Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
 
Users online: 2480  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
Table of Contents 
E–CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 2  |  Page : 160
Unilateral multi-segmental leiomyomas: A report of rare case


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

Date of Web Publication5-Mar-2013

Correspondence Address:
Chandramohan Kudligi
Department of Skin and STD, Karnataka Institute of Medical Sciences, Hubli, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.108086

Rights and Permissions

   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.


Keywords: Leiomyomas, multi-segmental, unilateral


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

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 2019 Sep 22];58:160. Available from: http://www.e-ijd.org/text.asp?2013/58/2/160/108086

What was known? 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. These lesions can be arranged in diffuse (disseminated), blaschkoid, or segmental (zosteriform) patterns.



   Introduction Top


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 Top


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: Multiple piloleiomyomas on the right arm and shoulder

Click here to view
Figure 2: Multiple piloleiomyomas distributed along 5th cervical, 6th dorsal, and 1st sacral segments of right half of the body

Click here to view


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: Photomicrograph of skin biopsy showing interlacing muscle bundles (H and E, ×100)

Click here to view



   Discussion Top


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 Top

1.Badeloe 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.  Back to cited text no. 1
[PUBMED]    
2.Fisher WC, Helwig EB. Leiomyomas of the skin. Arch Dermatol 1963;88:510-20.  Back to cited text no. 2
[PUBMED]    
3.Alam 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.  Back to cited text no. 3
[PUBMED]    
4.Lucil 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.  Back to cited text no. 4
    
5.Sahoo B, Radotra BD, Kaur I, Kumar B. Zosteriform pilar leiomyoma. J Dermatol 2001;28:759-61.  Back to cited text no. 5
[PUBMED]    
6.Kaliyadan F, Manoj J, Dharmaratnam AD. Multiple cutaneous leiomyomas: Pain relief with pulsed hysocine butyl bromide. Indian J Dermatol 2009;54:72-4.  Back to cited text no. 6
[PUBMED]  Medknow Journal  

What is new? Segmental distribution of leiomyoma is common. However 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.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (1,633 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed1936    
    Printed40    
    Emailed3    
    PDF Downloaded40    
    Comments [Add]    

Recommend this journal