Cutaneous polyarteritis nodosa in a child following hepatitis B vaccination

ARTICLE

Auteur(s) : Filipa Ventura¹, Henedina Antunes², Celeste Brito¹, Fernando Pardal³, Teresa Pereira¹, Ana Paula Vieira¹

¹Dept of Dermatology and Venereology, Hospital de São Marcos, Apartado 2242, 4701-965 Braga, Portugal
²Pediatric Department, Hospital de São Marcos, Braga, Portugal
³Pathology Department, Hospital de São Marcos, Braga, Portugal

Polyarteritis nodosa (PAN) is a rare vasculitis in childhood, characterized by necrotizing inflammatory changes in small and medium sized arteries [1]. The classification criteria for childhood vasculitis included cutaneous polyarteritis nodosa (CPAN) and microscopic polyangiitis as two distinct additional categories to PAN [2]. CPAN can be distinguished from classical PAN by the absence of systemic involvement and a benign, but chronic and relapsing course [3, 4].

An 11-year-old boy presented with a 3 month history of extensive livedo reticularis mainly affecting the lower extremities (figure 1A) but also the abdomen and upper extremities. Dermatological findings were accompanied by asthenia, myalgias and anorexia. The patient reported worsening of the lesions with the cold. There were no cutaneous nodules or additional lesions on physical examination. This clinical picture had appeared one week after injection of the third dose of hepatitis B vaccine. Histology of the livedo reticularis on the thigh revealed an inflammatory arteritis of medium and small sized arteries of the lower dermis, consistent with the diagnosis of PAN (figure 1B). Immunohistochemical exploration using an anti-antigen HBs antibody was negative. Laboratory investigations revealed anemia, elevated erythrocyte sedimentation rate and C-reactive protein. Renal and liver function tests were normal and anti-streptolysin O titre was negative. Immunological work-up revealed positive circulating immune complexes (78.61 U/L) and hypergammaglobulinemia (1760 mg/dL). The determination of antinuclear antibodies, antineutrophil cytoplasmic antibodies, antiphospholipid antibodies, cryoglobulins and complement were normal. Serological results only showed positive anti-HBs antibodies. The test of HBV-DNA by PCR was negative. Radiological evaluation including chest X-ray, electrocardiogram, echocardiogram, abdominal ultrasound, chest, abdominal and pelvic CT, renal magnetic resonance angiography and pulmonary function tests were normal. The diagnosis of CPAN was made. The patient was treated with prednisolone (1 mg/kg/day) successfully reduced after 6 weeks of treatment, azathioprine and omeprazole, which relieved the symptoms but did not much benefit the cutaneous lesions. In a follow-up of 2 years, the boy was asymptomatic apart from a persisting livedo reticularis on the lower extremities, however, less inflammatory. The laboratory examinations conducted during this period were normal. He has not been taking any medication for one year.

CPAN is characterized by the presence of painful cutaneous nodules associated with livedo reticularis and ulceration [5]. Livedo reticularis appeared to be an important clinical feature in most series and, although rare, might be the only cutaneous manifestation, as in our case [4]. Extra-cutaneous symptoms like fever, anorexia, myalgia and arthralgia are frequent [5].

Most cases of childhood CPAN have been associated with streptoccocal infection [3, 6]. Associations with hepatitis B virus infection and hepatitis B vaccination have never been described in children. Twenty-seven cases of vasculitis occurring after this vaccination have been published and among them only two cases of CPAN have been reported [4]. Both were young women with no relevant past medical history. One patient developed fever and infiltrated nodules on the legs two weeks after a five-year booster of hepatitis B vaccination and remission was obtained with oral corticosteroids. The other presented with livedo reticularis on the legs that appeared one month after the first injection of hepatitis B vaccination. She was treated with colchicine that had no effect on the livedo. The cause was never formally demonstrated because vascular deposits of HBs antigen were not found. Some authors believe that the physiopathology might be related to vascular deposits of excess circulating immune complexes of antigens, which, in certain conditions, could persist, form a deposit and activate the complement in the vessels [4]. To our knowledge this association has been described only twice in the literature and this is the first case reported in a child.

Acknowledgements

References

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4 Bourgeais AM, Dore MX, Croue A, et al. Cutaneous polyarteritis nodosa following hepatitis B vaccination. Ann Dermatol Venereol 2003; 130: 205-7.

5 Sunderkötter C, Sindrilaru A. Clinical classification of vasculitis. Eur J Dermatol 2006; 16: 114-24.

6 Assicot C, Bourrat E, Prigent F, et al. Cutaneous polyarteritis nodosa in children: three cases. Ann Dermatol Venereol 2002; 129: 207-11.