Progressive Cutaneous Scleroderma

AUTHORS:
Sebastian J. Ksionski, MD, and Laurie Willhite, PharmD
Hennepin County Medical Center, Minneapolis, Minnesota

CITATION:
Ksionski SJ, Willhite L. Progressive cutaneous scleroderma. Consultant. 2016;56(8):765-766.

A 60-year-old woman presented with painful and sclerotic hands and fingers. She had been an airline attendant but had to quit her job as a result of the pain and her inability to use her hands over the past 7 years.

History. The patient recently had been seen by a pain physician, had been started on opioids, and had received stellate ganglion blocks that had yielded very little relief. A rheumatologist had prescribed methotrexate and corticosteroids in an attempt to stop excessive overproduction of connective tissue and to help control her disease state, which also did not help. She had opted out of tacrolimus therapy, which can soften the fibrosis but also is associated with an increased risk of malignancy.¹

Physical examination. The patient had no lung or heart problems or complaints. No vascular abnormalities or telangiectasia were seen on examination. Her fingers were unable to be opened or flexed and were wooden-like on palpation; some digits were missing from self-amputation with chronic pain (Figures 1 and 2).

Figure 1. The patient's hands at presentation.

Figure 2. A photograph of the patient’s hands 15 years before presentation.

The woman received a clinical diagnosis of progressive cutaneous scleroderma.

Discussion. Localized scleroderma has an estimated prevalence of 50 per 100,000 before age 18 years and 220 per 100,000 by age 80.¹ Scleroderma is a chronic autoimmune disorder that is manifested by excess synthesis and deposition of collagen in skin and connective tissue.¹ 

Vascular abnormalities occur as Raynaud phenomenon and swelling of the acral portions of the extremities, with thickening of the skin of the fingers as the most common complaints in CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia). Cutaneous manifestations were the only findings present in our patient’s case. Generalized scleroderma, on the other hand, can affect the cardiovascular and pulmonary systems; kidney failure may occur in severe cases.²

The usual skin presentation in progressive localized scleroderma includes sclerotic fibroblasts (myofibroblasts) in the dermis that are capable of multiple replicative passages; this often affects the arms, face, and legs.³ The skin in affected areas often takes the form of morphea—patches or linear bands that become thick, hard, and discolored.^2,3 Morphea usually is not progressive; however, if it is progressive, as in progressive cutaneous scleroderma, it may lead to disfiguring and debilitating deformities, especially in the limbs.

Outcome of the case. Consultation with a pain psychologist was recommended to help the woman cope with her painful hands and to help her be more independent, including managing even simple everyday activities. She also obtained custom-made utensils (Figure 3) and other items of daily living.

Figure 3. Custom utensils such as this spoon were fashioned to help the woman eat.

A topical compounded cream containing 10% ketamine, 2% baclofen, 6% gabapentin, 6% verapamil, and 3% pentoxifylline also was started, focused on reducing fibrosis and peripheral neuropathy.³ Within a month of using this cream, the patient noticed remarkable improvement in sensation and pain, so much that we were able to begin weaning her from chronic opioid therapies. At a 3-month follow-up visit, she was visually excited and tearful, reporting that “the cream is the only thing that truly works for my hands.” She reported “feeling her fingers again” as an indicator that her hand pain and sclerotic/fibrotic digits had some return of sensation.

The compounded cream’s active ingredients, which address the vascular perfusion and inhibition of certain receptors (pentoxifylline)⁴ and the up-regulation of collagenase and collagen breakdown (verapamil),⁵ in conjunction with the regulation of calcium channels and pain transmitters (gabapentin and ketamine)^6-8 and potential improvements in soft tissue relaxation with analgesic properties (baclofen),⁹ make this combination very effective.

Physical therapy would have been an appropriate treatment prior to self-fusion of her joints; however, at this stage the joints are fused and sclerotic. Opioids should be viewed as a means of pain control when other conservative therapies and medications have failed. Spinal cord stimulation could be a good final option to help with the neurogenic pain, given the responses to the stellate blocks if topical therapy were ineffective.

Six months into her continued daily use of the compounded cream, the patient had been weaned from most of her short-acting hydrocodone/acetaminophen and had completely discontinued fentanyl.

References:

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