Cutaneous polyarteritis nodosa. Report of a case associated with Crohn's disease

Abstract

A patient had cutaneous polyarteritis nodosa associated with Crohn's disease. Immunopathologic studies disclosed evidence of circulating immune complexes as measured by C1q binding. Immunofluorescent microscopy of lesional skin showed C3 and fibrin deposition. Histamine injection of normal skin and subsequent biopsy specimens and immunofluorescent microscopy showed immune deposits. The patient was treated with cyclophosphamide and prednisone with a good clinical response, but within two years, histiocytic lymphoma developed. There is an association of cutaneous polyarteritis and Crohn's disease, and also, immunosuppressive therapy plays a role in the development of histiocytic lymphoma.

Ulcerative colitis presenting as leukocytoclastic vasculitis of skin

“…Skin manifestations occur in about 15% of patients with inflammatory bowel disorders [4] . Most frequently accompanying skin manifestations are pyoderma gangrenosum and erythema nodosum, while necrotizing vasculitis, cutaneous polyarteritis nodosa and granulomatous perivasculitis are less frequently seen [5][6][7][8] . Although the etiopathogenesis of extra-intestinal manifestations is not clear, a partial defect of immunity common to the skin and intestines has been suggested [5] .…”

Cutaneous polyarteritis nodosa in a patient with Crohn’s disease

“…Since then, 11 similar cases have been described in the English literature [5][6][7][8][9][10][11][12][13]. Here, we report the first Japanese case of cutaneous PAN associated with CD.…”

Fever, arthralgias, skin lesions, and ischemic digits in a 59‐year‐old man

“…This vasculitic syndrome is typically manifested as painful subcutaneous nodules and ulcers, primarily involving the ankle region, but also occasionally involving the thighs, trunk, and upper extremities. It is characterized by the presence of polyarthralgia or polyarthritis, myalgia, and occasionally peripheral neuropathy (27)(28)(29). The histology is that of a panarteritis of small-and medium-sized arteries, often with fibrinoid necrosis and almost exclusively in subcutaneous tissue.…”

“…Dermal arteries are generally not involved, and there is minimal panniculitis, distinguishing this entity from pyoderma gangrenosum and from erythema nodosum (29). Circulating immune complexes have been described in this disorder (28). Interestingly, the activity of the bowel disease does not appear to necessarily correlate with the activity of the vasculitis (27,29).…”

“…Perhaps somewhat surprising is the fact that this patient was receiving azathioprine therapy for his Crohn's disease, which has been reported to be useful in treatment of cutaneous PAN associated with Crohn's disease (32). There has been at least one reported case in which azathioprine did not result in significant improvement, and cyclophosphamide along with high-dose prednisone therapy was required to control the activity of the skin lesions (28).…”