364 Emotional triggers to pain in a patient with cutaneous leiomyomas associated with Reed syndrome

364 Emotional triggers to pain in a patient with cutaneous leiomyomas associated with Reed syndrome

Farid Y; Mostow E N

Journal of Investigative Dermatology

2019

2019-05

A 39 y/o man presented with hundreds of painful, flesh-colored <0.1 to 12 mm papules and nodules on his right forearm, upper arm, shoulder, and chest. The lesions began around puberty and gradually grew and spread. Throbbing/stabbing pain was caused by touch, pressure, sleep, wind, physical activity, swimming, showering, sweating, and changes in pressure and temperature. Piloerection also caused excruciating pain and occurred with sexual intercourse, strong emotions, scary movies, and classical music. Two punch biopsies revealed circumscribed nodular lesions in the dermis with fascicles of smooth muscle with elongated blunt nuclei and fibrillar eosinophilic cytoplasm. No significant atypia or atypical mitoses were noted. This was consistent with cutaneous pilar leiomyomata. Genetic testing revealed fumarate hydratase (FH) gene inactivation. Reed Syndrome, also known as hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC), is an autosomal dominant disease in which the FH gene is inactivated. HLRCC has been reported in 200 families worldwide. It predisposes patients to cutaneous leiomyomas, uterine leiomyomas, and renal cell carcinomas. Unique in this case was the age, location, and emotional triggers to pain. Cutaneous leiomyomas typically present at a mean age of 25 and involve the extensor surfaces, trunk, face, and neck. Previous reports have described pain with stress, strong emotions, light touch, and cold temperatures. Our patient added significant detail to emotional triggers. Local excision provided relief but was followed by recurrence. A clinical trial of Botox was offered but rejected in fear of receiving the placebo and pain at injection site. Our patient is managed by a pain clinic with oxycodone 5 mg/acetaminophen 325 mg and a topical cream that includes ketamine, diclofenac, baclofen, gabapentin, cyclobenzaprine, and bupivacaine. This regimen improved our patient’s functional status. Our patient’s emotional triggers allowed us to understand the impact of his illness and provided us with a useful metric to measure pain control and functional improvement.

Dermatology

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S63–S63

5

139

0022-202X