Cutaneous polyarteritis nodosa

An eight-year-old well grown boy, first born of non-consanguineous marriage, presented with fever, maculopapular eruptions, and joint pain since 4 months. The maculopapular skin lesions progressed to nodules and hyperpigmented plaques [Figure 1a], described as livedo reticularis [Figure 1b and c] which was associated with myalgia and restriction of joint movements without joint swelling or effusion. He had similar lesions in the past with bluish discoloration of the digits [Figure 1b and c], which responded to a short course of oral prednisolone. Laboratory investigations revealed normocytic normochromic anemia, lymphocytosis, thrombocytosis with elevated C-reactive protein (118 mg/L) and erythrocyte sedimentation rate (105 mm/h). Antistreptolysin O, Hepatitis B surface antigen, and Hepatitis C Antibody were negative. Rheumatological and Vasculitis panel of investigations were normal and incisional biopsy of the lesion revealed medium vessel vasculitis consistent with Cutaneous Polyarteritis Nodosa (PAN). Echocardiography, renal angiography, and ophthalmologic evaluation done were normal. The child responded to oral Prednisolone (2 mg/kg/day) and Azathioprine (2 mg/kg/day). Chronic or recurrent fever without major organ involvement with typical skin lesion(proven on skin biopsy) is defining feature of Cutaneous PAN.^[1]

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Figure 1:

(a) Maculopapular skin eruptions and hyperpigmented plaques over lower limbs; (b) bluish discoloration of digits with livedo reticularis; and (c) bluish discoloration of toes.

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