Reed's Syndrome.

Sir,

A 42-yr-old female presented with the complaints of papular and nodular eruptions on the right lower cheek and neck for the last 12 yrs. Lesion were slightly painful during winters and on touch. On examination skin-colored soft and slightly tender nodules and papules of size 0.5 – 1 cm were present [Figures 1 and 2].

Figure 1

Papulonodular lesions on the right cheek and neck

Figure 2

Close-up view

Laboratory investigations along with histopathology was done; CBC, LFT, renal profile were insignificant but urine examination showed occasional erythrocytes. Histopathology revealed encapsulated tumors composed of interlacing fascicles of smooth muscles having eosinophilic cytoplasm and blunt ended nuclei. Tumors appeared to be arising from arrector pili muscles suggestive of pilar leiomyoma.

Her hysterectomy, right salpingo-oophorectomy and excision of left ovarian mass were done 12 years back because of uterine fibromas causing menstrual irregularities and dysmenorrhea. These uterine fibromas are infact leiomyomas. Although the treatment of leiomyoma is not very satisfactory our patient was symptomatically benefited with analgesics and calcium channel blockers.

Cutaneous leiomyomas first described by Virchow in 1854[1] are the benign tumors arising from smooth muscles cells. According to the site of origin they are of three types; a) pilar leiomyoma derived from the arrector pili muscles of the hair follicles, b) angioleiomyoma originating from the vascular smooth muscles, c) dartoic leiomyoma arises from the smooth muscles of genital skin and areola.[12] In contrast to the solitary nature of presentation of angioleiomyoma the dartoic and pilar leiomyomas manifest as multiple tumors. Its autosomal dominant inheritance pattern was first described by Kloepper et al, in 1958.[3] Pilar leiomyomas are usually seen in 2nd to 4th decade of life as brown or red color papules and nodules localized to the face, trunk and extremities. Size of the lesions varies between 1 and 1.5 cm in diameter.

These benign tumors are painful in response to physical stimulus like pressure or low temperature and is generally aggravated by contact with cold object.[4] In 1973 Reed et al., for the first time reported the association of cutaneous leiomyoma with the leiomyoma of the uterus and labeled as Reed's syndrome[5] which is in present scenario known as Multiple cutaneous and uterine leiomyomatosis syndrome (MCUL; OMIM 150800) and the mutation is fumarate hydratase.[5] Recently its association with renal cell carcinoma has been shown by Launonen et al.[6]

In our case occasional erythrocytes in the urine was seen. Since this is an under-recognized condition, its increase clinical awareness is important because of the associated risk of severe uterine fibroid and with renal cell carcinoma.