Henoch-Schonlein Purpura Successfully Treated with Dexamethasone: A Case Report of Six-Year-Old Female

Abstract

Henoch-Schonlein Purpura (HSP) is one of the most common causes of small vessel vasculitis in children. A six-year-old female presented with abdominal pain, swelling and rashes over both the legs associated with multiple episodes of vomiting for around ten days. Stool for the occult blood test was positive but there was an absence of hematuria and albuminuria. The case was diagnosed as HSP and treated with dexamethasone for ten days. The patient was finally discharged on the resolution of her symptoms. Early diagnosis and treatment favor the better outcome in cases without any renal complications. Keywords: Henoch-Schönlein Purpura, Vasculitis, Steroids, Dexamethasone.

Full text

Journal of Medical Research and Innovaon Volume 2, Issue 1, e000095
Direct Access: hps://jmri.org.in/jmri/arcle/view/e000095
1
Journal of Medical Research and Innovaon | Volume 2 | Issue 1 | e000095
Case Report
Henoch-Schonlein Purpura Successfully Treated
with Dexamethasone: A Case Report of Six-year-
old Female
Purushoam Adhikari
Department of Medicine, Gandaki Medical College, Nepal
Address for correspondence: Purushoam Adhikari, Department of Medicine, Gandaki Medical
College, Nepal. E-mail: puru.ad12@gmail.com
Abstract
Henoch-Schonlein Purpura (HSP) is one of the most common causes of small vessel vasculis in
children. A 6-year-old female presented with abdominal pain, swelling, and rashes over both the legs
associated with mulple episodes of voming for around 10 days. Stool for the occult blood test was
posive, but there was an absence of hematuria and albuminuria. The case was diagnosed as HSP
and treated with dexamethasone for 10 days. The paent was nally discharged on the resoluon of
her symptoms. Early diagnosis and treatment favor the beer outcome in cases without any renal
complicaons.
Keywords: Henoch-Schonlein Purpura, Vasculis, Steroids, Dexamethasone
How to cite this arcle:
Adhikari P. Henoch-
Schonlein Purpura
Successfully Treated
with Dexamethasone:
A Case Report of Six-
year-old Female. Journal
of Medical Research
and Innovaon.
2018;2(1):e000095.
Doi: 10.15419/jmri.95.
Publicaon history:
Received: 29/09/2017
Accepted: 01/11/2017
Published: 01/11/2017
Editor: Dr. Surya Parajuli
Copyright: Adhikari P.
This s an open access
arcle distributed under
the terms of the Creave
Commons Aribuon
License CC-BY 4.0., which
permits unrestricted
use, distribuon, and
reproducon in any
medium, providead
the original author and
source are credited.
Funding: NIL
Conict of Interest: NIL
Quick Access Code
Introducon
Henoch-Schonlein Purpura (HSP), also known as
IgA vasculis, is a small vessel vasculis with IgA1-
dominant immune deposits predominantly on
capillaries, venules, or arterioles. It oen involves
skin and gastrointesnal system and may also cause
arthris.[1] It is commonly seen in children and
characterized by palpable purpura more commonly
located in the dependent body parts such as lower
extremies and buocks, arthris/arthralgia, and
bowel angina along with hematuria/proteinuria.[2]
The various eologies have been suggested such as
variees of pathogens, drugs, and environmental
exposure among which Group A beta-hemolyc
Streptococcus has been much studied.[3]
The natural history of the disease has self-
liming course in most of the cases except
that those of renal complicaons associated
with it. Symptomac treatment with non-
steroidal an-inammatory drugs along with
the steroids will have the joint and abdominal
pain relief.[4] Corcosteroids if given in the early
course of disease can help to provide a beer
clinical outcome.[5] The prognosis is good, with
excepon of renal involvement that may need
the follow-up ll 6 months or longer.[6,7]
The exact prevalence of HSP in our sengs
has yet to be discovered, but various studies
revealed it to be one of the important causes of
childhood renal disease.[8,9]
Case Report
The case is reported aer taking informed
wrien consent from the paent’s mother.
6-year-old female presented with the complaints
of pain in abdomen, swelling, and rashes
over the lower limbs associated with mulple
episodes of voming for 10 days. Pain in the
abdomen was localized around the umbilicus
and was sudden in onset, intermient in nature,
non-radiang, and alleviated on lying at on
the bed. She developed swelling in both legs
subsequently a day aer abdominal pain along
with the appearance of rashes starng from feet
and progressing to thigh and buocks. She also
developed three to four episodes of voming in
last 5 days. The voming was non-projecle and
non-blood stained.
On examinaon, the general condion of the
paent was fair and vitals were stable. The
abdomen was so and non-tender. Mild bilateral

Adhikari: Case report of six year old female with Henoch-Schonlein Purpura
Journal of Medical Research and Innovaon Volume 2, Issue 1, e000095
Direct Access: hps://jmri.org.in/jmri/arcle/view/e000095
2
non-ping edema was present over both the legs. There was
the presence of non-tender, non-blanching, purpuric rashes
over both lower limbs, and extending up to the buocks.
Laboratory tests showed leukocytosis with WBC count of
12,000/mm3; neutrophils: 8800/mm3; lymphocyte: 3200/
mm3; platelets: 4,22,000/mm3; hemoglobin: 14 g/dl; ESR:
12 mm/1st h; serum urea: 33 mg/dl; serum creanine: 0.6 mg/
dl; sodium: 139 mmol/l; potassium: 4.6 mmol/l; CRP: 0.92 mg/l;
urinalysis: No hematuria or proteinuria, and stool analysis
showed the presence of occult blood. The plain X-ray abdomen
and ultrasound abdomen/pelvis revealed no abnormalies.
Diagnosis of HSP was made in accordance with the American
College of Rheumatology and European League Against
Rheumasm (EuLAR) and Pediatric Rheumatology Society
(PReS) criteria.[10,11]
She was treated with dexamethasone 0.14 mg/kg/dose 4 mes
a day intravenously for 5 days connuously and then tapered
to 3 mes a day for 3 days following by 2 mes a day for next
2 days.
The recovery from purpura and bilateral swelling of the legs was
observed aer treatment in the 2nd and 3rd week [Figures 1-3].
Discussion
Henoch-Schonlein Purpura (HSP) was rst described by William
Heberden in 1801. Later, Schonlein recognized the associaon
between purpura and arthris, whereas Henoch reported a
case that also included gastrointesnal symptoms along with
the renal involvement.[12] HSP is the most common vasculis
of the children. Half among all the cases occur before the age
of ve and males are aected twice as common as females.[13]
The exact eology and pathogenesis of HSP are yet to be
determined. Seasonal variaon has been related with a high
prevalence rate in autumn and winter.[2] However, the case
described here was diagnosed in the spring season. It has also
been proposed that various triggers such as bacterial and viral
infecons, vaccinaons, drugs, and autoimmune mechanisms
may result in the formaon of an angen and anbody
complex and the deposion of such formed immune complex
in the small vessels may acvate the alternate complement
pathway leading to neutrophil aggregaon which results
in inammaon and vasculis.[14] Among all, the preceding
infecon of β-hemolyc Streptococcus has been one of the
most studied cases. The posive throat cultures, as well as
increased ters of an-streptolysin O, have been oen found in
many paents.[3] The evidence of prior infecon has not been
recorded in the present case.
The paent generally presents with the classic tetrad of rashes,
polyarthralgia, abdominal pain, and renal disease. The non-
blanching rashes clinically appear as a palpable purpura on
the lower legs and arms.[13] The joint involvement is generally
characterized by the pain and swelling of the joints, most
Figure 1: Purpura and swelling of bilateral lower limbs in the
1st week
Figure 2: Progress of purpura in the 2nd week aer the
admission of paent in hospital
Figure 3: 3rd week before the discharge from hospital

Adhikari: Case report of six year old female with Henoch-Schonlein Purpura
Journal of Medical Research and Innovaon Volume 2, Issue 1, e000095
Direct Access: hps://jmri.org.in/jmri/arcle/view/e000095
3
aecng the knees and ankles. The abdominal pain followed
by voming and intesnal bleeding is the dominant features
involving the gastrointesnal system. Microscopic hematuria
and albuminuria are the prominent renal ndings.[5,15]
Our case had the symptoms of rashes over both lower legs,
pain in abdomen, and voming. Joint involvement was absent.
Signicant laboratory nding was occult blood test posive
in stool. However, there were no signs of hematuria and
albuminuria.
The diagnosis of HSP was made by American College of
Rheumatology-1990 criteria [Table 1] and EuLAR/PReS - 2006
criteria [Table 2].
The paent is said to have HSP if at least two of the four above
criteria is present. In our case, there was the presence of
palpable purpura, age <20 at disease onset and bowel angina,
and thus was diagnosed as HSP.
The paent is said to have HSP if mandatory criteria are present
along with at least one of the addional criteria. Our case
fullled the mandatory criteria along with diuse abdominal
pain as the addional criteria.
There is a predominantly spontaneous resoluon of all
symptoms except that of the renal disease in the majority
of the cases. Steroids are more oen used for the relief of
abdominal pain, joint pain, and skin disease. Alternavely,
methotrexate and dapsone have been quite eecve (steroid-
sparing agent) for the treatment of chronic abdominal pain and
skin involvement.[4] The role of the corcosteroids in prevenng
the long-term outcome of renal complicaons is controversial.
A meta-analysis in the Medline database and the Cochrane
Controlled Trials Register based on a comprehensive review of
the literature by Weiss et al. stated that early treatment with
corcosteroid signicantly reduces the odds of developing
persistent renal disease along with surgical intervenon and
recurrence.[5] In contrary, other trials and updates in the literature
have menoned that there is no long-term renal protecve
outcome on the early treatment with prednisolone.[16,17]
In general, prednisolone is the commonly used steroid for the
treatment of HSP. Although in our case, we used dexamethasone,
there is no evidence in literature to prove the superiority of one over
another. Several cases and studies have been reported resulng in
beer outcomes on treatment with dexamethasone.[18,19]
The renal involvement has a high morbidity and mortality;
otherwise, the disease has beer prognosis.[7] A systemac
review by Narchi stated that even if urinalysis is normal at the
presentaon, follow-up urine tesng should be connued for
at least 6 months as 97% children will develop abnormal urine
ndings by that me.[6]
Rarely, few cases of complicated HSP as intussuscepons,
gastrointesnal bleeding and with cardiac involvement have
been reported.[18,20,21]
Conclusion
HSP being one of the most common vasculides of the children
and its classic presentaon of palpable purpura, arthris,
abdominal involvement, and renal features makes the diagnosis
quite easier. Early iniaon of treatment with steroids will help
in symptomac relief and bring a posive outcome. The renal
disease may need long-term follow-up; otherwise, the diseases
have favorable prognosis.
Acknowledgment
The author would like to acknowledge the paent’s mother
for providing consent for the case report. Furthermore,
authors would like to thank to the faculty of the Department
of Pediatrics, Gandaki Medical College, for their support,
guidance, and suggeson during the course of study.
References
1. Jennee JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F,
et al. 2012 revised internaonal chapel hill consensus
Table 1: The American College of Rheumatology - 1990 criteria for the diagnosis of HSP[10]
Criterion Denion
Palpable purpura Slightly raised “palpable” hemorrhagic skin lesions, not related to thrombocytopenia
Age < 20 at disease onset Paent 20 years or younger at onset of rst symptoms
Bowel angina Diuse abdominal pain, worse aer meals, or the diagnosis of bowel ischemia, usually including bloody diarrhea
Wall granulocytes on biopsy Histologic changes showing granulocytes in the walls of arterioles or venules
Table 2: EuLAR/PReS – 2006 criteria[11]
Criteria Denion
Mandatory
criteria
Palpable purpura
Addional
criteria
Diuse abdominal pain
Any biopsy showing predominant IgA deposion
Arthris or arthralgia
Renal involvement (any hematuria and/or
proteinuria)

Adhikari: Case report of six year old female with Henoch-Schonlein Purpura
Journal of Medical Research and Innovaon Volume 2, Issue 1, e000095
Direct Access: hps://jmri.org.in/jmri/arcle/view/e000095
4
conference nomenclature of vasculides. Arthris Rheum
2013;65:1-11.
2. Yang YH, Yu HH, Chiang BL. The diagnosis and classicaon
of Henoch-Schonlein purpura: An updated review.
Autoimmun Rev 2014;13:355-8.
3. Saulsbury FT. Clinical update: Henoch-Schonlein purpura.
Lancet (London, England) 2007;369:976-8.
4. Tizard EJ, Hamilton-Ayres MJ. Henoch schonlein purpura.
Archives of disease in childhood Educ Pract Ed 2008;93:1-8.
5. Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C.
Eects of corcosteroid on Henoch-Schonlein purpura:
A systemac review. Pediatrics 2007;120:1079-87.
6. Narchi H. Risk of long term renal impairment and duraon
of follow up recommended for Henoch-Schönlein purpura
with normal or minimal urinary ndings: A systemac
review. Arch Dis Child 2005;90:916-20.
7. Calvo-Rio V, Loricera J, Mata C, Marn L, Orz-Sanjuan F,
Alvarez L, et al. Henoch-Schonlein purpura in northern
Spain: Clinical spectrum of the disease in 417 paents
from a single center. Medicine 2014;93:106-13.
8. Bhaa NK, Shrestha P, Budhathoki S, Kalakhe BK, Poudel P,
Sinha A, et al. Prole of renal diseases in Nepalese children.
Kathmandu Univ Med J (KUMJ) 2008;6:191-4.
9. Shah G. Clinical prole and paern of Henoch-Schonlein
purpura in children. J Patan Acad Health Sci 2015;2:17-21.
10. Mills JA, Michel BA, Bloch DA, Calabrese LH, Hunder GG,
Arend WP, et al. The American college of rheumatology
1990 criteria for the classicaon of Henoch-Schonlein
purpura. Arthris Rheum 1990;33:1114-21.
11. Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC,
et al. EULAR/PReS endorsed consensus criteria for the
classicaon of childhood vasculides. Ann Rheum Dis
2006;65:936-41.
12. Ballinger S. Henoch-Schonlein purpura. Curr Opin Rheum
2003;15:591-4.
13. Roberts PF, Waller TA, Brinker TM, Rie IZ, Sayre JW,
Braon RL. Henoch-Schonlein purpura: A review arcle.
South Med J 2007;100:821-4.
14. Sohagia AB, Gunturu SG, Tong TR, Hertan HI. Henoch-
schonlein purpura-a case report and review of the
literature. Gastroenterol Res Pract 2010;2010:597648.
15. Chen O, Zhu XB, Ren P, Wang YB, Sun RP, Wei DE. Henoch
Schonlein purpura in children: Clinical analysis of 120 cases.
Afr Health Sci 2013;13:94-9.
16. Dudley J, Smith G, Llewelyn-Edwards A, Bayliss K, Pike K,
Tizard J. Randomised, double-blind, placebo-controlled
trial to determine whether steroids reduce the incidence
and severity of nephropathy in Henoch-Schonlein purpura
(HSP). Arch Dis Child 2013;98:756-63.
17. Bluman J, Goldman RD. Henoch-Schönlein purpura in
children: Limited benet of corcosteroids. Can Fam Phys
2014;60:1007-10.
18. Seo MK, Hong J, Yim HE, Pai KS. A paent with Henoch-
Schönlein purpura with intussuscepon and intractable
nephris. Child Kidney Dis 2016;20:92-6.
19. Shin JI, Lee SJ, Lee JS, Kim KH. Intravenous dexamethasone
followed by oral prednisolone versus oral prednisolone in
the treatment of childhood Henoch-Schonlein purpura.
Rheumatol Int 2011;31:1429-32.
20. Yamazaki T, Akimoto T, Iwazu Y, Sugase T, Takeshima E,
Numata A, et al. Henoch-Schonlein purpura complicated
with severe gastrointesnal bleeding. CEN Case Rep
2015;4:106-11.
21. Lutz HH, Ackermann T, Krombach GA, Grone HJ, Rauen T,
Floege J, et al. Henoch-Schonlein purpura complicated
by cardiac involvement: Case report and review of the
literature. Am J Kidney Dis 2009;54:e9-15.