1610-0387 [Electronic] Germany
• PMID: 17176412 (Publication Type: Case Reports, Comparative Study, Journal Article)

Chemical References

• Anti-Bacterial Agents
• Anti-Infective Agents
• Anti-Inflammatory Agents
• Autoantibodies
• Autoantigens
• Desmocollins
• Desmoglein 1
• Immunoglobulin A
• Ointments
• Sulfadiazine
• Cefamandole
• Dapsone

Topics

• Anti-Bacterial Agents (administration & dosage, therapeutic use)
• Anti-Infective Agents (administration & dosage, therapeutic use)
• Anti-Inflammatory Agents (administration & dosage, therapeutic use)
• Autoantibodies (analysis, immunology)
• Autoantigens (immunology)
• Cefamandole (administration & dosage, therapeutic use)
• Dapsone (administration & dosage, therapeutic use)
• Desmocollins (immunology)
• Desmoglein 1 (immunology)
• Enzyme-Linked Immunosorbent Assay
• Fluorescent Antibody Technique
• Humans
• Immunoglobulin A (immunology)
• Male
• Middle Aged
• Ointments
• Pemphigus (drug therapy, immunology, pathology)
• Skin (pathology)
• Sulfadiazine (administration & dosage, therapeutic use)
• Time Factors
• Treatment Outcome