Case Letter

Management of Refractory Pain From Hereditary Cutaneous Leiomyomas With Nifedipine and Gabapentin

Author and Disclosure Information

Practice Points

  • Cutaneous leiomyomas (piloleiomyomas) are benign smooth muscle tumors derived from the arrector pili muscle.
  • Patients presenting with multiple cutaneous leiomyomas should be evaluated for hereditary leiomyomatosis and renal cell carcinoma syndrome, an autosomal-dominant disorder, which also predisposes to the development of symptomatic uterine fibroids and uterine leiomyosarcoma.
  • Cutaneous leiomyomas may be a source of considerable pain, which may respond to treatment with nifedipine in combination with gabapentin.


 

References

To the Editor:

Leiomyomas are benign smooth muscle tumors. There are 3 types of cutaneous leiomyomas: (1) piloleiomyomas, arising from the arrector pili muscles; (2) angioleiomyomas, arising from the muscles surrounding dermal blood vessels; and (3) leiomyomas of the external genitalia, arising from the dartoic, vulvar, or mammary smooth muscles.1 There is no gender predilection for cutaneous leiomyomas, and lesions present on average at approximately 40 to 45 years of age.2

Piloleiomyomas are the most common type of cutaneous leiomyomas and typically present as red-brown papules and nodules on the trunk, arms, and legs.3 Piloleiomyomas often are associated with spontaneous or induced pain (eg, with cold exposure). The pain associated with piloleiomyomas can be severely debilitating to patients and may have a considerable impact on their quality of life.

A 40-year-old woman presented to our clinic with numerous widespread, painful, red-brown papules and nodules on the head, neck, chest, abdomen, back, arms, and legs of 6 years’ duration that were increasing in number (Figure 1). She had a history of uterine leiomyomas and type 2 renal papillary carcinoma following a left nephrectomy at 38 years of age. The patient’s mother had a history of similar skin lesions as well as uterine cancer. Multiple excisional biopsies were performed, all of which showed piloleiomyomas on histopathology (Figure 2). The pain associated with the patient’s extensive cutaneous leiomyomas considerably impaired her quality of life. Although she experienced pain in all affected areas of the body, the pain was the worst in the upper arms. She reported having requested a nerve ablation procedure from an outside pain management clinic, which was denied for unknown reasons.

Figure 1. Numerous painful red-brown papules and nodules on the neck, chest, and left arm.


Figure 2. Piloleiomyoma in the reticular dermis forming a nodule with fascicles of myocytes between collagen bundles at the periphery (H&E, original magnification ×100).

Two years prior to the current presentation, the patient had been treated by a pain management specialist with gabapentin 300 mg twice daily as needed for pain associated with leiomyomas. The patient followed this regimen approximately 3 times weekly for the preceding 1 to 2 years with reduction in her pain symptoms; however, the painful episodes became more frequent and severe over time. The patient reported being unable to further increase the gabapentin dosing frequency because it made her too drowsy and impacted her ability to work a job that required heavy lifting. Thus, the patient requested additional therapy and was subsequently treated at our clinic with numerous excisional biopsies of the most painful lesions during the 2 years prior to her current presentation.

When the patient re-presented to our clinic, she requested additional lesion excisions given that she had experienced some pain relief from this treatment modality in the past; however, these prior excisions only resulted in local pain relief limited to the site of the excision. Because of the extent of the lesions and the patient’s inability to tolerate pain from the lidocaine injections, we did not feel multiple excisions were a practical treatment option. The patient subsequently was offered a trial of cryotherapy for symptom relief based on a reported case in which this modality was successfully used.4 After discussing the risks and benefits associated with this treatment, cryotherapy was attempted on a few of the leiomyomas on the patient’s right shoulder; however, she experienced severe pain during cryotherapy treatment, and the procedure had to be aborted.

Next Article: