Make the Diagnosis

A 63-year-old white female presented with a 2-week history of hemorrhagic purpuric lesions and necrotic vesicles on the bilateral lower extremities.

A 63-year-old white female presented with a 2-week history of hemorrhagic purpuric lesions and necrotic vesicles on the bilateral lower extremities.

Nearly 1 year prior to presentation, the patient underwent surgical resection for lung cancer. The patient also complained of joint swelling and pain in her ankles. She denied abdominal pain. She denied recent illness, including sore throat and upper respiratory infection. Skin biopsies were performed, including for direct immunofluorescence.

Make the Diagnosis

Urticarial vasculitis

Cryoglobulinemic vasculitis

Henoch-Schönlein purpura (IgA vasculitis)

Wegener granulomatosis (granulomatosis with polyangiitis)

Vasculitis is a process in which blood vessels become inflamed and necrotic. Classic small-vessel vasculitis reveals a leukocytoclastic vasculitis and most commonly presents as palpable purpura. In addition to skin, organs such as joints, kidneys, and intestines can be involved.

Henoch-Schönlein Purpura (HSP), also called anaphylactoid purpura, is a subtype of small-vessel vasculitis where immunoglobulin A (IgA) is deposited in the vessel walls. It is the most common form of vasculitis in children (usually aged 4-8 years). The incidence is higher in the winter. Some patients experience a prodrome of fever, colicky abdominal pain, and joint pain prior to the development of cutaneous symptoms. Disease in children tends to be self-limited. Adults may present with HSP as well, and often exhibit more severe disease that may become chronic with relapses and is more difficult to treat. In both children and adults, infectious causes, such as streptococcus pharyngitis, are the most common trigger. In adults, malignancy may be associated with HSP. A literature search revealed medications implicated in HSP such as antibiotics (vancomycin, penicillin, cephalosporins, clarithromycin), ACE inhibitors, and nonsteroidal anti-inflammatories. Many cases of HSP are idiopathic.

Patients present with erythematous macules that progress to purpura on the extremities. Lesions may be vesicular or bullous and may become necrotic and ulcerate. Arthralgias, often of lower-extremity joints, may be present. Abdominal pain and renal disease may occur in both children and adults. Adults are more likely to develop chronic kidney disease and must be followed carefully with serial blood work and urinalysis to evaluate for hematuria and proteinuria. Severe abdominal pain is an emergency as intussusception may occur.

Dr. Donna Bilu Martin, a dermatologist in private practice in Aventura, Fla.

Dr. Donna Bilu Martin

Histologically, leukocytoclastic vasculitis of small vessels is present. On direct immunofluorescence of perilesional skin, IgA, C3, and fibrin deposits can be seen. Serum IgA is unreliable and may be seen in healthy adults as well.

Treatment is generally supportive as the disease is self-limited. The use of corticosteroids is controversial. This may be effective for joint inflammation, abdominal disease, active nephritis, and ulcerated skin lesions, but doesn’t prevent the recurrence of skin lesions. Dapsone or colchicine can be used for resistant cutaneous lesions. In severe cases, intravenous immunoglobulin may be warranted.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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