Adult Henoch-Schönlein Purpura in a Patient With Myelodysplastic Syndrome and a History of Follicular Lymphoma
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.
More recently, Zurada et al23 presented the cases of 3 adults who developed HSP within months of diagnosis of a solid tumor or metastasis of a previously diagnosed malignancy. In their review of the world literature, the authors found that 31 cases of malignancy-associated HSP had been reported, and in most cases, the patients were male (94%; 29/31), presented with solid tumors (61%; 19/31), and developed HSP within one month of cancer diagnosis or detection of metastases (55%; 17/31).23
The occurrence of vasculitis and malignant disease in the same patient often is difficult to interpret because some patients may exhibit both diseases independently and by chance, while others may have vasculitis as a paraneoplastic syndrome.6 Currently, the principal sources of data on the association between HSP and malignancy are anecdotal case reports, which can be difficult to use to determine causality and relationship with significance. However, because of the relative rarity of paraneoplastic vasculitis, large studies are difficult, making case reports and smaller literature reviews of unusual presentations valuable.8
Conclusion
In summary, 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 that demonstrated leukocytoclastic vasculitis and strong anti-IgA labeling in the dermal endothelial cells consistent with immunocomplex deposition. The patient was placed on a corticosteroid taper and scheduled for stem cell transplantation, with marked improvement demonstrated clinically at her most recent visit.
