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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 88-90

Immunoglobulin a vasculitis after a not so innocuous wasp bite


1 Department of Pediatrics, Army Hospital (R&R), New Delhi, India
2 Department of Pathology, Army Hospital (R&R), New Delhi, India

Date of Submission07-Feb-2022
Date of Decision31-Mar-2022
Date of Acceptance26-Apr-2022
Date of Web Publication30-May-2022

Correspondence Address:
Dr. Suprita Kalra
Department of Pediatrics, Army Hospital (R&R), New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ipcares.ipcares_41_22

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  Abstract 

Background: Immunoglobulin A vasculitis (IgAV), previously known as Henoch–Schonlein purpura (HSP), is the most common vasculitis in children. Previous studies have identified various triggers of IgAV, with infections being the most common. We present herein a 9-year-old girl who developed IgAV with nephritis following a wasp sting. Clinical Description: A 9-year-old girl presented to us with a history of wasp sting 7 days ago, followed by the appearance of reddish, raised rashes over the back of her lower limbs, which later spread all over the body. She also developed edema over the face, abdomen, and lower limbs along with pain abdomen. On examination, she was afebrile, was normotensive, and had periorbital edema and bilateral pedal edema with multiple discrete palpable, nonblanching purpura predominantly over the extensor surfaces of the lower and upper extremities and the trunk. Abdominal examination revealed no tenderness. Complete blood counts, blood urea, serum creatinine, and liver function tests were normal. Urinalysis showed microscopic hematuria and nephrotic range proteinuria. Skin biopsy of the lesions showed evidence of IgA vasculitis. Renal biopsy was suggestive of HSP nephritis class 3. Management and Outcome: She was managed with oral corticosteroids, mycophenolate mofetil, and enalapril and had remission of proteinuria. The renal function tests and blood pressure continue to be normal. Conclusion: Few case reports exist of IgAV precipitated by insect bites; however, we could not find any previous reports of IgAV with nephritis following a wasp sting in children. This report adds to existing knowledge regarding precipitating factors for IgAV in children.

Keywords: Henoch–Schonlein purpura, immunoglobulin A vasculitis, nephritis, wasp sting


How to cite this article:
Sharma A, Singh V, Kalra S. Immunoglobulin a vasculitis after a not so innocuous wasp bite. Indian Pediatr Case Rep 2022;2:88-90

How to cite this URL:
Sharma A, Singh V, Kalra S. Immunoglobulin a vasculitis after a not so innocuous wasp bite. Indian Pediatr Case Rep [serial online] 2022 [cited 2022 Jul 2];2:88-90. Available from: http://www.ipcares.org/text.asp?2022/2/2/88/346256

Immunoglobulin A vasculitis (IgAV), previously known as Henoch–Schonlein purpura, is the most common vasculitis observed in the pediatric age group. It is a small-vessel vasculitis that presents with characteristic skin lesions in the form of erythematous palpable purpura. In a subset of children, the complete triad of the disease that includes gastrointestinal, musculoskeletal, and renal manifestations is seen.[1],[2] Various triggers of IgAV have been identified, with infections being the most common precipitating factor.[3] There are published case reports of IgAV occurring following insect bites (fire ant) and bee stings.[4],[5]

Here, we present a girl who developed IgAV with nephritis following a wasp sting that was managed with immunosuppressive therapy. To the best of our knowledge, this is the first reported case since an exhaustive scientific literature search was unable to identify any previous case.


  Clinical Description Top


A 9-year-old girl presented with a history of being stung by a wasp 7 days earlier. This was immediately followed by the appearance of generalized pruritic, reddish, raised rashes that subsided within 24 h after treatment with oral anti-inflammatory and antihistaminic medication. Three days afterward, reddish, raised, painless, and nonpruritic skin lesions and rashes were noted. These initially erupted over the back of her legs and later spread all over her body. The size varied, with a diameter ranging from 1 to 1.5 cm. Within the next 24–48 h, the child developed swelling over her face (mainly around her eyes) that progressed to involve her abdomen and legs. There was no history of joint swelling or pain, abdominal pain, reduced urine output, or passage of reddish or cola-colored urine.

There was no preceding history of cold, cough, or fever. There was no past history suggestive of allergies such as wheezing, redness and/or excessive watering of the eyes, recurrent bouts of sneezing or eruption of itchy, red rashes following the ingestion of any food items or other triggers. She was the fourth born of a nonconsanguineous marriage with no significant family history. She was immunized, and her diet and academic performance were appropriate for her age.

She was conscious and alert. She was afebrile. Her pulse rate was 84/min and regular. She had a blood pressure (BP) of 98/72 mmHg (between 50th and 95th centiles for age). There was no respiratory distress. The generalized physical examination revealed periorbital and bilateral pedal edema. Multiple, discrete, palpable, nonblanching purpura were present predominantly on the extensor surfaces of the lower extremities, both arms, and trunk. There was no pallor, icterus, or significant lymphadenopathy. The joint examination was normal. There was no tenderness or organomegaly on abdominal examination, but shifting dullness was present suggestive of ascites. The examination of other systems was normal. Based on the combination of purpura predominantly involving the extensor surfaces and edema, a possibility of IgAV was considered. The edema suggested possible renal involvement, but there was no oliguria, hematuria, or hypertension. The likelihood of an adverse drug reaction following paracetamol and antihistaminic drugs that had been taken after the wasp sting was less.


  Management and Outcome Top


First-line investigations hemogram (hemoglobin 12.1 g/dL, total leukocyte count 9300/mm3, and platelet count 3.3 lac/mm3), kidney function test (urea 19 mg/dl and creatinine 0.42 mg/dl), and liver function test (specifically serum albumin 2.3 g/dl) were normal. Urinalysis showed numerous red blood cells (RBCs) per high-power field (HPF) <20% of which were dysmorphic and proteinuria (2+ to 3+ on dipstick). The urine protein–creatinine ratio was 3.5 (elevated), and 24-h urine protein excretion was 4.9 g/day (increased). Serum C3 was 98 mg/dl (normal 90–110 mg/dl), and antinuclear antibodies and dsDNA were negative. Abdominal ultrasound revealed normal-sized kidneys (right 8.4 cm and left 8.6 cm) with mild attenuation of corticomedullary differentiation. The biopsy of the skin lesions confirmed small-vessel vasculitis and IgA was present 3+ in the dermal vessels, consistent with IgAV [Figure 1]. A renal biopsy was performed in view of the proteinuria being in the nephrotic range. This showed 22 glomeruli with variable degrees of mesangial expansion, mesangial hypercellularity, endocapillary proliferation, and tuft necrosis. There was no evidence of segmental sclerosis or crescents, and the blood vessels and tubulointerstitial compartment were unremarkable. This corresponded to the International Study of Kidney Disease in Children (ISKDC) grade III IgA nephropathy [Figure 2].[6] Direct immunofluorescence showed IgA 3+ with the absence of C3, C1q, immunoglobulin G (IgG), and IgM. The final diagnosis was IgAV with class III nephritis.
Figure 1: Photomicrographs of histopathological and DIF findings of skin biopsy. (a) Photomicrograph of skin biopsy shows epidermis and dermis (H and E, ×10), (b) High-power view shows moderate perivascular mixed inflammatory infiltrate around dermal capillaries (black arrow), evidence of vasculitis noted in the form of infiltration into the vessel wall, extravasation of RBCs, and endothelial swelling. (c) DIF for IgA showed 3+ granular positivity in the dermal capillary wall (blue arrow) (FITC, ×40). (d) DIF for IgA showed 1+ granular positivity in the dermal capillary wall (blue arrow) (FITC, ×40). DIF: Direct immunofluorescence

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Figure 2: Photomicrographs of histopathological and DIF findings of renal biopsy. (a and b) Photomicrograph of renal biopsy with the glomeruli showing variable degrees of mesangial expansion and mesangial hypercellularity (H and E, ×10 and ×20). (c and d) PAS stain sections show highlight variable degrees of mesangial expansion and mesangial hypercellularity (PAS stain, ×20 and ×40). (e) DIF for IgA shows 3 + granular mesangial and glomerular capillary wall positivity (FITC, ×40). (f) DIF for IgG showed 1+ granular mesangial and glomerular capillary wall positivity (FITC, ×40). DIF: Direct immunofluorescence, IgG: Immunoglobulin G, PAS: Periodic acid–Schiff. FITC: Fluorescein isothiocyanate–dextran

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She was managed with oral corticosteroids (2 mg/kg/day for 4 weeks followed by tapering doses), mycophenolate mofetil (MMF) (800 mg/m2), and enalapril (as per the European consensus guidelines).[7],[8] The rash and edema resolved in 2 weeks. Monthly follow-up visits included monitoring of BP (remained normotensive), renal function tests (within normal limits), and 24-h urine protein (reduced to 800 mg/day by 2 months and 350 mg/day by 6 months' follow-up). Microscopic hematuria is persisting (8 RBCs/HPF) at the last follow-up.


  Discussion Top


The incidence of IgAV is 3–27 cases per 100,000 children. IgAV with nephritis is seen in approximately one-third of children. Most affected children exhibit gross or microscopic hematuria with no or only mild proteinuria. Only about 2% have been reported to have proteinuria in the nephrotic range and other features of severe glomerulonephritis.[2],[7] In this case, IgAV with nephritis was confirmed by nephrotic range proteinuria and ISKDC grade III IgA nephropathy on kidney biopsy. Since recent guidelines recommend early immunosuppressive therapy in cases with moderate-to-severe proteinuria,[9] we treated the child with corticosteroids and MMF, following which the skin lesions and edema resolved and proteinuria reduced considerably.

There are many similarities as well as differences between IgA vasculitis (IgAV) and IgA nephropathy (IgAN). IgAV and IgAN could represent different extremities of a continuous spectrum of the same disease, as evidenced by episodes of IgAV as well as IgAN among siblings, parents in the same patients at two periods of life, and, more recently, recurrences in kidney allograft.[10] As a result, IgAV could well be the systemic form of the IgAN. The differences between both entities could be primarily the clinical presentation, with IgA nephropathy usually affecting older children and young adults and typically involving only the kidneys, whereas IgAV affects mostly children and involves the skin and connective tissues, gastrointestinal tract, joints, as well as the kidneys.

The four-hit theory elucidates the pathogenesis of IgAV with nephritis. This includes increased production of galactose-deficient IgA1 and IgG autoantibodies that recognize galactose-deficient IgA1, form immune complexes, and get deposited in the glomeruli. The first two hits apparently occur when there is exposure to infectious agents or other antigens. Thus, these may have a role in the disease precipitation, especially due to dysregulated mucosal immunity.[8] The most common triggering microorganism is Group A Streptococcus, but other bacteria, viruses, and protozoa have been incriminated.[3] Our patient developed typical features of IgAV within 3 days of the wasp sting. In our literature search, we found a case report of IgAV having being precipitated by a bee sting. The essential difference between a bee sting and a wasp sting is that the former can sting only once as it loses its stinger and the amount of bee venom injected is 50 μg, whereas the wasp can sting multiple times, each time introducing 2–15 μg of wasp venom. Bee venom contains melittin, while wasp venom contains acetylcholine and serotonin. Both contain substances that trigger histamine release and hyaluronidase that helps the venom to spread within the tissues.

To conclude, genetic factors, disrupted mucosal immunity, and immune complexes with abnormal IgA or IgA antibodies are essential in the pathogenesis of IgAV. Insect bites and bee stings have been known to trigger IgAV with nephritis in children. Given the similarity between bee and wasp venom, whether the wasp sting represents an association or causation is a question that can only be answered by more reports of similar cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Hastings MC, Rizk DV, Kiryluk K, et al. IgA vasculitis with nephritis: Update of pathogenesis with clinical implications. Pediatr Nephrol 2022;37:719-33.  Back to cited text no. 1
    
2.
Aalberse J, Dolman K, Ramnath G, et al. Henoch schonlein purpura in children: an epidemiological study among Dutch paediatricians on incidence and diagnostic criteria. Ann Rheum Dis 2007;66:1648-50.  Back to cited text no. 2
    
3.
Wang JJ, Xu Y, Liu FF, et al. Association of the infectious triggers with childhood Henoch-Schonlein purpura in Anhui province, China. J Infect Public Health 2020;13:110-7.  Back to cited text no. 3
    
4.
Mazumder S, Ma M, Champigny M, et al. A case of adult-onset Henoch-Schönlein purpura triggered by fire ants. Cureus. 2020;12:e7341.  Back to cited text no. 4
    
5.
Burke DM, Jellinek HL. Nearly fatal case of Schoenlein-Henoch syndrome following insect bite. AMA Am J Dis Child 1954;88:772-4.  Back to cited text no. 5
    
6.
Koskela M, Ylinen E, Ukonmaanaho EM, et al. The ISKDC classification and a new semiquantitative classification for predicting outcomes of Henoch-Schönlein purpura nephritis. Pediatr Nephrol 2017;32:1201-9.  Back to cited text no. 6
    
7.
Ozen S, Marks SD, Brogan P, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative. Rheumatology (Oxford) 2019;58:1607-16.  Back to cited text no. 7
    
8.
Luciana B, Ilaria C, Isabella C, et al. Epidemiological and clinical aspects of immunoglobulin A vasculitis in childhood: a retrospective cohort study. Ital J Pediatr 2021;47:237.  Back to cited text no. 8
    
9.
Lau KK, Suzuki H, Novak J, et al. Pathogenesis of Henoch-Schönlein purpura nephritis. Pediatr Nephrol. 2010;25:19-26.  Back to cited text no. 9
    
10.
Pillebout E. IgA Vasculitis and IgA nephropathy: Same disease? J Clin Med 2021;10:2310.  Back to cited text no. 10
    


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