Chonnam Med J.  2009 Dec;45(3):207-210. 10.4068/cmj.2009.45.3.207.

A Case of Henoch-Schonlein Purpura with Hemorrhagic Bullous Lesions

• Lee KY
• Jeong JY
• Suh JH
• Kim JS

• KMID: 2274897
• DOI: http://doi.org/10.4068/cmj.2009.45.3.207

Abstract

Henoch-Schonlein purpura (HSP) is the most common vasculitis in children and is characterized by cutaneous purpura, arthritis, abdominal pain, and nephritis. The characteristic rash of HSP consists of palpable purpura on the buttocks and lower extremities. Bullous lesions often appear in adults with HSP, whereas they are very rare in children with HSP. We experienced a 9-year-old male patient who presented with colicky abdominal pain and arthralgia and severe hemorrhagic bullae in both lower legs. The skin biopsy of the patient revealed typical leukocytoclastic vasculitis in small vessels of the dermis, and prominent IgA deposition was shown on capillary walls by direct immunofluorescence. His clinical symptoms were markedly improved with conservative management including corticosteroid therapy, which left no complications.

Keyword

Purpura, Schoenlein-Henoch; Hemorrhagic bullae; Vasculitis; Child

MeSH Terms

Abdominal Pain
Adult
Arthralgia
Arthritis
Biopsy
Blister
Buttocks
Capillaries
Child
Dermis
Exanthema
Fluorescent Antibody Technique, Direct
Humans
Immunoglobulin A
Leg
Lower Extremity
Male
Nephritis
Purpura
Purpura, Schoenlein-Henoch
Skin
Vasculitis
Vasculitis, Leukocytoclastic, Cutaneous
Immunoglobulin A
Vasculitis, Leukocytoclastic, Cutaneous

Figure

• 

  Fig. 1 The photograph shows hemorrhagic bullae with various sized purpura on both lower legs.

• 

  Fig. 2 Microscopic findings of bullous lesion in the patient. (A) The specimen shows suprabasal hemorrhagic blister and dermal inflammatory infiltrates and hemorrhage (H&E, ×40). (B) High power view of dermis shows perivascular infiltration of neutrophils, nuclear debris and extravasated red blood cells (Inset: dermal blood vessels with fibrin deposition and inflammatory infiltrates, H&E, ×400). (C) Direct immunofluorescence for IgA shows granular depositions in the small vascular walls of dermis (arrows, ×200).

Reference

1. Saulsbury FT. Henoch-Schönlein purpura in children Report of 100 patients and review of the literature. Medicine (Baltimore). 1999. 78:395–409.

2. Calviño MC, Llorca J, García-Porrúa C, Fernández-Iglesias JL, Rodriguez-Ledo P, González-Gay MA. Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine. 2001. 80:279–290.

3. Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, et al. Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum. 2005. 35:143–153.

4. Abdel-Al YK, Hejazi Z, Majeed HA. Henoch Schönlein purpura in Arab children. Analysis of 52 cases. Trop Geogr Med. 1990. 42:52–57.

5. Trapani S, Mariotti P, Resti M, Nappini L, de Martino M, Falcini F. Severe hemorrhagic bullous lesions in Henoch Schönlein purpura: three pediatric cases and review of the literature. Rheumatol Int. 2009. [Epub ahead of print].

6. Saulsbury FT. Henoch-Schönlein purpura. Lancet. 2007. 369:976–978.

7. Nussinovitch M, Prais D, Finkelstein Y, Varsano I. Cutaneous manifestations of Henoch-Schönlein purpura in young children. Pediatr Dermatol. 1998. 15:426–428.

8. Leung AK, Robson WL. Hemorrhagic bullous lesions in a child with Henoch-Schönlein purpura. Pediatr Dermatol. 2006. 23:139–141.

9. Oh YM, Jung MR, Choi HJ, Cha HJ, Jeong JY. A case of Henoch-Schönlein purpura with hemorrhagic bullae in a child. J Korean Soc Pediatr Nephrol. 2006. 10:40–44.

10. Leung SP. Use of intravenous hydrocortisone in Henoch-Schönlein purpura. J Paediatr Child Health. 2001. 37:309–310.