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A 74-year-old man with a history of rheumatoid disease, and a left lower lobectomy for carcinoma of lung 25 years previously was admitted as an emergency to hospital with a 3-day history of a purpuric rash. The rash was noted initially on the feet and spread proximally to involve the thighs and buttocks with involvement of the extensor surface of the upper limbs but sparing of the trunk. There were no other symptoms apart from mild colicky abdominal pain and a minor sore throat before the onset of the rash. His rheumatoid disease was quiescent at the time and there had been no recent changes in his drug therapy.
Examination confirmed the presence of a purpuric rash (figures 1and 2) in the absence of systemic illness. Routine blood testing was normal aside from a slightly elevated urea at 7.6 mmol/l with a normal creatinine. A clotting screen was also within normal limits. The erythrocyte sedimentation rate was 26 mm/h and a mid-stream urinalysis revealed microscopic haematuria with 1 + red blood cells and no growth. During his period of hospitalisation he passed a melaena stool on one occasion but there was no haemodynamic compromise and all parameters remained normal.
- What is the probable diagnosis ?
- What complications can occur ?
Henoch-Schönlein purpura (HSP). This is an inflammatory disorder of blood vessels characterised by non-thrombocytopenic purpura, commonly distributed over the buttocks and lower extremities, arthralgia, abdominal pain and a form of glomerular nephritis. It is mediated by immune complex deposition, predominantly IgA, which affects the small blood vessels resulting in a local vasculitis. Histologically the predominant cell type is the polymorphonuclear leucocyte.
Complete recovery from this condition is the rule, but complications can occur (box).
Complications of Henoch-Schönlein purpura
abdominal pain, peritonitis, bowel perforation, gastrointestinal blood loss
Henoch-Schönlein purpura preferentially affects children between the ages of 5 and 15 years with a slight bias towards the male gender (M:F = 1.5:1). In this population, which has been extensively studied, the condition is deemed to be benign and self-limiting with few complications. This disorder can, however, be expressed in adult life and until recently received little attention. A comparison of HSP in children and adults revealed that differences do exist between the two population groups.1 Cutaneous lesions were found to be the main clinical manifestation in both groups and in the adult population joint symptoms were predominant over abdominal pain in comparison with the paediatric group. Similarly, adults were preferentially subject to more renal involvement than children and often required immunosuppressive treatment. Interestingly, with regard to this particular case, preceding upper respiratory tract infection was a more frequent feature amongst children.
Case reports have appeared in the literature underlining the potential for serious sequelae of HSP in adults with nephritis,2pulmonary haemorrhage,3 and bowel perforation4 being reported. There is also evidence for HSP as a cutaneous marker for underlying malignancy. An association with leukaemias and lymphomas, although rare, is recognised and one case report quotes the expression of HSP in tandem with the progression of a malignant breast tumour.5 Despite the different expression between the paediatric and adult forms, the final outcome in both groups is equally good.