Reply to J. I. Shin et al.

We thank Drs. Jae Il Shin and Jae Seung Lee for their interest in our article ”Henoch-Schönlein purpura (HSP) in an elderly patient with unusual manifestation” [1]. They, like other authors, suggest the utility of measurement of factor XIII activity in the diagnosis of atypical HSP without skin rash. We agree with them that the measurement of plasma factor XIII activity might be helpful in the diagnosis of atypical HSP. Nevertheless all the patients in the reports cited by Lee & Shin were children with severe abdominal pain [2] [3]. Abdominal pain is one of the major manifestations of HSP [4]; however, it was absent in our patient. Moreover, as Gunasekaran et al. reported, decrease of factor XIII activity is not specific for HSP [3].

Therefore, it was difficult to make a definite diagnosis of HSP in our patient at an early stage. In fact, factor XIII was also examined in our patient on hospital day 8, when purpuric lesions appeared but a definite diagnosis had not yet been established; a decreased level was found (15 %; normal range 70 %-140 %). Methylprednisolone pulse therapy, factor XIII administration, and plasmapheresis were started as rescue treatment before the pathologic diagnosis of the skin; however, his condition led to a fatal outcome.

In addition, Shin & Lee suggest the possibility of the presence of polyangitis overlap syndrome in our patient, because the clinical course was similar to that of the patient with HSP combined with polyarteritis nodosa reported by Watanabe et al. [5]. However, unlike the patient of Watanabe et al., our patient did not have the characteristic purpuric rash, arthralgia, and renal dysfunction on admission. Also, postmortem examination in our patient demonstrated leukocytoclastic vasculitis that affected small vessels (arterioles and venules), but not the small to medium-sized arteries that are predominantly affected by polyarteritis nodosa.

In conclusion, we should bear in mind that gastrointestinal bleeding even without skin rash or abdominal pain could be an initial manifestation of HSP, and that this condition can progress to a fatal outcome whether or not polyangitis overlap syndrome is present.

Competing interests: None

References

• 1 Uza N, Yazumi S, Tanabe K. et al . Henoch-Schönlein purpura in an elderly patient with unusual manifestation.  Endoscopy. 2007;  39 E35-E36
• 2 Kaneko K, Fujii S, Shono T. et al . Diagnostic value of plasma factor XIII in Henoch-Schönlein purpura.  Pediatr Nephrol. 2004;  19 702-703
• 3 Gunasekaran T S, Berman J, Gonzalez M. Duodenojejunitis: is it idiopathic or is it Henoch-Schönlein purpura without the purpura?.  J Pediatr Gastroenterol Nutr. 2000;  30 22-28
• 4 Saulsbury F T. Henoch-Schönlein purpura.  Curr Opin Rheumatol. 2001;  13 35-40
• 5 Watanabe K, Abe H, Mishima T. et al . Polyangitis overlap syndrome: a fatal case combined with adult Henoch-Schönlein purpura and polyarteritis nodosa.  Pathol Int. 2003;  53 569-573