Management of painful pemphigus vulgaris – Relieving pain and restoring dignity

Dear Editor, Pemphigus is an autoimmune disease that causes painful blistering of the skin and mucus membrane, significantly affecting patient's well-being. Pemphigus vulgaris (PV) is the most prevalent type of pemphigus that presents as painful persistent erosions and nonhealing ulcers. Treatment is focused on suppression and remission of PV. Despite immunosuppressive therapy, there is a protracted course until achievement of remission. Since remission is not instantaneous, associated pain is severe and at times intractable, culminating in a chronic pain condition.[1,2] We present a patient with painful PV and its management. A 39-year-old female presented to the pain clinic with excruciatingly painful PV. She had history of multiple painful oral ulcers 3 months ago, complicated by multiple fluid-filled lesions all over the body in the last 2 months. Histopathologic examination of skin biopsy was consistent with PV. She was subsequently admitted in dermatology ward for phase I dexamethasone–cyclophosphamide pulse (DCP) therapy. Patient had severe pain despite treatment with oral nonsteroidal anti-inflammatory drugs, opioids, and completion of phase I DCP therapy. She was then referred to the pain clinic. On examination, the patient was barely able to dress herself [Figure 1a and b]. Even the touch of clothes was extremely painful. The pain score (Numeric Rating Scale) was 10/10. She was started on oral gabapentin 300 mg twice daily, amitriptyline 10 mg in the morning and 25 mg at bedtime, tramadol (37.5 mg) and paracetamol (325 mg) combination six hourly, and clonazepam 0.5 mg once a day. After 2 weeks, her pain score was 5/10. Consequently, amitriptyline was revised to 25 mg at bedtime only and oral tramadol and paracetamol combination was reduced to eight hourly. The second phase of DCP therapy was also completed. By the end of 3 weeks, her pain score was 3/10 and the pain medications were gradually tapered over next 6 weeks. Gabapentin was reduced to 300 mg at bedtime, amitriptyline was reduced to 10 mg at bedtime, and oral tramadol and paracetamol combination was reduced to twice a day for 2 weeks and subsequently once a day for 2 weeks and then as and when required. At the end of 6 weeks, she was only on gabapentin 300 mg and amitriptyline 10 mg at bedtime [Figure 1c]. By the end of 3 months, all medications were stopped.Figure 1: (a and b) Painful severe pemphigus vulgaris on day 1. (c) Severe pemphigus vulgaris after 6 weeks of treatmentThe proposed cause of neuropathic pain in PV is postulated to be perineural inflammation due to PV lesions. The literature is sparse, and treatment options are opioids, neuromodulators, and DCP therapy, with difficulty weaning off after discharge. The unremitting nature of the symptoms predisposes such patients to become depressive or even suicidal.[1,3,4] The management of pain in our patient was directed toward both achieving pain relief as well as restoring dignity of a young female who was barely able to cover herself owing to excruciating pain and had to go through a lot of pudency. Pain management strategies resulted in pain relief in our patient and she was able to dress (cover) herself. There is a well-known neuropathic component to the pain associated with PV. The combination of antiepileptic drug and tricyclic antidepressant along with titrated dose of analgesics turned out to be extremely effective in alleviating the pain. Considering the chronic and relapsing nature of PV, supportive measures are equally important.[1,3,4] Due to well-known association of chronic pain with PV, early referral to a pain clinic is warranted. Timely intervention by the pain physician not only relieved her pain, but also restored her dignity. 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.