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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

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Linear immunoglobulin A dermatosis: A case report.

Fernando Valenzuela Ahumada¹, Roberto Bustos Macaya², Gabriela Paz Romero Morgado²

¹Facultad de Medicina, Universidad de Chile, Santiago, Chile. Address: Departamento de Dermatología, Hospital Clínico Universidad de Chile, Santos Dumont 999, Independencia, Santiago, Chile .

|April 22, 2017

Summary

A sixty-five-year-old woman presented with itchy, hyperpigmented lesions and vesicles. Direct immunofluorescence confirmed linear immunoglobulin A disease, a rare autoimmune blistering condition.

Keywords:

bullous disease linear IgA bullous dermatosis

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Area of Science:

Dermatology
Immunodermatology
Autoimmune Blistering Diseases

Background:

Autoimmune blistering diseases are a group of rare disorders characterized by autoantibodies targeting structural proteins of the skin.
Linear immunoglobulin A disease (LABD) is an uncommon autoimmune subepidermal blistering disease.

Observation:

A 65-year-old woman experienced pruritus and hyperpigmented annular lesions on her trunk, buttocks, and extremities for two months.
Vesicles on a healthy skin base were noted on the flexor wrists, with no mucosal involvement.
Histopathology revealed subepidermal vesicular dermatitis with a neutrophil and eosinophil infiltrate.

Findings:

Direct immunofluorescence demonstrated linear and continuous deposition of immunoglobulin A (IgA) along the basement membrane zone.
These immunofluorescence findings are characteristic of linear immunoglobulin A disease.
The clinical presentation and diagnostic findings support the diagnosis of LABD.

Implications:

Accurate diagnosis of LABD is crucial for appropriate management and treatment.
Understanding the immunopathogenesis of LABD can lead to targeted therapies.
This case highlights the importance of direct immunofluorescence in diagnosing subepidermal blistering diseases.