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Necrotizing vasculitis with a polyarteritis nodosa‐like pattern and selective immunoglobulin A deficiency: case report and review of the literature
Author(s) -
Paradela Sabela,
Sacristán Felipe,
Almagro Manuel,
Prieto Víctor G.,
Kantrow Sara M.,
Fonseca Eduardo
Publication year - 2008
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.1600-0560.2007.00911.x
Subject(s) - medicine , serum protein electrophoresis , pathology , immunology , polyarteritis nodosa , vasculitis , primary immunodeficiency , antibody , disease , monoclonal antibody , monoclonal
Selective immunoglobulin A deficiency (IgAD) is a primary immunodeficiency disease characterized by low levels (< 7 mg/dl) of serum immunoglobulin (Ig) A and normal serum levels of IgG and IgM. Patients with IgAD have increased risk for recurrent respiratory and gastrointestinal infections, autoimmune disease, asthma and allergy. A 26‐year‐old woman was admitted with sudden onset of painful cutaneous lesions on her lower extremities, pyrexia and arthromyalgia. Her medical history was remarkable for recurrent respiratory tract infections, self‐limited episodes of acute diarrhea, atopy, splenomegaly and a 4‐year history of a lung granulomatous lesion. Laboratory and imaging tests ruled out severe life‐threatening infection, connective tissue disease and neoplasm. Serum protein electrophoresis showed a low IgA serum level (6.67 mg/dl), with normal serum levels of IgG and IgM, conducting to a diagnosis of selective IgAD. A skin biopsy showed necrotizing vasculitis without any sign of internal organ disease. We report a patient with IgAD and granulomatous involvement of lungs, spleen and medium‐sized arteries of the skin. Although IgAD results from a failure of B‐cell differentiation, we propose that deregulated immune response with production of cross‐reactive antibodies and hyperstimulation of T cells and macrophages could contribute to this widespread granulomatous reaction.