Indian Journal of Dermatology
E–CASE REPORT
Year
: 2013  |  Volume : 58  |  Issue : 2  |  Page : 160-

Unilateral multi-segmental leiomyomas: A report of rare case


Chandramohan Kudligi1, Binod K Khaitan2, Pradeep Vittal Bhagwat1, Dinesh Prasad Asati3,  
1 Department of Skin and STD, Karnataka Institute of Medical Sciences, Hubli, Karnataka, India
2 Department of Skin and STD, All India Institute of Medical Sciences, New Delhi, India
3 Department of Skin and STD, LN Medical College and JK Hospital, Bhopal, India

Correspondence Address:
Chandramohan Kudligi
Department of Skin and STD, Karnataka Institute of Medical Sciences, Hubli, Karnataka
India

Abstract

A 30-year-old female presented to us with multiple tender erythematous papules and nodules. These lesions showed multi-segmental distribution along 5 th cervical, 6 th dorsal, and 1 st sacral segments of right half of the body. Histopathological examination confirmed the clinical diagnosis of leiomyoma. Patient was started on nifedipine 10 mg thrice-daily with significant symptomatic improvement in 3 months. Though segmental distribution of leiomyoma is common, unilateral multi-segmental distribution has not been so far reported in the literature. Hence, the case is being reported for its rare presentation and the need for long-term follow-up in view of its association with aggressive renal carcinoma.



How to cite this article:
Kudligi C, Khaitan BK, Bhagwat PV, Asati DP. Unilateral multi-segmental leiomyomas: A report of rare case.Indian J Dermatol 2013;58:160-160


How to cite this URL:
Kudligi C, Khaitan BK, Bhagwat PV, Asati DP. Unilateral multi-segmental leiomyomas: A report of rare case. Indian J Dermatol [serial online] 2013 [cited 2021 Apr 10 ];58:160-160
Available from: https://www.e-ijd.org/text.asp?2013/58/2/160/108086


Full Text

 Introduction



Cutaneous leiomyomas are rare benign tumors arising from smooth muscle cells. According to their site of origin, they can be classified into piloleiomyomas, angioleiomyomas, and dartoic leiomyomas. Piloleiomyomas are the most common type among cutaneous leiomyomas and are often multiple than solitary. These lesions can be arranged in diffuse (disseminated), blaschkoid or segmental (zosteriform) patterns. Piloleiomyomas are transmitted as an autosomal-dominant trait and may be associated with uterine leiomyomas and aggressive renal carcinoma.

 Case Report



A 30-year-old unmarried female presented to us with 15 years history of painful papules and nodules only on right half of the body. These lesions initially appeared on the right upper limb and the shoulder but subsequently developed over the trunk, gluteal region, and the thigh of the same side. Each lesion started as a papule, which gradually increased in size to form a nodule. She also gave history of pain associated with these lesions, especially on exposure to cold and touch. Menstrual history did not reveal any abnormality, and there were no known family members with cutaneous leiomyomas or uterine fibroids. General physical examination was unremarkable. Cutaneous examination revealed multiple tender erythematous papules and nodules [Figure 1], varying in size from 0.5 cm to 2 cm, distributed in segmental pattern over the upper limb, trunk, and thigh on the right side of the body with sharp limitation in the midline [Figure 2].{Figure 1}{Figure 2}

Based on history and cutaneous examination findings, we considered the differential diagnosis of leiomyomas, angiolipomas, eccrine spiradenomas, and neurofibromas. Routine hematological investigations, blood urea, serum creatinine, and ultrasound examination of the abdomen were within normal limits. HIV serology was negative. Histopathological examination of skin biopsies taken from the arm, trunk, and thigh showed bundles of spindle-shaped cells arranged in an interlacing and whorled pattern with elongated nuclei having rounded ends confirming the diagnosis of piloleiomyoma [Figure 3]. Patient was started on nifedipine 10 mg thrice-daily with significant symptomatic improvement in 3 months.{Figure 3}

 Discussion



Cutaneous leiomyomas are rare benign tumors arising from smooth muscle cells. According to their site of origin, they can be classified into 3 types: (i) those derived from the arrector pili muscle of hair follicles (piloleiomyomas), (ii) those originating from the vascular smooth muscle (angioleiomyomas), and (iii) those arising from the smooth muscle of genital skin (dartoic leiomyomas). [1] Piloleiomyomas are the most common type among cutaneous leiomyomas and are often multiple. [2]

These are transmitted as an autosomal-dominant trait and may be associated with uterine leiomyomas (multiple cutaneous and uterine leiomyomatosis, also known as Reed's syndrome or MCUL) and aggressive renal carcinoma (hereditary leiomyomatosis and renal cell carcinoma or HLRCC). Recently, a loss of function mutation in the gene encoding fumarate hydratase on chromosome 1q42.3-43 has been shown to predispose individuals to MCUL and HLRCC. [3]

Piloleiomyomas usually present as small, red-brown, firm papules on the extremities or trunk of young adults. Individual lesions range in size from few millimeters to 1 cm that are fixed to the skin and are freely movable over underlying deeper structures. Patients with piloleiomyoma often have pain that may be spontaneous or secondary to cold, pressure, or emotion. [4] Pressure of the tumor on local nerve fibers and contraction of the smooth muscle fibers are the possible explanation for this phenomenon. These lesions can be arranged in diffuse (disseminated), blaschkoid, or segmental (zosteriform) patterns. [5] Segmental patterns can further be classified as type 1 segmental and type 2 segmental. Type I segmental reflects heterozygosity of a post-zygotic mutation, leading to segmental skin lesions comparable to non-mosaic phenotype, whereas type 2 segmental reflects post-zygotic mutational event in a heterozygous embryo with subsequent loss of heterozygosity, resulting in pronounced pattern of the segmental lesions superimposed on the ordinary phenotype of the underlying disease. Histopathologically, piloleiomyomas are circumscribed, non-encapsulated tumors. The tumor is composed of bundles of smooth muscles arranged in an interlacing and sometimes whorled pattern. The cells have abundant eosinophilic cytoplasm and elongated nuclei with blunt ends.

Treatment of the leiomyomas is not satisfactory. Surgical excision is often considered if the lesions are few in number. However, in case of extensive lesions, various pharmacological options like nifedipine, doxazocine, gabapentine, and topical 9% hyoscine hydrobromide have been used to alleviate the pain associated with these tumors. [6]

In conclusion, we present a case of unilateral multi-segmental leiomyomas. Though segmental distribution of leiomyoma is common, multi-segmental variant has not been reported so far, and PubMed search between 1966 and 2011 could not locate any case reports of piloleiomyomas showing unilateral and multi-segmental distribution. Hence, the case is being reported for its rare presentation and the need for long-term follow-up in view of its association with aggressive renal carcinoma.

References

1Badeloe S, van Geel M, van Steensel MA, Bastida J, Ferrando J, Steijlen PM, et al. Diffuse and segmental variants of cutaneous leiomyomatosis: Novel mutations in the fumarate hydratase gene and review of the literature. Exp Dermatol 2006;15:735-41.
2Fisher WC, Helwig EB. Leiomyomas of the skin. Arch Dermatol 1963;88:510-20.
3Alam 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.
4Lucil EW, Ross ML, Murad A. Neoplasias and hyperplasias of muscular and neural origin. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick's Dermatology in General Medicine. 7 th ed. New York: McGraw-Hill; 2009. p. 1172-83.
5Sahoo B, Radotra BD, Kaur I, Kumar B. Zosteriform pilar leiomyoma. J Dermatol 2001;28:759-61.
6Kaliyadan F, Manoj J, Dharmaratnam AD. Multiple cutaneous leiomyomas: Pain relief with pulsed hysocine butyl bromide. Indian J Dermatol 2009;54:72-4.