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International Journal of Pharmaceutical Science Invention
ISSN (Online): 2319 – 6718, ISSN (Print): 2319 – 670X
www.ijpsi.org || Volume 4 Issue 5|| May 2015 || PP.13-14
www.ijpsi.org 13 | Page
Henoch Scholien Purpura: Not Just a Pediatric Diagnosis
Dr. Ankit Manglunia, Dr. Piyush Kumar, Dr. Vineet Kumar, Dr. Saumya
Mathur, Dr. Aashish Sharma, Dr. Prakhar Garg, Dr. Vimal Mathur, Dr. GN
Saxena
Department Of Medicine, MGUMST, Jaipur
ABSTRACT : Henoch Schonlein Purpura (HSP) is a systemic vasculitis mediated by IgA and characterised by
the clinical triad of non-thrombocytopenic palpable purpura, abdominal pain and arthritis. In addition, there
may be varying degree of renal involvement. It is the most frequent vasculitis in children and the incidence in
adults varies.
CASE REPORT
39 year old male presented with history of recurrent renal stones since last 8-10 years. He underwent PCNL
right side for the same in January 2015. Platelet counts were also 81000/uL during the same time. He developed
non blanchable rash on the extremities and on lower abdomen and trunk a week later. The skin lesions were non
pruritic and the lesions were both palpable and non-palpable. Patient developed left knee swelling associated
with pain followed by right knee swelling and pain 3 days later and then the patient was admitted in our
hospital. Laboratory Investigations showed HB – 14.2 gm/dL, WBC – 11.65*103 /uL with Neutrophils 66.4%,
Eosinoplils- 1.1%, platelet count were 1.5 lakhs .Renal function tests and serum electrolytes were within normal
range. Liver Function Test revealed slightly raised levels of SGOT/SGPT [89/187] with normal bilirubin level.
Urine analysis was normal. ANA, C3 C4 levels was normal.Ig-A levels was 391mg/dl.Skin Biopsy was taken
which showed focal upper dermal perivascular neutrophils with mild leucocytoclasis and some extravasated
RBCs. Deeper sections shows similar features. Immunofluorescence stains show granular fluorescence of IgA in
the papillary dermal vessels. Findings was suggestive of Small Vessel Vasculitis.
Figure 1: Skin Lesion of the Patient on Abdomen
Figure 2:Skin Lesion of the Patient on Lower Extremity
Henoch Scholien Purpura: Not Just A…
www.ijpsi.org 14 | Page
DISCUSSION
HSP is a small vessel vasculitis characterised by IgA complexes deposition in tissues. It generally has a self-
limiting course, and its main clinical manifestations can be seen in skin, the gastrointestinal tract, and the
kidneys. Less frequently other organs and/or system, such as lungs, the CNS and the genito-urinary tract can be
affected.
Though the etiology of the disease is unknown, multiple infectious agents have been suggested to be
responsible. In adults, renal involvement and the possibility of progression to renal failure is greater as
compared to children. The prevalence is greater in males.
Although there are no randomised control trials that prove the efficacy of one therapy over another (especially
for kidney involvement), corticosteroids have been used at high doses, either orally or in pulses of
methylprednisolone, and in association with immunosuppressive agents, such as cyclophosphamide or
azathioprine.
Although HSP is infrequent in adults, renal involvement deserves special attention because it darkens the
prognosis. Thus these patients should be detected and treated as early as possible.
CONCLUSION
Henoch-Scholien purpura is a fairly common paediatric disorder. The prognosis for the majority of patients is
very good. Less than 2% of patients develop a serious complication associated with long-term morbidity. The
most common serious complication is end-stage kidney disease, which may develop late in the disease after the
symptoms from other organ systems have resolved. Patients with HSP should be carefully followed for several
years to look for evidence of late renal involvement, and those patients who develop renal involvement should
be followed regularly throughout their lifetime. Primary care physicians should be well aware of the disease
because the true incidence is probably underestimated.
REFERENCES
[1]. Tancrede-Bohin E, Ochorrisky S, Vignon-Pennamen MD, Flaseul B, Morel P, Rybojad M:Schonlein-Henoch purpura in adult
patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases. Arch Dermatol 1997, 133:438-
442.
[2]. Ly MN, Breza TS Jr: Henoch-Schonlein purpura in an adult. Skin Med 2003, 2:262-264.
[3]. Austin HA 3d, Balow JE: Henoch-Schönlein nephritis: Long-term prognostic features and the challenge of therapy. Am J
Kidney Dis 1983, 2:512-520.
[4]. Sohagia AB, Gunturu SG, Tong TR, Hertan HI. Henochschönlein purpura-a case report and review of the literature.
Gastroenterol Res Pract 2010;597648:1-7.
[5]. Reamy BV, Williams PM, Lindsay TJ. Henoch-Schönlein purpura. Am Fam Physician 2009;80:697-704.
[6]. Sinclair P. Henoch –Schönlein Purpura – A review. Curr Allergy ClinImmunol 2010;23:116-20.

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Henoch Scholien Purpura: Not Just a Pediatric Diagnosis

  • 1. International Journal of Pharmaceutical Science Invention ISSN (Online): 2319 – 6718, ISSN (Print): 2319 – 670X www.ijpsi.org || Volume 4 Issue 5|| May 2015 || PP.13-14 www.ijpsi.org 13 | Page Henoch Scholien Purpura: Not Just a Pediatric Diagnosis Dr. Ankit Manglunia, Dr. Piyush Kumar, Dr. Vineet Kumar, Dr. Saumya Mathur, Dr. Aashish Sharma, Dr. Prakhar Garg, Dr. Vimal Mathur, Dr. GN Saxena Department Of Medicine, MGUMST, Jaipur ABSTRACT : Henoch Schonlein Purpura (HSP) is a systemic vasculitis mediated by IgA and characterised by the clinical triad of non-thrombocytopenic palpable purpura, abdominal pain and arthritis. In addition, there may be varying degree of renal involvement. It is the most frequent vasculitis in children and the incidence in adults varies. CASE REPORT 39 year old male presented with history of recurrent renal stones since last 8-10 years. He underwent PCNL right side for the same in January 2015. Platelet counts were also 81000/uL during the same time. He developed non blanchable rash on the extremities and on lower abdomen and trunk a week later. The skin lesions were non pruritic and the lesions were both palpable and non-palpable. Patient developed left knee swelling associated with pain followed by right knee swelling and pain 3 days later and then the patient was admitted in our hospital. Laboratory Investigations showed HB – 14.2 gm/dL, WBC – 11.65*103 /uL with Neutrophils 66.4%, Eosinoplils- 1.1%, platelet count were 1.5 lakhs .Renal function tests and serum electrolytes were within normal range. Liver Function Test revealed slightly raised levels of SGOT/SGPT [89/187] with normal bilirubin level. Urine analysis was normal. ANA, C3 C4 levels was normal.Ig-A levels was 391mg/dl.Skin Biopsy was taken which showed focal upper dermal perivascular neutrophils with mild leucocytoclasis and some extravasated RBCs. Deeper sections shows similar features. Immunofluorescence stains show granular fluorescence of IgA in the papillary dermal vessels. Findings was suggestive of Small Vessel Vasculitis. Figure 1: Skin Lesion of the Patient on Abdomen Figure 2:Skin Lesion of the Patient on Lower Extremity
  • 2. Henoch Scholien Purpura: Not Just A… www.ijpsi.org 14 | Page DISCUSSION HSP is a small vessel vasculitis characterised by IgA complexes deposition in tissues. It generally has a self- limiting course, and its main clinical manifestations can be seen in skin, the gastrointestinal tract, and the kidneys. Less frequently other organs and/or system, such as lungs, the CNS and the genito-urinary tract can be affected. Though the etiology of the disease is unknown, multiple infectious agents have been suggested to be responsible. In adults, renal involvement and the possibility of progression to renal failure is greater as compared to children. The prevalence is greater in males. Although there are no randomised control trials that prove the efficacy of one therapy over another (especially for kidney involvement), corticosteroids have been used at high doses, either orally or in pulses of methylprednisolone, and in association with immunosuppressive agents, such as cyclophosphamide or azathioprine. Although HSP is infrequent in adults, renal involvement deserves special attention because it darkens the prognosis. Thus these patients should be detected and treated as early as possible. CONCLUSION Henoch-Scholien purpura is a fairly common paediatric disorder. The prognosis for the majority of patients is very good. Less than 2% of patients develop a serious complication associated with long-term morbidity. The most common serious complication is end-stage kidney disease, which may develop late in the disease after the symptoms from other organ systems have resolved. Patients with HSP should be carefully followed for several years to look for evidence of late renal involvement, and those patients who develop renal involvement should be followed regularly throughout their lifetime. Primary care physicians should be well aware of the disease because the true incidence is probably underestimated. REFERENCES [1]. Tancrede-Bohin E, Ochorrisky S, Vignon-Pennamen MD, Flaseul B, Morel P, Rybojad M:Schonlein-Henoch purpura in adult patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases. Arch Dermatol 1997, 133:438- 442. [2]. Ly MN, Breza TS Jr: Henoch-Schonlein purpura in an adult. Skin Med 2003, 2:262-264. [3]. Austin HA 3d, Balow JE: Henoch-Schönlein nephritis: Long-term prognostic features and the challenge of therapy. Am J Kidney Dis 1983, 2:512-520. [4]. Sohagia AB, Gunturu SG, Tong TR, Hertan HI. Henochschönlein purpura-a case report and review of the literature. Gastroenterol Res Pract 2010;597648:1-7. [5]. Reamy BV, Williams PM, Lindsay TJ. Henoch-Schönlein purpura. Am Fam Physician 2009;80:697-704. [6]. Sinclair P. Henoch –Schönlein Purpura – A review. Curr Allergy ClinImmunol 2010;23:116-20.