Abstract
Polyarteritis nodosa is a rare form of systemic vasculitis. Cutaneous polyarteritis nodosa (cPAN) is subtype of systemic PAN which predominantly affects the skin, extra-cutaneous findings include fever, malaise, myalgias, arthralgias, and neuropathy. The true incidence of cutaneous PAN is unknown. cPAN is recognized as a separate entity and is essentially a benign disorder which should be distinguished from systemic PAN, as the clinical course and management of the two conditions is different. In this case report, we have described a patient with fever and gangrene of both hands and feet. The patient underwent a very extensive workup in order to determine the cause of her condition, which finally turned out cPAN on skin biopsy
Highlights
Classic polyarteritis nodosa was the first systemic vasculitis to have been described
Cutaneous polyarteritis nodosa predominantly affects the skin, extracutaneous findings include fever, malaise, myalgias, arthralgias, and neuropathy. cutaneous polyarteritis nodosa (cPAN) is recognized as a separate entity but there are no diagnostic criteria for cPAN [4]. cPAN is essentially a benign disorder and should be distinguished from systemic Polyarteritis nodosa (PAN), as the clinical course and treatment of these two conditions is different from each other
We report first case of childhood cPAN in Pakistan
Summary
Classic polyarteritis nodosa was the first systemic vasculitis to have been described. Conclusion cPAN rare form of systemic vasculitis, its true incidence is yet unknown. Rheumatologist and Pediatrician should always think this rare disease whenever dealing a child with gangrene and systemic symptoms. In 1866, Kussmaul and Maier [1] characterized this fatal condition which was originally called ‘‘periarteritis nodosa.’’ It was not until 1931 that Lindberg [2] first recognized PAN limited to skin.
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