Treatment of cutaneous leiomyomas with 5% lidocaine patches in a patient with hereditary leiomyomatosis and renal cell cancer (Reed syndrome).

Introduction

Hereditary leiomyomatosis and renal cell cancer (HLRCC) (formerly known as Reed syndrome, multiple cutaneous and uterine leiomyomatosis, leiomyomatosis cutis et uteri, and multiple leiomyomatosis) is an autosomal dominant syndrome comprising cutaneous leiomyomas, uterine leiomyomas, or renal tumors. The cutaneous leiomyomas of HLRCC are classically painful and difficult to treat. We report the first case, to our knowledge, of cutaneous leiomyomas symptomatically treated with 5% lidocaine patches.

Report of a case

A 37-year-old white woman presented with painful lesions on the trunk and extremities of 3 years' duration. These lesions intermittently ached and itched, especially when bumped, scratched, or exposed to cold. Symptoms worsened with fatigue and stress. Her father suffered from similar lesions. Physical examination found numerous smooth, firm, pink dermal papules and nodules. These were grouped in tight clusters on the upper back, right shoulder, and left thigh (Fig 1). A skin biopsy was performed from the upper back. Histopathology found a well-circumscribed proliferation of fascicles of spindle cells that stained positive with smooth muscle actin immunohistochemistry (Fig 2, A and B) compatible with cutaneous leiomyoma.

Fig 1

Clinical image of cutaneous leiomyomas. Numerous smooth, firm, pink dermal papules and nodules coalescing into plaques on the left upper back.

Fig 2

Histopathology A and B, Cutaneous leiomyoma: there is a well-circumscribed dermal proliferation composed of fascicles of spindle cells that stain diffusely with smooth muscle actin immunohistochemistry. (A, Hematoxylin-eosin stain; B, smooth muscle actin; original magnifications: A and B, ×100.) C and D, Atypical leiomyoma: in the uterus, there is a well-circumscribed somewhat fascicular neoplasm composed of smooth muscle cells with mild pleomorphism. (C and D, Hematoxylin-eosin stain; original magnifications: C, ×16; D, ×100.)

Before presentation, the patient struggled with menorrhagia and infertility secondary to uterine fibroids, necessitating myomectomy. Uterine pathology found a large smooth muscle neoplasm with focal atypia, compatible with atypical uterine leiomyoma (Fig 2, C and D). Images of the kidneys were normal. The patient was referred for genetic testing, and analysis of the fumarate hydratase gene found a pathogenic FH mutation. Her clinical, genetic, and histopathologic findings were consistent with a diagnosis of HLRCC.

Discussion

HLRCC is characterized by cutaneous leiomyomas, early-onset uterine leiomyomas, and an increased risk of renal cell carcinoma. Renal cell carcinoma is a serious complication of HLRCC but present in only 10% to 15% of individuals. In contrast, cutaneous leiomyomas are present in all patients. Eighty-nine percent of patients report lesion-associated pain, characteristically precipitated by heat/cold, trauma, or light touch.

Cutaneous leiomyomas do not resolve spontaneously; thus, symptoms should guide treatment. In mild cases, avoidance of painful triggers may be sufficient. Painful lesions can be treated with excision; however, this treatment has a high rate of recurrence, may necessitate skin grafting with an undesired cosmetic outcome, and may be impractical, as leiomyomas tend to group together in larger “school-of-fish” arrays. Destructive techniques such as electrodessication, cryotherapy, and radiotherapy have been used with varying results and are complicated by scarring, hypopigmentation, and incomplete removal.2, 3, 4 Medications such as nitroglycerin, nifedipine, phenoxybenzamine, doxazosin, and gabapentin have been used with limited-to-moderate success in providing periodic pain relief by decreasing smooth muscle contraction and nerve stimulation.2, 3, 4 However, some of these medications can provoke systemic side effects. Newer therapeutic options include botulinum toxin injection and carbon dioxide laser ablation,3, 4 but these procedures are often expensive and, in the case of botulinum toxin injection, have not found a significant decrease in average lesional pain or pain after ice provocation.

Our patient's main concern was pain relief, which she was unable to achieve with daily nonsteroidal anti-inflammatory medications. She preferred a noninvasive, nondisfiguring approach and was started on 5% lidocaine patches. She easily cut the patches to size and applied them topically for pain relief. Each patch is 10 × 14 cm with an adhesive layer of 5% lidocaine (700 mg per patch). Up to 3 patches can be applied for 12 hours within a 24-hour period. On a pain scale of 0 to 10 (0 = no pain, 10 = the worst pain possible), a single patch reduced her pain from 5 to 0. After using the patches for several months, the patient's pain frequency significantly decreased from a patch application of 1 to 3 times per week to once every 1 to 2 months.

Topical lidocaine treats localized pain by decreasing the transmission of excitatory pain and itch impulses to the dorsal horn of the spinal cord. Lidocaine patches are inexpensive and easily transportable and can be customized to size. Systemic adverse reactions are rare because of the small dose absorbed. Our patient reported a significant decrease in severity and frequency of pain and has found lidocaine patches to be a preferable treatment to oral medications. Lidocaine patches provide an alternate noninvasive, safe, and effective treatment for painful cutaneous leiomyomas.