Henoch-Schönlein purpura (HSP) is a systemic leukocytoclastic vasculitis involving arterioles and venules most commonly in the skin, glomeruli, and gastrointestinal tract. In skin, it is associated with IgA deposition around dermal blood vessels. While an exact cause of HSP has not been elucidated, several processes have been implicated in its development, including infections; drugs; and allergic, rheumatologic, and neoplastic diseases. We present a 57-year-old woman with a history of follicular lymphoma who developed HSP likely associated with myelodysplastic syndrome. This case is clinically significant because the patient was thought to be in remission of her hematologic disease until her skin findings prompted further evaluation. Her diagnosis of HSP was based on clinical presentation with palpable purpura and abdominal pain, and was confirmed with biopsy and immunohistochemical analyses of purpuric papules demonstrating leukocytoclastic vasculitis and strong anti-IgA labeling in the dermal endothelial cells consistent with immunocomplex deposition. The occurrence of vasculitis and malignant disease in the same patient often is difficult to interpret, as some patients may exhibit both diseases independently and by chance, while others may have vasculitis as a paraneoplastic syndrome. We review cases of adult HSP associated with malignancy in the literature.
Case Report
A 57-year-old woman with a history of follicular lymphoma in complete remission presented to the dermatology clinic with a 3-week history of a new asymptomatic erythematous rash distributed across her arms, legs, and buttocks. She denied the use of any new medications but did report resuming use of pilocarpine 3 months prior to presentation and ursodiol 2 months prior to presentation. She had previously taken both of these medications without adverse effects. On initial presentation, the patient denied fever, chills, nausea, vomiting, dyspnea, arthralgia, or myalgia. She denied any upper respiratory tract symptoms or preceding viral illness. The patient's prior medical history included diabetes mellitus, hypertension, follicular lymphoma in remission, and severe chemotherapy-induced peripheral neuropathy. Her follicular lymphoma initially was treated with chemotherapy. She relapsed and was treated with an allogeneic bone marrow–derived stem cell transplant from an HLA antigen–identical sister approximately 9 years prior to presentation. The patient's medications included pilocarpine, ursodiol, a fentanyl citrate patch, gabapentin, nortriptyline, duloxetine, clonidine hydrochloride, magnesium oxide, methylphenidate hydrochloride, metformin, and glyburide. She reported no known drug allergies. Full cutaneous examination revealed nonblanching erythematous macules and papules on the bilateral lower extremities, with scattered macules and papules on the upper arms, abdomen, and buttocks. Results of a punch biopsy showed a predominant perivascular infiltrate of lymphocytes, neutrophils, and eosinophils. There was focal endothelial swelling with mural fibrin deposits, extravasation of red blood cells, and rare necrotic keratinocytes consistent with leukocytoclastic vasculitis (Figure 1). Triamcinolone acetonide cream 0.1% was prescribed pending laboratory evaluation, which ultimately revealed a negative antinuclear antibody, negative rheumatoid factor, white blood cell count of 4.2 k/µL (reference range, 4–11 k/µL), hemoglobin level of 7.8 g/dL (reference range, 12–16 g/dL), and platelet count of 192 k/µL (reference range, 140–440 k/µL). A urinalysis was negative for red blood cells, proteins, and nitrites, but did show small leukocyte esterase and 5 to 10 white blood cells per high-power field.
Two weeks later, the patient complained of crampy abdominal pain (requiring morphine); decreased appetite; weakness; headache, as well as nausea; and extension of the eruption to involve her arms, legs, and entire trunk. She denied melena, hematochezia, and any urinary tract symptoms. Physical examination revealed diffuse tenderness in the epigastric area and a nonpalpable liver and spleen. Full cutaneous examination showed hemorrhagic bullae on the patient's lower extremities (Figure 2) and palpable purpura on her arms. Since the initial presentation 2 months prior, the patient's hematocrit level had dropped approximately 10% from 34.6% to 24.3% (reference range, 41.0%–50.0%). Her white blood cell count was slightly lower (3.6 k/µL) and her platelet count was 144 k/µL. Of note, her D-dimer level was elevated (1846 ng/mL; reference range, 0–230 ng/mL), as was her fibrinogen level (613 mg/dL; reference range, 220–530 mg/dL). Repeat urinalysis showed no proteinuria, hematuria, or nitrites.
The patient subsequently was admitted to the hospital. A workup of her gastrointestinal tract, including a computed tomographic scan of the abdomen and endoscopy, failed to show an etiology for the abdominal pain. Her amylase and lipase levels were normal, and stool cultures and guaiac occult blood tests were negative. Biopsies performed during endoscopy showed mild acute colitis of the hepatic flexure and the ileocecal valve. The patient was prescribed a prophylactic regimen of meropenem and metronidazole for colitis, though no precise etiology was identified. An anti-IgA immunostudy was performed on paraffin sections of the original skin biopsy and showed strong labeling in the dermal endothelial cells consistent with immunocomplex deposition (Figure 3). The patient was diagnosed with Henoch-Schönlein purpura (HSP) and her rash and abdominal pain improved with prednisone 60 mg daily. Biopsy of a bone marrow specimen showed myelodysplastic changes and a cellular bone marrow with trilineage dyspoiesis and increased blasts.