The clinical implications of adult-onset henoch-schonelin purpura

Abstract

Henoch-Schonlein Purpura (HSP) is a small vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Pathologically, it can be considered a form of immune complex-mediated leukocytoclastic vasculitis (LCV) involving the skin and other organs. Though it primarily affects children (over 90% of cases), the occurrence in adults has been rarely reported. Management often involves the use of immunomodulatory or immune-suppressive regimens.

Figure 1

A 56 year old man with prior history of hypertension, diabetes mellitus presented with a 4 week-duration skin eruption over the lower extremities (Figure 1A and 1B). This was accompanied by crampy lower abdominal pain and hematuria. Punch biopsy of involved skin demonstrated leukocytoclastic vasculitis, accompanied by deposition of IgA on vascular walls on direct immunofluorescent staining.

Figure 2

Examination revealed edema and a vesiculobullous rash in both hands and feet that showed varied degrees of healing (Figure 2A and B). Skin biopsy revealed the presence of a perivascular inflammatory infiltrate in the superficial dermal blood vessels, with nuclear dust and focal fibrinoid necrosis, consistent with leukocytoclastic vasculitis (Figure 2C; Hematoxylin and eosin stain-40x objective), accompanied by the deposition of IgA on vascular walls detected by direct immunofluorescent staining. Renal biopsy revealed focal segmental endocapillary proliferation (Figure 2D; Periodic acid- Schiff stain-40x objective) with positive immunofluorescence testing for mesangial IgA (Figure 2E; Immunoflurescence stain-40x objective)

Figure 3

In some HSP cases reported in the literature, overlap with microscopic polyangiitis (MPA) or Polyarteritisa Nodosa (PAN)-like disease have been reported, resulting in a plethora of severe renal and pulmonary manifestations.