Atypical presentation of Henoch-Schonlein purpura: Case report and review of the literature

Abstract Rationale Henoch-Schonlein purpura (HSP) is a systemic vasculitis primarily affecting children, characterized by nonthrombocytopenic purpura and may include joint, gastrointestinal, or renal involvement. Clinical history and a characteristic rash of palpable purpura concentrated on the lower extremities and buttocks aid in the diagnosis. We report a case of HSP presenting with scalp edema and hemorrhagic vesiculobullous lesions and review of the literature. Methods A 4YO male presented with scalp edema and palpable purpuric lesions on his lower extremities and buttocks. He had an upper respiratory infection (URI) the previous week. Review of systems and exam were otherwise negative. Laboratory tests revealed normal white cell and platelet counts, sedimentation rate, complement, BUN/Cr, urinalysis, and a negative stool hemoccult. He was diagnosed with HSP. One month later he had a URI and knee swelling, followed by a hemorrhagic vesiculobullous eruption over his buttocks and lower extremities. Laboratory evaluation was again unremarkable. Literature review of the cutaneous manifestations in HSP was performed. Results Review of the literature revealed few case reports of either scalp edema or hemorrhagic vesiculobullous lesions in HSP. The documented incidence of vesiculobullous lesions in childhood HSP is only 2-4% (compared to 16% in adult cases). Scalp edema is also uncommon in children over 3YO with HSP. Our patient was 4YO with both features over the course of his illness. Conclusions Scalp edema and hemorrhagic vesiculobullous lesions are uncommon findings in childhood HSP and may be a source of diagnostic confusion. Physicians should recognize such signs as possible manifestations of HSP.