Henoch-Schönlein purpura with hemorrhagic bullae in children: report of two cases
Po-Mai Liu, Chin-Nam Bong, Hsin-Hsu Chen, Yi-Chuan Huang, Chao-Cheng Huang,Kuender D. Yang, Chih-Lu Wang
Department of Pediatrics, E-Da Hospital, Kaohsiung; Department of Pediatrics, Pingtung Christian Hospital, Pingtung; and Departments of Pediatrics and Pathology, Chang Gung Children¡¦s Hospital, Kaohsiung, Taiwan, ROC
Received: January 19, 2004 Revised: March 1, 2004 Accepted: June 6, 2004
Henoch-Schönlein purpura (HSP) is the most common form of acute vasculitis primarily affecting children. Clinical features include skin rashes, arthritis, abdominal pain and nephritis. Skin biopsy on immunofluorescence often reveals granular depositions of immunoglobulin A (IgA) and C3 within the walls of the dermal vessels as well as in the connective tissue of the upper dermis. The diversity of skin rashes produces confusion in diagnosis of HSP, especially in the presence of bullous lesions. Bullous lesions are very rare in children with HSP, whereas they often appear in adults with HSP. We report 2 cases of HSP in whom hemorrhagic bullae manifested predominantly. In our report, the skin biopsies of both patients revealed typical leukocytoclastic vasculitis without IgA and complement depositions on direct immunofluorescence studies. Dramatic improvement of clinical symptoms and signs was observed within a few days after corticosteroids were administered. There was neither recurrence nor nephritis in these 2 patients.
Hemorrhagic bullae, Henoch-Schoenlein purpura, leukocytoclastic vasculitis, vesiculobullous skin diseases
J Microbiol Immunol Infect 2004;37:375-378.