HENOCH SCHOENLEIN PURPURA

Section Editor

Dr. A. Nagarajan

Department of Pulmonology, Kauvery Hospital, Cantonment, Trichy

Pushkala Mittur Sundararajan*

Department of Paediatrics, Kauvery Hospital, Chennai, Tamilnadu, India

History

A seven-year-old boy, thriving well, who presented with a fever associated with cough and cold. He was given symptomatic treatment for the same and the fever resolved.

Associated Illnesses

The next day, he developed small erythematous palpable rashes, initially over the left ankle, non-pruritic, non-painful, which gradually progressed proximally to involve both the lower limbs over the next day as well as right hand and was associated with pain and swelling in the left knee joint as well. Two days later, he developed pain all over the abdomen which was constant, at times colicky in nature, aggravated with meals.

What is your diagnosis?

Answer

Henoch Schoenlein purpura.

Description

Henoch-Schoenlein purpura (HSP) is a self-limited, systemic, non-granulomatous, autoimmune complex, small vessel vasculitis, with multi-organ involvement.

The incidence varies from 6.2 to 70.3 per 1,00,000 in children with slight male predominance (M:F = 1.2:1.0). Peak age incidence is 4 – 6 years and 90% of HSP cases occur before the age of 10 years.

Cutaneous manifestations include non-thrombocytopenic rash which evolves from erythematous to urticarial and macular wheels to non-blanching palpable purpura with petechiae and ecchymoses.

Palpable purpura presents in groups, symmetrical, in the lower extremities, buttocks and upper extremities lasting up to 3 – 10 days.

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