Abstract |
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.
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Authors | Sabela Paradela, Felipe Sacristán, Manuel Almagro, Víctor G Prieto, Sara M Kantrow, Eduardo Fonseca |
Journal | Journal of cutaneous pathology
(J Cutan Pathol)
Vol. 35
Issue 9
Pg. 871-5
(Sep 2008)
ISSN: 1600-0560 [Electronic] United States |
PMID | 18494827
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Anti-Inflammatory Agents, Non-Steroidal
- Immunoglobulin A
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Topics |
- Adult
- Anti-Inflammatory Agents, Non-Steroidal
(therapeutic use)
- Arteries
(pathology)
- Female
- Granuloma, Respiratory Tract
(complications, pathology)
- Humans
- IgA Deficiency
(complications, drug therapy, pathology)
- Immunoglobulin A
(blood)
- Lung Diseases
(complications, pathology)
- Necrosis
- Polyarteritis Nodosa
(complications, drug therapy, pathology)
- Skin
(blood supply, pathology)
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