Which dermatologic findings are characteristic of Henoch-Schönlein purpura (IgA vasculitis)?

Updated: Oct 28, 2020
  • Author: Rajendra Bhimma, MBChB, MD, PhD, DCH (SA), FCP(Paeds)(SA), MMed(Natal); Chief Editor: Craig B Langman, MD  more...
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Answer

In most patients, skin lesions are the first sign of IgAV. The eruption commonly begins as erythematous macular or urticarial lesions, progressing to blanching papules and later to palpable purpura, usually 2-10 mm in diameter (see the image below). Various stages of eruption may be present simultaneously. Lesions usually occur in crops and may fade over several days.

Purpuric papules and plaques of the lower extremity characteristic of IgA vasculitis (Henoch-Schönlein purpura).

Purpuric papules and plaques of the lower extremit Purpuric papules and plaques of the lower extremity characteristic of IgA vasculitis (Henoch-Schönlein purpura).

Lesions typically are symmetrical and tend to be distributed in dependent body areas, such as the ankles and lower legs in older children and adults (see the images below), and the back, buttocks, upper extremities, and upper thighs in young children (because these regions tend to be dependent in young children). The face, palms, soles, and mucous membranes usually are spared, except in infants, in whom facial involvement may not be uncommon.

Typical rash distribution of IgA vasculitis (Henoch-Schönlein purpura).

Typical rash distribution of IgA vasculitis (Henoc Typical rash distribution of IgA vasculitis (Henoch-Schönlein purpura).

A 9-year-old boy with IgA vasculitis (Henoch-Schönlein purpura). Note confluence of purpura around the ankles. Image courtesy of Pamela L Dyne, MD.

A 9-year-old boy with IgA vasculitis (Henoch-Schön A 9-year-old boy with IgA vasculitis (Henoch-Schönlein purpura). Note confluence of purpura around the ankles. Image courtesy of Pamela L Dyne, MD.

A 7-year-old girl with IgA vasculitis (Henoch-Schönlein purpura). Image courtesy of Pamela L Dyne, MD.

A 7-year-old girl with IgA vasculitis (Henoch-Schö A 7-year-old girl with IgA vasculitis (Henoch-Schönlein purpura). Image courtesy of Pamela L Dyne, MD.

Hemorrhagic macules, papules, and patches on the ankle and foot of a child with IgA vasculitis (Henoch-Schönlein purpura).

Hemorrhagic macules, papules, and patches on the a Hemorrhagic macules, papules, and patches on the ankle and foot of a child with IgA vasculitis (Henoch-Schönlein purpura).

Palpable purpura can also be present on the forearms and pinnae. Scalp edema can occur. Hemorrhagic vesicles and bullae are rare.

Within 12-24 hours, the macules evolve into purpuric lesions that are dusky red and have a diameter of 0.5-2 cm. The lesions may coalesce into larger plaques that resemble ecchymoses (see the image below). Color in the areas of purpura progresses from red to purple and then becomes rust-colored or brown before fading. Recurrences tend to take place in the same sites as previous lesions.

Older lesions of IgA vasculitis (Henoch-Schönlein purpura) demonstrating increased extravasation with ecchymoses on dorsal foot and ankle.

Older lesions of IgA vasculitis (Henoch-Schönlein Older lesions of IgA vasculitis (Henoch-Schönlein purpura) demonstrating increased extravasation with ecchymoses on dorsal foot and ankle.

Hives, angioedema, and target lesions can also occur. Vesicular eruptions and swelling and tenderness of an entire limb have been noted. Erythema multiforme–like lesions can be present. IgAV with hemorrhagic bullae in children has been noted.

In children younger than 2 years, the clinical picture may be dominated by edema of the scalp, periorbital area, hands, and feet (AHEI). The severity of edema is correlated with the severity of the vasculitis and not with the degree of proteinuria. However, the edema has been attributed to the enteric loss of protein. The “cockades” characteristic of AHEI display variable stages of evolution at any given time and look different from the normal purpura of IgAV.

The subcutaneous edema of AHEI is more common in infants. Urticaria, petechiae, and necrosis of the ear lobe are additional rare skin manifestations of AHEI. AHEI is rarely associated with visceral involvement.


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