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.

Keywords:

• Combination therapy
• Cutaneous polyarteritis nodosa (CPAN)
• E148Q
• HLA alleles
• MEFV mutation
• Siblings

Acknowledgements

We would like to thank Dr. Yasushi Kawaguchi and Takako Miyamae (Institute of Rheumatology, Tokyo Women’s Medical University) for analyzing MEFV mutations.

Conflict of interest

None.