Polyarteritis nodosa-like vasculitis in association with minocycline use: a single-center case series.

OBJECTIVE: To describe the clinical features, treatment, and outcomes of polyarteritis nodosa (PAN)-like vasculitis in association with minocycline therapy. METHODS: We identified all subjects ≥18 years old with PAN-like vasculitis in the context of minocycline use seen at our institution between January 1995 and October 2010. Cases of hepatitis B-associated PAN were excluded. PAN was defined based on angiographic findings or tissue biopsy. Minocycline use was defined as medication use at the time of onset of first symptom. RESULTS: We identified 9 patients (5 females; 56%) with a median age of 30 (range 18 to 55) years. Four patients (44%) had isolated cutaneous disease, while 5 cases (56%) had systemic involvement including renal artery microaneurysms (2 patients), cholecystitis (1 patient), mononeuritis multiplex (2 patients), and mesenteric vasculitis (1 patient). Median duration of minocycline use was 2 (range 1 to 4) years. Three patients had a positive antinuclear antibody with negative extractable nuclear antigen antibodies. All patients had positive antineutrophil cytoplasmic antibody in a perinuclear pattern but specificity to myeloperoxidase was observed in 2 patients (22%). Diagnosis was confirmed by histopathology in 6 patients (67%) and angiography in 3 patients (33%). Minocycline was discontinued in all cases. Further immunosuppressive therapy was added in 6 cases (67%). CONCLUSIONS: Cutaneous, as well as systemic, PAN-like vasculitis may occur in association with minocycline use. Clinicians should consider the possibility of drug-induced vasculitis, especially in cases of medium-vessel vasculitis with atypical antineutrophil cytoplasmic antibody serologies or in patients with negative hepatitis B testing.

Atypical Omenn Syndrome due to Adenosine Deaminase Deficiency.

We present here a novel case of an atypical Omenn syndrome (OS) phenotype due to mutations in the ADA gene encoding adenosine deaminase. This case is noteworthy for a significant increase in circulating CD56(bright)CD16- cytokine-producing NK cells after treatment with steroids for skin rash.