Henoch Schoenlein Purpura

Key Points
Form of vasculitis, typically seen in children
Exact cause is unknown, but may be related to respiratory and other infections, drug and food reactions
Consists of a vasculitic rash (beginning as small, red spots and developing into dark purple bumps (palpable Purpura) accompanied by abdominal and joint pain, and reduced kidney function

Henoch Schoenlein Purpura, or HSP, is a form of vasculitis, an inflammation of the small blood vessels and other tissues in the skin. In all cases, the condition presents as a vasculitic rash which begins as small, red spots, commonly forming on the buttocks, lower legs, knees and elbows. Within 24 hours, the rash will shift to dark purple purpura (bumps), and may become generalized as the condition progresses. This rash may be accompanied by blistering and/or ulceration in the affected areas. Joint pain, abdominal pain, and even reduced kidney function or permanent kidney damage may also accompany the rash. In extremely rare cases, other organs such as the brain, lungs or heart may be affected.

While HSP may affect any age group, it is typically seen in children. The exact cause is unknown, but many cases develop following respiratory infections. HSP has also been seen to develop following other types of infection, and exposures to certain foods and drugs.

Differential Diagnosis (Other conditions with similar appearance)
Hand-Foot-and-Mouth Disease
Disseminated Intravascular Coagulation
Kawasaki disease
Idiopathic Thrombocytopenic Purpura
Meningitis and Encephalitis
Systemic Lupus erythematosus
Chicken Pox or Varicella
Thrombocytopenic Purpura
Rocky Mountain spotted fever

Diagnosis
Key Points
Diagnosis based on skin appearance
Skin biopsy may be performed to confirm diagnosis
Blood and urine tests should be performed to determine level of involvement of the kidneys

Henoch Schoenlein Purpura is generally diagnosed based on the appearance of characteristic cutaneous symptoms. A skin biopsy may be performed to confirm the diagnosis, and rule out other conditions. Health care professionals should administer blood and urine tests to determine the level of involvement of the kidneys.

Treatment
Typically resolves spontaneously without treatment
Some persons may see reappearance of lesions over up to 16 weeks
*Goal of treatment is to control symptoms and prevent permanent damage

Henoch Schoenlein Purpura typically resolves spontaneously without treatment within approximately 1 week. Lesions may recur up to 16 weeks after the initial outbreak has healed, and in rare cases lesions may be persistent. Use of non-steroidal anti-inflammatories such as ibuprofen may be prescribed to alleviate pain associated with the condition. In severe cases, oral steroids, Dapsone, and colchine may be used to combat symptoms. In affected persons who experience serious kidney involvement, health care professionals may prescribe steroids in combination with immunosuppressants or Immunoglobulin to combat permanent damage.