Familial myomatosis cutis et uteri, segmental type 2
A 50-year-old female presented to us with history of developing multiple brown painful lesions which appeared initially over her left leg. They extended to appear insidiously over trunk, chest, and upper limbs about 25 years back which extended to appear insidiously despite excision of the left leg lesion. Pressure and exposure to cold aggravated the pain. She also underwent hysterectomy, owing to menorrhagia and multiple uterine fibromas, 25 years ago. Dermatological examination revealed multiple tender, hyperpigmented papulonodules over the left lower limb, trunk, right side of the chest, and bilateral upper limbs [Figures , and ,]. A clinical suspicion of leiomyomas was confirmed by histopathological findings of a circumscribed tumor in the dermis composed of bundles of smooth muscles cells arranged in an interlacing and whorled pattern, having abundant eosinophilic cytoplasm and elongated nuclei with blunt ends. Deep red color with Masson's Trichome confirmed smooth muscle fibers [Figure ,]. Mild lymphocytic infiltrate was present in the interstitial fibrous tissue. Gynecological consultation for her daughter's complaints of menorrhagia revealed uterine fibroids. A diagnosis of familial myomatosis cutis et uteri, segmental type 2 was made. She experienced pain relief with nifedipine and gabapentin.