Pyoderma gangrenosum Associated with Selective Hereditary IgA Deficiency

Abstract

A case of pyoderma gangrenosum is described in a girl aged 4. The condition was associated with selective IgA deficiency. The father and the 2 brothers suffered from the same deficiency (autosomal dominant transmission). Treatment with prednisolone and clofazimine produced an excellent clinical response.

Treatment recommendations for pyoderma gangrenosum: An evidence-based review of the literature based on more than 350 patients

“…P yoderma gangrenosum (PG) was once considered pathognomonic of idiopathic ulcerative colitis. It has since been described in association with a wide variety of disorders, 1,2 including Crohn's disease, arthritis, rheumatologic and hematologic conditions, 3,4 HIV infection, 5 sarcoidosis, 6 hereditary hypogammaglobulinemia, 7 iatrogenic immune suppression, 8 or malignancy. 1,2 The pathogenesis is unknown, but autoimmune mechanisms including immune complexemediated neutrophilic vascular reactions have been suggested.…”

Pyoderma Gangrenosum in an Infant

“…Ulcerative colitis is the most common disease associated with childhood PG, followed by leukemia, Crohn's disease, and arthritis. Furthermore, there are anecdotal reports of childhood PG associated with HIV infection (5), Takayasu disease (6), and immunodeficiency disorders including hypogammaglobulinemia (7), agammaglobulinemia (8), chronic granulomatous disease and hyper IgE syndrome (9), IgA deficiency (10), leukocyte adherence glycoprotein deficiency (11), and streaking leukocyte factor deficiency (12). Finally, a familial occurrence of PG in two siblings has been reported (13).…”

Cutaneous infection by Mycobacterium haemophilum and kansasii in an IgA-deficient man

“…Moreover, lower cutaneous sIgA levels and increased skin infections have been reported in patients with atopic dermatitis, while these patients of course often present also an impairment of the normal skin barrier. In conclusion, there are some clues about an association of skin infections with IgA-deficiency, although no clear pathophysiological link may be provided [11-13]. Strikingly, there is increasing evidence that serum IgA is able to trigger effector functions that have the potential to destroy microorganisms and mammalian cells.…”