Idiopathic pyoderma gangrenosum and impaired lymphocyte function: failure of azathioprine and corticosteroid therapy

Abstract

A 58-year-old man with chronic "idiopathic' pyoderma gangrenosum, recurrent secondary staphylococcal infection, nail candidiasis, markedly impaired lymphocyte function and a serum blocking factor is described. Despite an initially favourable response to immunosuppressive therapy, the patient later relapsed whilst on high dose systemic corticosteroids and azathioprine. Immune function remains abnormal 3 years cessation of azathioprine. Attempted immunostimulation with Corynebacterium parvum proved unsuccessful. A striking but transient increase in lymphocyte function followed parenteral iron therapy for an unexplained iron-deficiency anaemia. Gradual reduction in prednisone dosage to 5 mg daily has coincided with a period of clinical resolution and modest improvement in lymphocyte reactivity. Prolonged immunosuppressive therapy may be inadvisable in chronic idiopathic pyoderma gangrenosum associated with defective cellular immunity.

Pyoderma gangrenosum: a review

“…At doses of 0.15 mg/kg twice per day, tacrolimus has healed some PG lesions. Nephrotoxicity, neurotoxicity and diabetes mellitus are concerns 126,139–144 . Azathioprine is a purine analogue that inhibits T‐ and B‐lymphocyte function.…”

“…Azathioprine is a purine analogue that inhibits T‐ and B‐lymphocyte function. Onset of action is slow 143,144 . Alkylating agents such as cyclophosphamide, melphalan and chlorambucil block DNA replication and may have a role in treatment of refractory PG.…”

“…Alkylating agents such as cyclophosphamide, melphalan and chlorambucil block DNA replication and may have a role in treatment of refractory PG. There is a risk, however, of secondary malignancy and of myelosuppression 143,145 , 146 …”

'Sticky' neutrophils, pathergic arthritis, and response to heparin in pyoderma gangrenosum complicating ulcerative colitis.

“…It may occur at any site, commonly affecting the legs, and exhibits pathergy -that is, occurring at sites of previous minor trauma such as needle puncture.3 Its presence is not closely related to the activity of colitis and it may even occur after colectomy.4 5 In In both conditions neutrophil abnormalities have been reported, including decreased migration into skin windows, reduced chemotaxis, abnormal phagocytosis, and decreased bactericidal activity.14 [19][20][21] In the case presented here the pathergic nature of the patient's arthritis led us to the hypothesis that increased neutrophil adherence to vascular endothelium might be the underlying abnormality. The marginated neutrophils would then be ready to migrate into adjacent tissue in response to minor local trauma.…”