Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases

Abstract

Cutaneous periarteritis nodosa (PAN) is a well-recognized entity characterized by tender subcutaneous nodules and livedo that may ulcerate. The pathogenesis of cutaneous PAN is not known. The objective of the study was to evaluate the clinical and histological features of 79 cases of cutaneous PAN and to investigate any clinical, pathological and immunological differences that may distinguish those cases likely to have a prolonged course. A retrospective analysis of 79 cases was conducted. Thirty-nine patients had ulcers during the course of their illness. Women were affected more than men. Painful nodules on the lower extremities, with oedema and swelling, were the most common clinical finding; 22% of patients had some evidence of neuropathy. Most of the laboratory findings were non-specific. There was no evidence for hepatitis B infection and hepatitis C infection was present in only one patient. Most patients (60%) had no associated medical condition. The disease course was prolonged but benign, and systemic PAN did not develop in any patient. Corticosteroids given systemically induced remission in most acute cases. The ulcerative form of disease was more prolonged and frequently associated with neuropathy.

Cutaneous Polyarteritis Nodosa Sine Nodosa

“…7 An extensive retrospective study analyzing 79 cases of CPAN found that painful nodules on the lower extremities with associated swelling and edema were the most common clinical findings, found in 80% of the reported cases. 8 Other notable findings include livedo reticularis in 56%, skin ulcers in 49%, and tender indurated plaques in 10% of cases. 8 A subset of CPAN patients have also been described as presenting with atrophie blanche as the predominant skin finding in the absence of venous insufficiency.…”

“…8 Other notable findings include livedo reticularis in 56%, skin ulcers in 49%, and tender indurated plaques in 10% of cases. 8 A subset of CPAN patients have also been described as presenting with atrophie blanche as the predominant skin finding in the absence of venous insufficiency. 9 In our patient, the classic nodular presentation was absent, with only minimal swollen and firm areas over the feet.…”

Differentialdiagnose Vaskulitis und Vaskulopathie aus dermatologischer Sicht

“…Für milde Formen ohne Nekrosen und ohne Allgemeinzustandsverschlechterung und -symptomen kann eine topische Therapie mit Steroiden 24 und eine Kompressionstherapie eingeleitet werden. Bei schweren Formen und/oder ausgeprägten Schmerzen und Nekrosen stehen systemische Therapieoptionen wie die orale Steroidtherapie 25 , Methotrexat, Cholchizin, Hydroxychloroquin, Dapson oder Azathioprin 26 oder intravenöse Immunglobuline 27 zur Verfügung. Außerdem profitieren die Patienten von einer Therapie mit NSAR, welche nicht nur antiinflammatorisch sondern auch schmerzlindernd wirken 20 .…”

Recent topics related to etiology and clinical manifestations of cutaneous arteritis

“…Although it is known that the peak of the onset of PAN is in the sixth decade of life and that PAN predominantly affects females, CA develops commonly in the fourth and fifth decades of life and predominantly affects males. Recent cohort studies reported a female-to-male ratio of 1.22–3.53 and that the mean or median age of onset was around the fourth decade of life ( Table 1 ) ( 7 , 10 – 15 ). Like PAN, CA also affects children.…”