Indian Journal of Dermatology
CORRESPONDENCE
Year
: 2012  |  Volume : 57  |  Issue : 2  |  Page : 156--157

Reed's syndrome


K Pradeep Srivastava1, AK Bajaj2,  
1 Department of Mansi Skin and Allergy Clinic, Allahabad, India
2 Bajaj Skin Clinic, Allahabad, India

Correspondence Address:
K Pradeep Srivastava
Department of Mansi Skin and Allergy Clinic, Allahabad
India




How to cite this article:
Srivastava K P, Bajaj A K. Reed's syndrome.Indian J Dermatol 2012;57:156-157


How to cite this URL:
Srivastava K P, Bajaj A K. Reed's syndrome. Indian J Dermatol [serial online] 2012 [cited 2021 Jun 12 ];57:156-157
Available from: https://www.e-ijd.org/text.asp?2012/57/2/156/94296


Full Text

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 [Figure 1] and [Figure 2].{Figure 1}{Figure 2}

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. [1],[2] 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 2 nd to 4 th 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.

References

1Virchow R. Über Makroglossie und pathologische Neubildung quergestreifter Muskelfasern. Virchows Arch Pathol Anat 1854;7:126-38.
2Fischer WC, Helwig EB. Leiomyomas of the skin. Arch Dermatol 1963;88:510-20.
3Kloepfer HW, Krafchuk J, Derbes V, Burks J. Hereditary multiple leiomyoma of the skin. Am J Hum Genet 1958;10:48-52.
4Alam NA, Barclay E, Rowan AJ, Tyrer JP, Calonje E, Manek S, et al. Clinical features of multiple cutaneous and uterine leiomyomatosis: An underdiagnosed tumor syndrome. Arch Dermatol 2005;141:199-206
5Reed WB, Walker R, Horowitz R. Cutaneous leiomyomata with uterine leiomyomata. Acta Derm Venereol 1973;53:409-16.
6Launonen V, Vierimaa O, Kiuru M, Isola J, Roth S, Pukkala E, et al. Inherited susceptibility to uterine leiomyomas and renal cell cancer. Proc Natl Acad Sci USA 2001;98:3387-92.