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Clinical Infection & Immunity

Case Report

Volume 1, Number 1, September 2016, pages 19-22

Adult Onset Henoch-Schonlein Purpura Following Varicella-Zoster Virus Infection (Chickenpox)

Henoch-Schonlein purpura (HSP) is an immunoglobulin-A (IgA) mediated small vessel vasculitis that predominantly affects children but is also seen in adults [1]. Approximately 75% of patients affected by HSP are aged 2 - 11 years and HSP is infrequent in adults over the age of 20 [2, 3]. Unfortunately, adult HSP represents a more severe clinical syndrome with worse outcomes compared to children. Adult HSP is characterized by a lower frequency of antecedent infection, abdominal pain and fever, and a higher frequency of joint symptoms and renal involvement [4]. HSP following varicella-zoster virus (VZV) infection has been reported in children [5-9]. Herein we describe a case of adult onset HSP with an antecedent VZV infection, which to the best of our knowledge has not been reported in the literature.

A 35-year-old man presented with low grade fever and centripetal pleomorphic vesicular rashes over the trunk and face appearing in successive crops. He was diagnosed chickenpox. After 7 days of onset, he developed acute onset bilaterally symmetrical inflammatory arthritis of the knees and ankles, associated with widespread erythematous non-pruritic rashes over the lower extremities. There were no symptoms suggestive of cardio-respiratory, gastrointestinal (GI) or nephro-urological systems. Clinical evaluation revealed bilateral knee and ankle arthritis (Fig. 1) with palpable purpuric vasculitic lesions on the lower extremities (Fig. 1). He also had hyperpigmented macules and crusted papules typical for VZV infection (Fig. 2). Rest of the general and systemic examination was unremarkable. Laboratory analysis showed raised C-reactive protein (CRP) levels (30.4 mg/L) with normal hematological and biochemical parameters. Urinalysis revealed sub-nephrotic range proteinuria (1,680 mg/day) with bland urinary sediments. Serum ANA, ANCA, IgA and complement (C3, C4) levels were within normal limits. Markers for viral hepatitis were negative. There was evidence of leucocytoclastic vasculitis (LCV) on skin biopsy (Fig. 3) with absence of IgA deposits on direct immunofluorescence (DIF).

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Figure 1. Vasculitic lesions over lower limbs and ankle arthritis.

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Figure 2. Healed chickenpox lesions over the trunk.

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Figure 3. Skin biopsy showing leucocytoclastic vasculitis (H&E, × 200).

The above clinical profile along with evidence of LCV and proteinuria supported a diagnosis of adult onset HSP in accordance with the European League Against Rheumatism (EULAR) criteria (Table 1) [10]. He responded well to symptomatic therapy with non-steroidal anti-inflammatory agents and his proteinuria also resolved in 9 days.

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Table 1. The EULAR Criteria for Diagnosis of HSP [10]

HSP is the most frequent vasculitis in children, whereas adult onset HSP has an incidence of 3.4 - 14.3 cases per million [1-4, 11]. The most common presenting features are purpuric rash (96%), arthritis (61%), GI disease (48%) and renal disease (32%) [12]. The clinical profile of adult onset HSP varies from that in children [4, 11], as described in Table 2. In the latter, the disorder is often self-limiting, while a more complicated course has been described in adults, including a high incidence of renal insufficiency and joint involvement. All these features were present in our case except for GI involvement.

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Table 2. Important Differences Between Childhood and Adult HSP [4]

Typically adult HSP is commoner in males and may follow an infectious illness [11, 13]. The etiology of HSP remains unknown, but various antigenic stimuli have been implicated in triggering this pathology, including infectious agents, allergens, malignancies or autoimmune diseases [11, 13]. An antecedent upper respiratory tract infection (URTI) is reported as an exogenous stimuli in 40% of adult onset HSP cases. The various agents include group A streptococci, VZV, hepatitis B, Epstein-Barr virus, parvovirus B19, Mycoplasma, Campylobacter, and Yersinia. These are less commonly seen in adults [13]. In the literature till 2005, only five cases with HSP in relation with VZV infection were reported in children, four following VZV [5-8] and one during VZV infection [9]. To the best of our knowledge, no case of adult onset HSP associated with VZV has been ever documented in the literature.

The diagnostic criteria for HSP have been proposed by the American College of Rheumatology [14], followed by the EULAR [10]. Since the latter does not consider age limit as one of the mandatory criteria, it was applied in achieving a diagnosis of HSP in our case. The diagnosis is usually based on clinical presentation with tissue biopsy showing evidence of LCV associated with IgA deposition [1, 2]. Skin biopsy in the present case also showed evidence of LCV, further supporting the diagnosis of HSP. Skin biopsy should be obtained from the lesion less than 24 h old which typically shows classical LCV in post-capillary venule with IgA deposition [15]. As the lesions get older, the IgA deposits get degraded and cleared [15].

A search for the literature for adult onset HSP cases using PubMed database was done [3, 16, 17]. The salient clinical and histological features of selected cases along with our patient are shown in Table 3. There was no triggering event in any except for two cases with history of URTI, while our patient had an antecedent VZV infection. It was noted that there was no histological evidence of IgA deposits in two of these cases. Similarly, our patient also did not reveal IgA deposits on DIF, which may be attributable to a delayed presentation [15].

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Table 3. Selected Reports of HSP in Adult Patients [3, 16, 17]

Adult onset HSP is a rare entity with few cases documented in the western literature [3, 16] and rarely reported from the Indian subcontinent [17]. Here, we have described a case of adult onset HSP following VZV infection (chickenpox). In adults, this complication of chickenpox has not been previously reported in the literature. Secondly, with the widespread availability of varicella vaccine, the prevalence of chickenpox in children has significantly decreased, while more cases are now being reported in the adult population [18]. Hence the possibility of HSP as a complication of VZV infection should be considered even in adult patients with a clinical profile similar to that of the case discussed.

Conflicts of Interest

None.

Funding

None.

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