RESUMO
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.
Assuntos
Antígeno HLA-A24/genética , Poliarterite Nodosa , Pirina/genética , Dermatopatias Vasculares , Tela Subcutânea , Alelos , Criança , Feminino , Heterozigoto , Humanos , Japão , Mutação , Poliarterite Nodosa/diagnóstico , Poliarterite Nodosa/genética , Poliarterite Nodosa/fisiopatologia , Irmãos , Pele/patologia , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/genética , Dermatopatias Vasculares/fisiopatologia , Tela Subcutânea/irrigação sanguínea , Tela Subcutânea/diagnóstico por imagem , Tela Subcutânea/patologiaRESUMO
Cutaneous polyarteritis nodosa (CPAN) is a form of necrotizing vasculitis of small and medium-sized arteries. It is limited to the skin and has a recurrent and chronic course, possibly associated with fever, arthralgia, myalgia and neuropathy, but without visceral involvement. We report the clinical case of a 7-year-old male patient with CPAN refractory to treatment with high doses of corticoids and cyclophosphamide, who was successfully treated with the TNF-α (tumor necrosis factor-alpha) inhibitor, etanercept, in monotherapy.
Assuntos
Imunoglobulina G/uso terapêutico , Fatores Imunológicos/uso terapêutico , Poliarterite Nodosa/tratamento farmacológico , Receptores do Fator de Necrose Tumoral/uso terapêutico , Dermatopatias/tratamento farmacológico , Criança , Etanercepte , Humanos , Masculino , Resultado do TratamentoRESUMO
OBJECTIVE: This study aims to analyze the clinical characteristics, treatment and prognosis of children with cutaneous polyarteritis nodosa (CPAN), in order to improve the understanding of this disease. METHODS: Data of 14 children with CPAN, who were hospitalized in the Beijing Children's Hospital of Capital Medical University from January 2006 to December 2016, were collected. The clinical characteristics of all patients were summarized, the antistreptolysin-O (ASO)-positive and ASO-negative groups were compared, and the follow-up results were analyzed. X2-test, Fisher's exact probability test, t-test and Mann-Whitney test were used for statistical analysis. RESULTS: Among these 14 CPAN patients, nodular rash was the most common manifestation (14/14). The ASO-positive group had more nodules in the lower limbs and the ASO-negative group appeared more in the upper limbs, which were statistically significant (p<0.05). ASOpositive children were more likely to have joint symptoms (P<0.05), and were more prone to elevated white blood cells (P<0.05). Follow-ups were performed on nine patients, and the prognoses were all good. The occurrence of systemic polyarteritis nodosa was not observed. CONCLUSION: The main clinical manifestation of children with CPAN is skin nodules, which rarely affects the internal organs. Streptococcal infection is often the main cause. Anti-infection treatment should be simultaneously considered.