Dermatologic Manifestations of Henoch-Schonlein Purpura
- Author: Andrew D Montemarano, DO; Chief Editor: William D James, MD more...
Overview
Henoch-Schönlein purpura (HSP), or anaphylactoid purpura, is an immunoglobulin (Ig) A-mediated, small-vessel vasculitis that predominantly affects children but also is seen in adults.[1] HSP is a subset of necrotizing vasculitis characterized by fibrinoid destruction of blood vessels and leukocytoclasis. Clinical manifestations primarily include palpable purpura, arthralgia or arthritis, abdominal pain, gastrointestinal (GI) bleeding, and nephritis. (See the images below.)
Go to Henoch-Schonlein Purpura for more information.
Purpuric papules and plaques of the lower extremity characteristic of Henoch-Schönlein purpura. 
Characteristic rash of Henoch-Schönlein purpura. The prodrome for HSP includes the following:
- Headache
- Anorexia
- Fever
After the prodrome, a rash, abdominal pain, peripheral edema, vomiting and/or arthritis develop. The hallmark of the disease is the characteristic palpable purpura.
Lesions in HSP tend to occur on the buttocks and upper thighs in younger children and on the feet, ankles, and lower legs of older children and adults only. (See the images below.)
Hemorrhagic macules, papules, and patches on the ankle and foot of a child with Henoch-Schönlein purpura. 
Older lesions of Henoch-Schönlein purpura demonstrating increased extravasation with ecchymoses on the dorsal foot and ankle. 
Typical rash distribution of Henoch-Schönlein purpura. Although purpura may be the presenting sign, as many as 50% of children present with symptoms other than purpura.
Although it may be present initially, renal disease often develops up to 3 months after the initial presentation of HSP.[2]
Disease recurrence occurs throughout weeks to months in adults and children. In a large pediatric study by Allen et al, children older than age 2 years had a recurrence rate of 50%, while those younger than age 2 years had a chance of recurrence of less than 25%.[3]
The primary differences between children and adults, according to one study of 57 adults with HSP, are the chronicity and severity of the eruption in the latter population.
Bullae and ulcers are more common in adults, and cutaneous exacerbations may be seen for 6 months or longer.
A full physical examination is indicated in HSP, since the disease can affect all organ systems.
Lesion Characteristics
In Henoch-Schönlein purpura (HSP), the eruption may begin as erythematous macular or urticarial lesions, progressing to blanching papules, and later to palpable purpura, usually 2-10 mm in diameter.
Bullae, vesicles, petechiae, and ecchymotic, necrotic, ulcerative, or target like lesions also may occur.
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 (as shown in the images below), and the back, buttocks, upper extremities, and upper thighs in young children, since these regions tend to be dependent in the latter group.
Hemorrhagic macules, papules, and patches on the ankle and foot of a child with Henoch-Schönlein purpura. Older lesions of Henoch-Schönlein purpura demonstrating increased extravasation with ecchymoses on the dorsal foot and ankle. Typical rash distribution of Henoch-Schönlein purpura. The face, palms, soles, and mucous membranes usually are spared, except in infants, in whom facial involvement may not be uncommon.
The subcutaneous edema prominent in children younger than age 2 years involves the scalp, periorbital regions, hands, feet, and scrotal area. 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.
Lesions usually occur in crops and may fade over several days. Recurrences tend to take place in the same sites as previous lesions.
Color in the areas of purpura progresses from red to purple and then becomes rust-colored or brown before fading.
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. Dec 2005;35(3):143-53. [Medline].
Takeuchi S, Soma Y, Kawakami T. IgM in lesional skin of adults with Henoch-Schönlein purpura is an indication of renal involvement. J Am Acad Dermatol. Dec 2010;63(6):1026-9. [Medline].
ALLEN DM, DIAMOND LK, HOWELL DA. Anaphylactoid purpura in children (Schonlein-Henoch syndrome): review with a follow-up of the renal complications. AMA J Dis Child. Jun 1960;99:833-54. [Medline].
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