A case report of cutaneous polyarteritis nodosa in siblings

Abstract

Cutaneous polyarteritis nodosa (CPAN) is characterized by a necrotizing vasculitis of small and medium-sized arteries in the skin, which can be associated with fever, arthralgia, myalgia, and neuropathy, but, unlike polyarteritis nodosa (PAN), there is no visceral involvement. CPAN is rare in childhood. We report two siblings who developed CPAN during childhood. Interestingly, both had Mediterranean fever gene (MEFV) mutation, i.e. heterozygous E148Q. They also shared HLA-A24, -DR15 alleles. Simultaneous occurrence of MEFV mutation and HLA alleles with CPAN has never been reported in Japan. These cases could provide some hereditary clue for the development of CPAN.

Cutaneous polyarteritis nodosa in a 7-year-old boy: difficulties in diagnosis

“…The case presented here exemplifies the possible difficulties in diagnosing vasculitis due to inconclusive clinical manifestations, which is especially true for rare disease entities, such as PAN in children [7, 21, 22]. In the case of our patient, the symptoms and laboratory test results were indicative of several disease entities.…”

Potential Applications of PET Scans, CT Scans, and MR Imaging in Inflammatory Diseases

“…Previous studies have found FDG-PET sensitive for detecting disseminated spots throughout subcutaneous tissue and muscle associated with cutaneous polyarteritis nodosa (CPAN), a rare manifestation sometimes referred to as leopard skin appearance (56). CPAN is characterized by necrotizing vasculitis of medium and small arteries of the skin and is also associated with extra-cutaneous findings such as fever, malaise, and neuropathy but no visceral involvement (57).…”