R. Bissonnette et al., PERFORATION OF LARGE AND SMALL-BOWEL IN HENOCH-SCHONLEIN PURPURA, International journal of dermatology, 36(5), 1997, pp. 361-363
A 43-year-old woman with an unremarkable medical history was admitted
with severe polyarthralgia, purpuric papules on her lower legs (Fig. 1
), and recent pain with blue discoloration of her third left finger, S
he was taking no medication, At the time of presentation the abdominal
examination was normal, Laboratory evaluation showed normal or negati
ve values of BUN, creatinine, AST WBC, Hb, VDRL, rheumatoid factor, HB
SAg, ANA, ASO, cryoglobulins, cryofibrinogen, anticardiolipins, anti-S
m, anti-RNP, anti-Re, anti-La, anti-SC170, and anti-centromere. Hepati
tis C titer was not obtained. Sedimentation rate was 61 mm/h and immun
e circulating complexes were present (14.2 mu g/ml; normal, <1.5 mu g/
ml). Urine sediment analysis revealed the presence of cellular, hyalin
, granular. and erythrocytic casts. IgA level was 1.98 g/l (normal, 0.
5-3.0 gill. Skin biopsy showed extensive leucocytoclastic vasculitis.
Deposits of IgA, IgM, and C3 were observed in the dermal vessels under
direct immunofluorescence microscopy. The patient was put on predniso
ne 60 mg/day, Three days after admission she complained of acute abdom
inal pain. Increased abdominal tenderness was noted on examination. An
abdominal angiogram was normal, Because of the deteriorating intestin
al condition, she received methylprednisolone 1 g IV per day. Despite
this treatment she developed acute peritonitis 2 days later. Laparotom
y disclosed three perforations on the caecum and ascending colon, and
one on the ileum, She underwent sub-total colectomy with terminal ileo
stomy. Following surgery she was kept on parenteral methylprednisolone
1 g daily; cyclophosphamide 300 mg daily was started with progressive
improvement of her condition. The histopathologic examination of the
colonic segment disclosed vasculitis of the medium-sized vessels (Fig.
2), Direct immunofluorescence microscopy revealed strong deposits of
IgA and C3 in the vessel walls of the large intestine (Fig. 3).