Correspondence Address: Dr. Ravi Shankar Sharma Room No. 16, OPD Block, Pain OPD, AIIMS, Rishikesh, Uttarakhand - 249 203 India
Source of Support: None, Conflict of Interest: None
Morphea is an autoimmune connective tissue disease of unrevealed etiology. It is associated with skin changes in the form of thickening along with deposition of excess of collagen in the affected lesion. Pain associated with morphea is usually a rare finding and is refractory to conventional analgesics. Hence, here we discuss a case of morphea with debilitating pain, that was successfully managed with stellate ganglion block.
Localized scleroderma, also known as morphea, is a chronic connective tissue disease of unrevealed etiology, and it is associated with skin changes in the form of thickening with excess collagen deposition in the hardened lesion. Apart from skin manifestation, pain in the affected lesion or area may be the presenting feature. Various options including nonsteroidal anti-inflammatory agents, opioids, topical therapy, and gabapentinoids have been used for pain management associated with it. Here, we present a case of morphea with debilitating pain, that was successfully managed with stellate ganglion block.
A 47-year-old male patient was referred from the dermatology department to pain outpatient department with chief complaints of severe pain (numerical rating scale [NRS] score – 9) in the left forearm and hand. It was associated with burning and tingling sensation without any motor or sensory loss. Medical management in the form of anticonvulsants (gabapentin) and antidepressants (duloxetine) along with opioids (tramadol) and topicals for a period of 3 months provided minimum pain relief. On examining this patient, multiple lesions were present over both the dorsal and ventral sides of the left hand. His previous documents revealed dermatological diagnosis of morphea, i.e., localized sclerosis. His skin was dry and reddish but was not associated with any swelling, temperature differences, allodynia, or hyperalgesia [Figure 1]. On evaluating him with pain detect tool, we obtained a score of 21, which suggested pain of neuropathic origin. When we assessed him for complex regional pain syndrome via Budapest criteria, results came as negative case. As there was failure of medical management for prolonged period, we decided to perform stellate ganglion intervention over the left side as history and examination suggested sympathetic system involvement. After explaining the procedure, informed written consent was obtained. His coagulation profile was sent prior to intervention, which yielded normal values. Thereafter, the patient was shifted to the procedure room. In the procedure room, standard monitors were attached and intravenous line was secured. Preliminary scanning which was done with the help of high-frequency (8–15 MHz) liner ultrasound transducer (LOGIQ™e, GE Healthcare Clinical Systems, Shanghai, China) revealed normal anatomy. Following initial scan, the patient's head was rotated toward the right side and the procedure area was prepared. Then, a probe was placed over the left side of the neck at the level of cricoid cartilage, and transverse process of the sixth cervical vertebra along with longus colli muscle was identified [Figure 2]. Following landmark identification, a 22G, 3.5” spinal needle was introduced via in-plane technique to reach lateral to the left side longus colli muscle. Following negative aspiration, 6 ml of total drug volume containing 1% lignocaine with 8 mg of dexamethasone was injected lateral to the longus colli muscle [Figure 3] and Video 1]. Following injection, ipsilateral upper limb temperature elevated without any associated Horner's syndrome or voice changes. Soon after the injection, the patient reported a near-complete pain relief (NRS – 2). He was advised to continue his previous medication, i.e., anticonvulsants (gabapentin) and tramadol till the next follow-up visit. Thereafter, the patient was discharged on the same evening. During the follow-up visit after 3 weeks, the patient was still comfortable (NRS – 3). We advised him to gradually deescalate the medication in a graded manner. Thereafter, the patient was transferred to the dermatology department for further management of skin problems. After 3 months, we had a telephonic conversation regarding his present pain intensity, which revealed a NRS of 3, and he was able to perform all the routine activities.
Figure 1: Morphea involving the left upper extremity
Stellate ganglion is formed by fusion of lower cervical and upper first thoracic sympathetic ganglion. Anatomically, it is located medial to the scalene muscles; lateral to the longus colli muscle, esophagus, trachea, and recurrent laryngeal nerve; anterior to the C7 transverse process and prevertebral fascia; superior to the subclavian artery; and posterior to the vertebral vessels., Stellate ganglion intervention is indicated for the treatment of various pain syndromes such as complex regional pain management, vascular insufficiency, hyperhydrosis, phantom pain, postherpetic neuralgia, cancer pain, cardiac arrhythmias, orofacial pain, and vascular headache.
Morphea is considered an autoimmune disorder characterized by fibrotic lesions of skin and the surrounding structures. Pain in morphea is considered unusual. The possible explanations for pain in our patient could be explained by the etiopathogenesis of morphea, which states autoimmune dysregulation or abnormal cytokine production which results in morphea also inflames nearby nerves, resulting in pain.
In our patient, it might be possible that the similar process of autoimmune impairment might have damaged the nearby nerves, leading to maladjusted autonomic system, which resulted in severe pain having sympathetic component.
Previously, Jeon and Kim have also described a patient of morphea with severe pain, who was successfully managed with methotrexate.
Stellate ganglion interventions can be performed via fluoroscopy, computed tomography, and ultrasonography (USG), but the use of ultrasound improves the safety of procedure by direct visualization of the related anatomical structures, minimizing the risk of injury to the neighboring structures, thereby helping in the deposition of drug subfascially with real-time imaging. Although in dermatology, stellate ganglion intervention has been previously employed for the improvement of acral circulation in a patient with systemic sclerosis, till now, no such cases or similar type of such interventions (sympathetic blocks) have been performed for pain relief in dermatological conditions. In our patient, we performed stellate ganglion intervention via USG using a total volume of 6 ml of local anesthesia with steroid, which resulted in immediate near-complete pain relief. This can explain the sympathetic nervous system involvement in patients of morphea having severe pain, which can be relieved by blockade of sympathetic supply of the involved extremity.
Morphea or limited scleroderma could result in debilitating pain having sympathetic component. USG-guided stellate ganglion intervention can be used for managing pain associated with such patients. USG not only allows direct monitoring of the spread of the local anesthetic but also avoids complication such as recurrent laryngeal nerve palsies or intravascular spread. To the best of our knowledge, this is the first time that stellate intervention has been utilized for pain associated with such dermatological condition.
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.