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July 1994

Cutaneous Polyarteritis Nodosa: Reports of Two Cases in Children and Review of the Literature

Author Affiliations

From the Division of Pediatric Dermatology, The Johns Hopkins Medical Institutions, Baltimore, Md.

Arch Dermatol. 1994;130(7):884-889. doi:10.1001/archderm.1994.01690070078012

Background:  Polyarteritis nodosa (PAN) is a necrotizing vasculitis of small- and medium-sized arteries that most often affects the kidneys, heart, and liver but can affect any organ system. A cutaneous form of PAN without visceral involvement, which follows a benign but often chronic course, has also been described. Both forms are rare in children.

Observations:  We describe two children with cutaneous PAN who were admitted to The Johns Hopkins Children's Center, Baltimore, Md, within a 1-week period. Both girls, aged 2 and 6 years old, presented with multiple, red, painful, edematous nodules on the extremities, face, and trunk. Dermatologic findings were accompanied by fever, arthralgias, and arthritis, but neither child had evidence of severe systemic disease. Skin biopsy specimens revealed necrotic small- and medium-sized muscular arteries with neutrophilic and eosinophilic infiltrate and fibrin thrombi. Both patients responded to treatment with prednisone.

Conclusions:  In children with cutaneous PAN, systemic symptoms may be present, but the lack of lifethreatening complications distinguishes this relatively benign disease from systemic PAN.(Arch Dermatol. 1994;130:884-889)

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