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Case Report
Bilateral Testicular Infarction from IgA Vasculitis of
the Spermatic Cords
Mazen Toushan,1Ashka Atodaria,2Stephen D. Lynch,2
Hassan D. Kanaan,1Limin Yu,1Mitual B. Amin,1Mamon Tahhan,2Ping L. Zhang,1
Paul S. Kellerman,3and Abhishek Swami3
1Division of Anatomic Pathology, Department of Pathology, Beaumont Health, Royal Oak, MI, USA
2Department of Internal Medicine, Beaumont Health, Royal Oak, MI, USA
3Division of Nephrology, Department of Internal Medicine, Beaumont Health, Royal Oak, MI, USA
Correspondence should be addressed to Abhishek Swami; abhishek.swami@beaumont.edu
Received 9 August 2017; Accepted 29 October 2017; Published 21 November 2017
Academic Editor: Ze’ev Korzets
Copyright © Mazen Toushan et al. is is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
A -year-old man with type diabetes mellitus and chronic obstructive pulmonary disease presented to the emergency room with
increasing bilateral leg pain, rash, and scrotal swelling with pain. Skin biopsy from his thigh revealed IgA-associated vasculitis. Due
to hematuria, a renal biopsy was performed and showed an IgA glomerulonephritis with focal brinoid necrosis and neutrophil
accumulation. Bilateral orchiectomies were performed in two separate procedures ten and thirteen days aer the renal biopsy, as
a result of uncontrolled abscess formation in testicles. Microscopically, both testicles revealed large abscess formation destroying
almost the entire testicular parenchyma without tumor cells. Spermatic cord margins were further scrutinized microscopically to
show bilateral vasculitis in many small size vessels, conrmed by positive endothelial staining for IgA. Some of the aected arteries
revealed central organizing thrombi with recanalization features, highly suggestive of vasculitis-associated thrombi formation,
resulting in testicular ischemic infarction and abscess formation. We conclude that this adult patient developed a severe form
of Henoch-Sch¨
onlein purpura, with vasculitis aecting multiple organs, including the most serious and unusual complication of
bilateral testicular infarction.
1. Introduction
Henoch-Sch¨
onlein purpura (HSP) is a systematic vasculitis
presenting primarily in children, but less so in adults, oen
resulting in IgA-associated vasculitis in skin and IgA nephri-
tis [, ]. HSP can also present with arthritis, gastrointestinal
bleeding, and orchitis with symptoms of testicular pain and
swelling in up to % of aected boys clinically [–],
but there has been no pathologically proven IgA-associated
vasculitis of the testicles documented even in patients with
testicular pain. In addition, IgA-associated orchitis has not
been previously described in adults. We report an unusual
case in a -year-old man who developed IgA-associated
vasculitis involving the skin, kidneys, and bilateral spermatic
cords resulting in bilateral testicular infarction. is is the
rst report of histologically proven IgA-associated orchitis in
the literature.
2. Case Presentation
A -year-old male with uncontrolled diabetes (type II)
presented to the hospital with severe lower extremity and
scrotal edema, associated with pain, and extremity rash. e
rash began weeks prior to presentation and involved his
lower abdomen, bilateral lower extremities, and scrotum. He
reported intermittent painful edema of his legs and scrotum
for the past year which had been attributed to neuropathic
pain related to uncontrolled diabetes and chronic venous
stasis. Patient also reported fatigue, malaise, -pound weight
loss over the past one year, intermittent bloody bowel move-
ments,anddysuriabutdeniedanyfevers,chills,hematuria,
history of sexually transmitted infections, HIV, or malig-
nancy.
Twoweekspriortopresentationatourhospital,the
patient had presented to an outside hospital with syncope and
Hindawi
Case Reports in Nephrology
Volume 2017, Article ID 9437965, 5 pages
https://doi.org/10.1155/2017/9437965
Case Reports in Nephrology
was found to be hypoglycemic. Biopsy of the rash from his
calf was positive for leukocytoclastic vasculitis. Autoimmune
workup was negative except for elevated C-reactive protein
(CRP). Bilateral lower extremity Doppler study was negative
for thromboembolism. Patient was treated with Vancomycin
followed by clindamycin for cellulitis. He le against medical
advice before presenting to our hospital.
e patient’s past medical history was positive for uncon-
trolled diabetes type II with peripheral neuropathy, periph-
eral vascular disease with chronic lower extremity ulcers,
chronic obstructive pulmonary disease, and opioid depen-
dence. His past surgeries included amputation of a digit on
his right hand due to osteomyelitis with gangrene and lumbar
spinal fusion. Family history was positive for breast cancer
in his sister. ere was no family history of autoimmune
disease. He is a current smoker with a -pack-year history
and denied any current alcohol or drug use. His medications
included basal-bolus insulin, glipizide mg twice daily,
furosemide mg twice daily, pregabalin, and methadone
maintenance.
At presentation, the patient was found to be afebrile
with blood pressure of / mmHg, heart rate of /per
minute, respiratory rate of /per minute, SpO of %, and
BMI of .. His laboratory indices are presented in Table .
e patient appeared to be cachectic with peripheral wasting.
Exam revealed tachycardia with regular rhythm and no mur-
murs. Lung exam revealed wheezes bilaterally. His abdomen
was distended and tender to palpation. ere was tender
scrotal edema as well as severe pitting edema of his lower
extremities. He had a diuse purpuric rash over his lower
extremities, genitalia, and abdomen. Smaller petechiae were
found on his hands and arms. He also had multiple healing
lesions on his legs, a chronic healing ulcer under the le heel,
andalargeulcerwithescharwithoutdrainageorodoronthe
right lower leg.
Records from his previous admission showed elevated
CRP serology, and autoimmune work was negative. A skin
biopsy of the lower extremity rash done at an outside hospital
was positive for leukocytoclastic vasculitis.
His chest X-ray was negative and ECG was unremarkable.
Scrotal ultrasound showed bilateral wall edema with inguinal
lymphadenopathy. CT of the abdomen and pelvis also showed
anasarca with bilateral inguinal and para-aortic and external
iliac lymphadenopathy. CT and ultrasound of the kidneys
were unremarkable. Lower extremity Doppler was negative
for deep vein thrombosis.
Repeat rheumatological workup revealed positive ANA
with a titer of : and negative anti-dsDNA, Smith, RNP,
Sjogren SSA, and SSB. C and C levels were normal. Serum
immunoglobulins revealed elevated IgA level at mg/dL
with normal IgG and IgM. Serum protein electrophoresis
showed elevated kappa and lambda light chains and low
albumin with elevated alpha and beta globulins, suggestive
of active inammation. Blood, urine, and stool cultures were
negative. e patient tested positive for C. dicile stool
antigen. EGD and colonoscopy were performed which were
negative for malignancy and hemolytic workup was negative.
Patient was initially started on IV Vancomycin for sepsis,
scrotal elevation, glucose control with basal-bolus insulin,
andlocalwoundmanagement.Vancomycinwasheldupon
negative cultures. Patient was started on intravenous solume-
drol mg every hours for vasculitis. Pain control with
pregabalin, patient controlled analgesia (PCA) pump, and
total parenteral nutrition (TPN) were initiated.
e patient’s purpuric rash improved signicantly and
rapidly with intravenous solumedrol, but his scrotal pain and
edema persisted and patient developed painful penile ulcer.
A repeat skin biopsy was performed from his le thigh which
showed leukocytoclastic vasculitis. Immunouorescence was
positive for IgA, IgM, and C in the vessel wall of the super-
cial dermis, consistent with IgA-associated leukocytoclastic
vasculitis (Figures (a) and (b)).
His serum creatinine levels were at . to .mg/dL, but
his urine analysis revealed + blood and -+ protein, and the
protein/creatinine ratio was .. A subsequent -hour urine
protein was mg/ hours. He was evaluated by nephrol-
ogist and a renal biopsy was performed. Light microscopy
revealed two cores of renal tissue. Eleven glomeruli were
identied without evidence of diuse proliferation, crescents,
and global or segmental sclerosis. Many of the glomeruli
showed an increase in mesangial cellularity with focal neutro-
philic inltration as well as brinoid necrosis. e glomerular
basement membrane showed no signicant microscopic
abnormality (Figure (c)). Masson trichrome stain revealed
minimaltomildinterstitialbrosis.ebloodvesselswere
mildly thickened without vasculitis or thrombi. Immunou-
orescence study showed + positive granular IgA staining
in the mesangial and peripheral loop of the glomeruli
(Figure (d)). ere was mesangial and peripheral granular
staining for IgM at +, C at +, kappa at +, and lambda at
+, while IgG and Cq stained negatively in the glomeruli.
Ultrastructurally, there was focal eacement of foot pro-
cesses. e basement membranes were slightly thickened.
Scattered immune complex deposits were identied in the
mesangial areas but not in subendothelial spaces or subep-
ithelial areas. e overall ndings supported a diagnosis of
IgA glomerulonephritis. Because there were no history of
staphylococcal infection and no diuse proliferative pattern
in the glomeruli, with no “humps” identied at subepithelial
spaces, a potential dierential diagnosis of IgA dominant
postinfectious glomerulonephritis was excluded [, ].
Repeat ultrasound of the scrotum with Doppler was done
due to persistent scrotal pain, which revealed hypoperfu-
sion of the le testicle without evidence of torsion. Le
orchiectomy was performed weeks aer admission. Grossly
the cut surface of the testicle revealed the entire testicu-
lar parenchyma to be brown-red, partially liqueed, and
necrotic. In the area of the epididymis and rete testes there
was yellow-green so discoloration. e microscopy sections
revealed testicular infarction with testicular/paratesticular
abscess that involved the epididymis and spermatic cord.
Pain in his right testicle persisted, and, four days later,
orchiectomy of the right testicle was also performed. Both
gross and microscopic ndings in the right testicle were
similar to those in the le orchiectomy specimen. Testicular
abscess was identied (Figure (e)). Spermatic cord margins
from bilateral orchiectomy specimens were further analyzed
to show diuse vasculitis in small arteries with scattered
Case Reports in Nephrology
T : Patient’s laboratory values upon admission.
Component Value Ref range & units
Complete blood count with dierential
WBC . .–. bil/L
RBC . (L) .–. tril/L
Hemoglobin . (L) .–. g/dL
Hematocrit . (L) .–.%
MCV – fL
MCH (L) – pg
RDW CV (H) –%
Platelets – bil/L
Neutrophils . .–. bil/L
Lymphocytes . (L) .–. bil/L
Monocytes . .–. bil/L
Immature granulocytes . (H) .–. bil/L
Urine analysis
Color Yellow
Clarity Clear
Glucose + Negative
Protein + Negative
Blood trace Negative
Ketones negative Negative
RBCs –/hpf –/hpf
WBCs –/hpf –/hpf
Casts, hyaline –/lpf –/lpf
Urine protein to creatinine ratio . –.
Blood chemistries
Sodium (L) – mmol/L
Potassium . (H) .–. mmol/L
Chloride (L) – mmol/L
Carbon dioxide (CO) – mmol/L
Anion gap –
Glucose (HH) – mg/dL
Blood urea nitrogen (BUN) (H) – mg/dL
Creatinine . .–. mg/dL
Calcium . (L) .–. mg/dL
Protein total . (L) .–. g/dL
Albumin . (L) .–. g/dL
Globulin . .–. g/dL
Albumin/globulin ratio .
Alkaline phosphatase (ALP) – U/L
Aspartate aminotransferase (AST) (H) – U/L
Alanine aminotransferase (ALT) – U/L
Bilirubin total . .–. mg/dL
Bilirubin direct . –. mg/dL
GFR non-African American > mL/min/. m
GFR African American > mL/min/. m
ESR (H) – mm/hr
Lactic acid . .–. mmol/ L
Lipase – U/L
Beta hydroxybutyrate . .–. mmol/L
BNP – pg/mL
Case Reports in Nephrology
(a) Skin biopsy (b) IgA positive in vessels and epithelium
(c) Renal biopsy (d) IgA positive in glomerulus
(e) Spermatic cord margin (f) Vasculitis in spermatic cord
(g) Organizing thrombosis (h) Positive IgA staining in small arteries
F : Evaluation of skin biopsy, renal biopsy, and orchiectomy specimens from the -year-old man. (a) Hematoxylin and eosin stained
section revealed surface ulceration in the skin. (b) IgA immunouorescence was positive in epidermis and vessels of dermis. (c) Hematoxylin
and eosin stained section revealed mesangial expansion with focal neutrophil aggregation in the glomerulus. (d) IgA immunouorescence
was positive mainly in the mesangium and some along the glomerular capillary loops. (magnications × in (a)–(d)). (e) A low power view
(×) revealed the unremarkable vas deferens at the right lower corner and necrosis and abscess in the testicular parenchyma at the le upper
corner. (f) Vasculitis was seen in multiple small arteries of spermatic cord at medium power view (×). (g) High power view (×) revealed
organizing thrombus in a small artery causing nearly total occlusion of the vessel in the spermatic cord. (h) IgA immunouorescence (×)
was positive (green granular staining) at the endothelium of multiple inamed small arteries. Hematoxylin and eosin stains were performed
in (e)–(g).
Case Reports in Nephrology
organizing thrombi in some (Figures (f) and (g)). Paran
embedded sections of bilateral spermatic cords were digested
and stained for IgA by direct immunouorescent method
(as previously reported) []. e immunouorescent section
revealed strongly positive IgA staining along the endothelium
of inamed small arteries (Figure (h)), conrming the IgA
vasculitisofthespermaticcordsasthecauseofthetesticular
ischemic infarction. His scrotal edema gradually improved
with wound care and nutritional support. In addition to
steroids, dapsone was started per Rheumatology.
His hospital course was complicated by persistent diar-
rhea, drug-seeking behavior, bacteremia, persistent hyper-
glycemia, and ischemia of multiple digits requiring amputa-
tions. His rash did not recur while on the steroids, and he
was discharged to a long-term acute care facility with close
follow-up.
3. Discussion
Scrotal manifestations of HSP are overwhelmingly described
in pediatric populations, based solely on clinical evaluations
[–]. No histologically proven cases of IgA-associated orchi-
tis have been reported in any pediatric study. Furthermore
scrotal disease due to IgA vasculitis is easily missed or
misdiagnosed due to low level of suspicion and its propensity
to manifest later in the course of disease, sometimes aer
initial signs and symptoms of HSP have resolved. HSP in
adultsisusuallyassociatedwithworseoutcomescompared
to children [, ]. It is unclear whether IgA-associated orchitis
in adults would have worse outcomes compared to children.
In this patient the involvement was severe leading to tissue
necrosis and required bilateral orchiectomies despite high
dose steroid therapy. Due to initial lack of awareness the
etiology for the patient’s scrotum swelling had remained
uncertain and was felt to be part of generalized edema and
nephrotic syndrome. Patient developed Klebsiella bacteremia
and the source of this bacteremia was felt to be from extremity
ulcers and so tissue infections. Later it was realized only aer
orchiectomy that the source of bacteremia was most likely
from testicular infarction.
During the examination of the spermatic cords, vasculitis,
characterized by edematous changes in vessel walls and
inltration of inammatory cells, was seen in small arteries
with occasional organizing thrombi. In addition, we repro-
cessed the paran embedded tissue for immunouorescent
staining of IgA, and IgA positivity was only present along
endothelium of inamed vessels, conrming that the IgA-
associated vasculitis was the etiology causing thrombotic
obstruction in the vessels with subsequent ischemic pain
in the scrotum, testicular infarction, abscess formation, and
possible overgrowth of bacteria.
Insummary,this-year-oldmalepatientdevelopeda
systemic IgA vasculitis involving the skin of the extremi-
ties, kidneys, and bilateral testicles with the most serious
complication of testicular infarctions and subsequent abscess
formation. is is the rst report of histologically proven
IgA-associated orchitis in the literature. is case illustrates
theneedforlowthresholdofsuspicionforvasculiticscrotal
involvement when caring for adult patients with HSP who
develop scrotal pain and swelling. Scrotal involvement may
be more prevalent than reported. Genital examination is
oen not performed; also scrotal pain may be mislabeled,
which may lead to diagnosis being missed. Genital examina-
tion should be routinely carried out in these patients for early
detection of scrotal involvement. Scrotal swelling, pain, and
tenderness should prompt immediate diagnostic evaluation
and urology consultation where needed.
Conflicts of Interest
e authors declare that there are no conicts of interest
regarding the publication of this article.
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