Complex regional pain syndrome associated with henoch-schonlein purpura: A rare occurrence

Sanjay Kumar; Sandeep Khuba; Shikha Awal; Sujeet Gautam; Anil Agarwal

doi:10.4103/ijpn.ijpn_122_20

CASE REPORT

Year : 2022 | Volume : 36 | Issue : 1 | Page : 59-62

Complex regional pain syndrome associated with henoch-schonlein purpura: A rare occurrence

Sanjay Kumar¹, Sandeep Khuba¹, Shikha Awal², Sujeet Gautam¹, Anil Agarwal¹
¹ Department of Anaesthesiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
² Department of Anaesthesiology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

Date of Submission	27-Aug-2020
Date of Decision	16-Feb-2021
Date of Acceptance	20-Feb-2021
Date of Web Publication	25-Apr-2022

Correspondence Address:
Dr. Sandeep Khuba
Department of Anaesthesiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Rai Bareilly Road, Lucknow - 226 014, Uttar Pradesh
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijpn.ijpn_122_20

Abstract

Introduction: Henochschonlein purpura (HSP) is an acute, systemic, immune complex-mediated, leukocytoclastic vasculitis, most commonly affecting children. It is a small-vessel vasculitis which is characterized by a tetrad of palpable purpura (without thrombocytopenia), abdominal pain, arthritis, and renal impairment. Although most commonly affecting the skin, joints, gastrointestinal tract, and kidneys, other organs may also be affected. Association of HSP with complex regional pain syndrome (CRPS) has not been reported in the literature. In the present case report, we are describing the successful management of CRPS of the upper limb in a diagnosed case of HSP. Case Presentation: A 14-year-old female patient, who was a diagnosed case of HSP, presented with features characteristic of CRPS in the right hand and forearm, based on the Budapest Diagnostic Criteria. Bone scintigraphy showed increased tracer uptake in joints of the right hand, also suggestive of CRPS. The pain was severe visual analog scale-90/100 and not controlled with conservative management. Patient was administered a series of 3 stellate ganglion minimally invasive pain and spine interventions (MIPSI) over the course of 2 weeks, which led to the resolution of her symptoms. Conclusion: HSP is a small-vessel vasculitis and may lead to peripheral nerve vasculitis. As reported in other cases, small-vessel vasculitis has led to the development of CRPS and thus may influence the development of CRPS in patients with HSP. CRPS as a complication of HSP has not been reported in the literature to date. Thus in a patient with HSP, CRPS could present as a rare complication and early intervention with the MIPSI of the affected region may lead to resolution of symptoms.

Keywords: Chronic pain, complex regional pain syndrome, henoch-schonlein purpura

How to cite this article:
Kumar S, Khuba S, Awal S, Gautam S, Agarwal A. Complex regional pain syndrome associated with henoch-schonlein purpura: A rare occurrence. Indian J Pain 2022;36:59-62

How to cite this URL:
Kumar S, Khuba S, Awal S, Gautam S, Agarwal A. Complex regional pain syndrome associated with henoch-schonlein purpura: A rare occurrence. Indian J Pain [serial online] 2022 [cited 2023 Mar 31];36:59-62. Available from: https://www.indianjpain.org/text.asp?2022/36/1/59/343822

Introduction

Henoch-Schonlein Purpura (HSP) is an acute, systemic, immune complex-mediated, small vessel, leukocytoclastic vasculitis, most commonly affecting children characterized by a tetrad of palpable purpura (without thrombocytopenia), abdominal pain, arthritis and renal impairment. HSP may involve either the central or peripheral nervous system or both and may present as mononeuropathy, polyneuropathy, mono neuritis multiplex or brachial plexopathy. [1] Complex Regional Pain Syndrome (CRPS) is regional pain syndrome characterized by abnormal sensory, motor, sudomotor, vasomotor oedema, and/or trophic changes. [2] The association of HSP with CRPS which has not been reported in the literature. The present case report describes successful management of CRPS of the upper limb in a diagnosed case of HSP.

Case Report

A 14-year-old female patient (body weight 52 kg) referred from the Department of Hematology presented to our pain clinic with complaints of severe pain in the right hand and wrist for 1 month. It was an acute onset with the beginning of the winter season and gradually increased in severity. The pain was severe with visual analog scale (VAS)-90/100, burning in character, constantly present and aggravated even with touch and mild pressure over the hand. It was relieved slightly with the hand kept in nondependent position, but there was no relief with medications. The pain was associated with swelling, redness, and weakness of the right hand and wrist. There was no history of numbness or tingling. There was no history of trauma. On local examination, there was swelling and redness over the right hand, wrist, and distal part of the forearm. No trophic changes were noted in skin, hair, or nails. There were no changes in the sweating pattern as compared to the left hand. Local temperature was raised. Brush allodynia was present. No sensory deficit or paraesthesia could be elicited. Motor weakness was documented, with motor power for right wrist flexion-2/5, wrist extension-2/5, thumb adduction-3/5, thumb abduction-3/5, fingers flexion-3/5, and fingers extension-2/5. The patient was diagnosed as having complex regional pain syndrome (CRPS) based on the Budapest Diagnostic criteria.^[1]

There was a history of a similar episode 1 year back. At that time, the patient underwent Triple Phase Scintigraphy [Figure 1], which showed increased tracer uptake in multiple metacarpophalangeal joints, suggestive of CRPS. She was prescribed intravenous pamidronate 60 mg every month for 6 doses by the Department of Hematology, which leads to complete resolution of her symptoms. The patient was a diagnosed case of henochschonlein purpura (HSP) for 3 years and was undergoing treatment for gastrointestinal and renal vasculitis. She was being prescribed prednisolone, cyclophosphamide, and azathioprine for the same. There were no other comorbidities.

Figure 1: Overall scan pattern, increased perfusion, raised blood pool activity, and significant asymmetric osteoblastic activity in the delayed phase of the bone scan are suggestive of complex regional pain syndrome

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The present episode was not relieved after taking 1-week course of naproxen 250 mg twice a day prescribed by the Department of Hematology. Thereafter, patient was referred to pain clinic. The patient was planned for right Stellate ganglion minimally invasive pain and spine interventions (MIPSI) with local anesthetic under local anesthesia, given the severity of pain. The MIPSI was performed under ultrasound guidance (USG) with high-frequency linear transducer (6–13 MHz) and 22 G, 10 cm quincke spinal needle. The patient was placed in the supine position with slight rotation of the head to the left side. The transverse process of C7 vertebrae was visualized along with the carotid artery. The 5 ml of 0.25% bupivacaine was deposited in the prevertebral fascia around longus colli muscle [Figure 2]. Thereafter, the patient was prescribed naproxen 250 mg twice a day (BD), amitriptyline 10 mg at night (HS), and pregabalin 75 mg twice a day (BD). The patient had 75% pain relief (VAS 30–40/100) with the MIPSI which remained for 3 days. Motor power improved, with an only slight decrease in swelling. Repeat MIPSI was performed using 5 ml 0.25% bupivacaine. The patient had 90% (VAS-10/100) relief for 1 week. The patient was advised gradual mobilization of all joints of the right upper limb. There was a significant improvement in motor power and the swelling and redness subsided. After 1 week, the pain started to increase again. Hence, the MIPSI was performed for the third time and 5 ml of 0.25% bupivacaine with 40 mg of methylprednisolone was given. The patient had 90% relief in her pain and complete recovery from motor weakness. The patient has been under our follow-up for 4 months now and has no pain (VAS-0/100), no motor weakness, and no swelling. She is currently taking amitriptyline 25 mg HS. The pregabalin was stopped after 3 months of the last MIPSI.

Figure 2: Stellate Ganglion minimally invasive pain and spine intervention

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Discussion

CRPS is a syndrome characterized by continuing regional pain that is disproportionate in time or degree to the usual course of pain after trauma or other lesions. The pain is disproportionate in intensity to the inciting event and is intense burning in character with associated hyperalgesia or allodynia.^[2] It is regional, not being limited to specific nerve territory or dermatome. CRPS usually involves the distal part of extremities along with abnormal sensory, motor, sudomotor, vasomotor edema, and/or trophic findings. The most common precipitating event is trauma affecting the distal part of an extremity (~75%), especially fractures, postsurgical conditions, contusions, and strain or sprain.^[2] Less common incidents are central nervous system (CNS) lesions such as spinal cord injuries and cerebrovascular accidents. Asymmetrical distal extremity pain and swelling develop in patients with CRPS 1 without an overt nerve lesion. The symptoms of CRPS 2 are similar to those of CRPS 1, the only exception being that a lesion of peripheral nerve structures and subsequent focal deficits are mandatory for the diagnosis.^[2] Diagnosis is made based on “Budapest Criteria” for CRPS which include four categories of symptoms and signs; sensory, vasomotor, sudomotor/edema, and motor/trophic. One symptom in at least 3 out of 4 categories and one sign in at least 2 out of 4 categories are required to make a diagnosis of CRPS.

Our patient was diagnosed as CRPS based on Budapest Criteria. Symptoms and signs were seen in 4 out of 4 categories. The bone scintigraphy was also suggestive of CRPS. The patient had almost complete relief following stellate ganglion MIPSI, further substantiating the sympathetically maintained nature of pain.

This patient was a diagnosed case of HSP for the past 3 years. The patient was undergoing treatment for her persistent gastrointestinal and renal vasculitis in the department of Haematology. She was prescribed prednisolone, cyclophosphamide, and azathioprine for it. She developed symptoms of CRPS in her right upper limb 1 year back which was confirmed with triple-phase scintigraphy. This episode of CRPS got relieved after 6 doses of pamidronate 60 mg with each dose given every month. After 6 months of this episode, the patient again developed symptoms of CRPS in her same limb for which she was referred to our pain clinic.

In the pathophysiology of CRPS I, trauma and immobilization of the limb and in CRPS II nerve injury plays a key role but in the present case, there was no such suggestive history apart from diagnosis of HSP for the past 3 years.

HSP is an acute, systemic, immune complex-mediated, leukocytoclastic vasculitis, most commonly affecting children.^[3] It is a small-vessel vasculitis which is characterized by a tetrad of palpable purpura (without thrombocytopenia), abdominal pain, arthritis, and renal impairment. Although most commonly affecting the skin, joints, gastrointestinal tract, and kidneys, other organs may also be affected.^[3] HSP may involve either the central or peripheral nervous system (PNS) or both. The involvement of the CNS in HSP presents as an altered level of conscious, seizures, or focal neurological deficits. Moreover, PNS involvement in HSP may present as mononeuropathy, polyneuropathy, mono neuritis multiplex, or brachial plexopathy.^[4] Many rare manifestations of HSP have been reported, such as interstitial lung disease,^[4] headache^[5]_, and seizures.^[6] However, CRPS as a complication of HSP has not been reported in the literature to date.

There is a single case series (comprising three cases) which reported the presentation of “Peripheral nerve vasculitis as CRPS”.^[7] While the patients presented with features suggestive of CRPS, the electrodiagnostic testing was suggestive of multiple mononeuropathies. Nerve biopsy confirmed vasculitis in one case and was suggestive of angiopathy in the other two cases. The author concluded that the inflammatory nerve injury seen with peripheral nerve vasculitis can result in CRPS.

HSP being a type of vasculitis may result in a similar presentation, which was the case in our patient. It is worthwhile to know that, HSP is a type of small-vessel vasculitis. Other examples of small vessel vasculitides include microscopic polyangiitis (MPA), ChurgStrauss Syndrome, Cryoglobulinemic vasculitis, Wegener's granulomatosis, and Behcet's syndrome among others.^[8] While involvement of PNS by vasculitis is a common occurrence in most small-vessel vasculitides (ChurgStrauss syndrome [65%–80%], MPA [6%–70%], Cryoglobulinemia [30%–70%], M. Behcet [20%], Cryoglobulinemia [30%–70%], Wegener's granulomatosis [14%–50%]), its involvement in HSP is very rare.^[9] This case report exemplifies one such rare instance.

The pathogenesis of neuronal damage in patients of vasculitides is suggested to be due to inflammation of the vasa nervorum, the arterial supply of the nerves, causing thrombosis and ultimately, ischemic damage of the nerves.^[10],[11] Another theory suggesting the role of auto-antibodies directly triggering axonal damage is proposed based on a finding of a strong association between severity of peripheral neuropathy and the titers of GM1 and anti-sulfatide antibodies.^[11]

In the present case, the patient was given stellate ganglion MIPSI two times with local anesthetic and third time with combined local anesthetic and corticosteroids. The analgesic effect of the local anesthetic block in chronic painful conditions usually outlives the duration of action of local anesthetic. It may be because the local anesthetic leads to temporary interruption of nociceptive input to dorsal root ganglion which helps in decreasing the central sensitization. Once the action of local anesthetic ends, it may take noxious input considerable time to develop central sensitization again.^[12] In the present case also, the analgesic effect of local anesthetic outlived its duration of action. However, at the third time, we planned for the MIPSI with a mixture of lignocaine and methylprednisolone as we thought the effect of this mixture may be prolonged as compared to local anesthetic alone. Although the mechanism with which it occurs is largely unknown; however, it has been shown in studies that steroids blocks activity in nociceptive C fibers.^[13] The corticosteroids also suppress spontaneous and ectopic discharges in chronic and experimental neuromas, respectively, likely through direct action on cell membranes.^[14] Hence, the combination of local anesthetic and steroid may work better than local anesthetic alone. After the third MIPSI also, the patient is asymptomatic till date.

In summary, here we provide an association of CRPS in a patient with HSP, suggesting that CRPS may be a potential complication from this small-vessel vasculitic disorder. Timely diagnosis and intervention with conservative treatments and sympathetic block of the affected region may result in improvement of or the resolution of symptoms. Further research may be warranted to confirm the exact association between CRPS and HSP.

Informed consent

Yes.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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