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October 1992

Primary Systemic Pustular Vasculitis With Severe Intestinal Involvement

Author Affiliations

Department of Dermatology Okayama University Medical School Shikata-cho 2-5-1 Okayama 700, Japan

Arch Dermatol. 1992;128(10):1408-1410. doi:10.1001/archderm.1992.01680200120030

To the Editor.—  Two patients with pustulosis palmaris et plantaris (PPP) followed by purpuric pustules, fever, and severe intestinal symptoms are described in the context of systemic pustular vasculitis.

Report of Cases.—Case 1.—  A 43-year-old man with PPP of 14 years' duration developed tender, purpuric, 2- to 40-mm macules and pustules on the dorsa of his feet, legs, and palms (Fig 1), sore throat, and intersternocostoclavicular (ISCC) pain after acute pharyngitis. Laboratory studies disclosed the following values or results: leukocytes, 14.0×109/L (neutrophils, 0.7); IgA, 7.21 g/L (normal, 0.90 to 4.50 g/L); erythrocyte sedimentation rate, 70 mm/h; C-reactive protein, 5+; CH50, 66.9 U/mL (normal, 30 to 40 U/mL); and proteinuria and microhematuria. A throat culture yielded Streptococcus pyogenes. Bone scintigraphy with technetium Tc 99m MDP (methylene diphosphonate) indicated incipient ISCC ossification. Pustules were not induced by trauma. Severe colicky abdominal pain

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