Pyoderma Gangrenosum‐like Lesions in Leukocyte Adhesion Deficiency I Treated with Intravenous Immunoglobulin

Abstract

A 31-year-old Caucasian male with leukocyte adhesion deficiency I and a 20-year history of recurrent, painful cutaneous ulcerations on the extremities presented with fatigue and worsening pain in both legs. He had experienced minimal improvement in his leg ulcers from treatment with systemic steroids, numerous courses of systemic antibiotics, and brief trials of infliximab and mycophenolate mofetil. He was treated with monthly intravenous immunoglobulin infusions. Upon completion of six courses of intravenous immunoglobulin his ulcerations had nearly healed for the first time in a decade.

Interleukin-12 and Interleukin-23 Blockade in Leukocyte Adhesion Deficiency Type 1

“…Chronic necrotic skin lesions, often incorrectly described as pyoderma gangrenosum (biopsy samples characteristically show no neutrophils), are common in LAD1 and are typically treated with antibiotics, surgery, or glucocorticoids, yet they still result in characteristic scarring. Case reports of successful treatment of these lesions with high-dose immune globulin, 10 tumor necrosis factor (TNF) blockade, 11 or glucocorticoids attest to the fact that immune modulators are often used as treatment, despite the lack of a mechanistic foundation to support their use. Interestingly, TNF blockade also inhibits the differentiation of type 17 helper T cells and the production of interleukin-17, potentially affecting the interleukin-17 axis in LAD1, as well.…”

Extensive phlegmon and pyoderma gangrenosum: diagnostic difficulties

“…Nord et al . [ 15 ] reported a case of good therapeutic response after intravenous infusion of immunoglobulin in a 31-year-old patient with PG in the course of leukocyte adhesion deficiency.…”

Leukocyte adhesion deficiency-I caused by a novel mutation in ITGB2 presenting with pyoderma gangrenosum

“…Patients with LAD-I presenting predominately with PG have been reported (Van de Kerkhof and Weemaes 1990;Bedlow et al 1998; Hinze al. 2010;Nord et al 2011;Thakur et al 2013;Madkaikar et al 2015). Similar to patient 1, most of these reported patients did not have significant infections prior to presenting with PG and therefore the diagnosis of LAD-I was often delayed.…”

“…The histopathology of classic PG shows predominant neutrophil infiltration. In contrast, the histopathology of LAD-I ulcerating lesions typically has an absence of neutrophils (Bedlow et al 1998;Nord et al 2011). The skin biopsy of patient 1 reported the presence of neutrophils suggesting partial neutrophil recruitment into the tissues.…”