Henoch-Schoenlein Purpura

Abstract

The distinctive syndrome of Henoch-Schoenlein purpura (HSP) was first described by Heberden before 1800, and in 1808 English physician Robert Willan described a patient who had swollen, painful joints and a rash. The syndrome owes its name to two German physicians. In 1837, Johan Schoenlein described several cases of purpura associated with arthritis, which he termed "peliosis rheumatica." In 1868, Edouard Henoch pointed out that the term "peliosis rheumatica" was inappropriate and restrictive because patients who had urticarial purpura and acute arthritis also manifested gastrointestinal symptoms, such as vomiting, colicky abdominal pain, and melena. He described four children who had purpura, colicky abdominal pain, gastrointestinal hemorrhage, and joint pain as well as renal involvement.1 Since these first descriptions, HSP also has been referred to as anaphylactoid, allergic, or rheumatoid purpura; leukocytoclastic vasculitis; and allergic vasculitis.1,2 Epidemiology Seventy-five percent of all cases of HSP occur in children between 2 and 11 years of age, with younger children rarely affected. Children younger than 2 y experience a milder course of illness, with less frequent renal and gastrointestinal manifestations.3 HSP is exceedingly rare in adults, among whom other causes of vasculitis should be considered more likely. The incidence of HSP is greater in males, with a male-to-female ratio of 1.5 to 2.0:1.

Septicemia as a Complication of Henoch‐Schönlein Purpura

“…The history and clinical signs of this case are undoubtedly typical for mild Henoch‐Schönlein purpura (1‐6). The acute severe septicemia, however, is very particular and has, to our knowledge, never been reported before.…”

“…Although the lesions caused by Henoch‐Schönlein purpura are confined largely to the skin, kidney, joints, and bowel, all body systems can be involved (1‐6). To our knowledge, however, the septicemia we report here has never been described as a related complication.…”

Henoch–Schönlein Purpura With Severe Jejunitis and Minimal Skin Lesions

“…Absence of skin manifestations in conjunction with severe gastrointestinal symptoms is unusual in HSP (5,6). Dermatologic signs are an early and frequently occurring clue to the diagnosis of HSP in almost all cases.…”

“…Dermatologic signs are an early and frequently occurring clue to the diagnosis of HSP in almost all cases. Few cases of HSP have been described in children less than 2 years of age, but in these children the gastrointestinal complications tend to be minimal (5,6). The classic symptoms, most frequently in boys 2 to 8 years of age, include a purpuric rash over the lower extremities and buttocks, associated with acute onset of colicky abdominal pain, vomiting, arthritis, and (infrequently) seizures and coma (7).…”

“…In young children, facial purpura may be the only dermatologic sign (5). Cutaneous manifestations of HSP precede gastrointestinal symptoms in 86% of patients (6).…”