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Year : 2005  |  Volume : 71  |  Issue : 6  |  Page : 449-451

Multiple painful nodules


1 Department of Skin and STD,Kasturba Hospital, Manipal, India
2 Department of Pathology, Kasturba Hospital, Manipal

Correspondence Address:
Vandana Mehta Rai
Assistant Professor, Dept of Skin and STD, Kasturba Medical College, Manipal- 576104, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.18961

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How to cite this article:
Rai VM, Balachandran C, Kudva R. Multiple painful nodules. Indian J Dermatol Venereol Leprol 2005;71:449-51

How to cite this URL:
Rai VM, Balachandran C, Kudva R. Multiple painful nodules. Indian J Dermatol Venereol Leprol [serial online] 2005 [cited 2019 Dec 6];71:449-51. Available from: http://www.ijdvl.com/text.asp?2005/71/6/449/18961


A 49-year-old lady presented with multiple painful reddish brown nodules over the body since the age of 15 years. At onset there was only one nodule on the left scapula that gradually increased in size associated with pain on tactile stimulus. Later, over the course of years, she developed multiple small reddish-brown papulonodules on the left forearm, left gluteal region [Figure - 1], and face, and on the right breast around the areola. On further questioning, she gave a history of having undergone hysterectomy a few years back due to nodules in the uterus. There was no history of similar lesions in her mother or sister. All routine investigations such as hemogram, urine analysis, liver and renal function tests, chest X-ray, and ultrasonography of abdomen were normal.

A biopsy from one of the new nodules showed spindle shaped smooth muscle cells with cigar shaped nuclei arranged in fascicles and whorls, with a sparse lymphocytic infiltrate in the interstitium, thus confirming the diagnosis of piloleiomyoma [Figure - 2] [Figure - 3].


  What is your diagnosis? Top


Diagnosis: Myomatosis cutis et uteri (Reed syndrome)


  Discussion Top


Women with multiple piloleiomyomas may have uterine leiomyomas. If the latter are present, the patient is likely to have a familial condition called as familial myomatosis cutis et uteri or Reed syndrome, which is inherited as an autosomal dominant trait with incomplete penetrance. [1],[2],[3] In such cases, a uterine myoma requires careful monitoring since it is associated with a significant risk of gynecological complications. Fumarate hydratase, a component of the tricarboxylic acid cycle, acts as a tumor suppressor gene. Germline mutations in the fumarate hydratase gene predispose to multiple cutaneous and uterine leiomyoma syndrome.[4] Mutations are found in 75% cases of multiple cutaneous and uterine leiomyoma syndrome.

Leiomyomas are benign soft tissue tumors that may originate from the arrector pili muscle of hair follicles (piloleiomyomas), tunica dartos of the scrotum, mammillary muscle of the nipple (genital leiomyomas), and the smooth muscles of blood vessels (angioleiomyoma).[5]

Piloleiomyomas generally occur in the age group of 10-30 years. They may be solitary or multiple and present clinically as erythematous to brown colored papulonodules located over the extensor extremities, trunk, neck and face. The most common feature in patients with multiple piloleiomyomas is pain, which may be induced by cold, tactile and emotional stimuli. Because piloleiomyomas develop in the superficial dermis, they are fixed in the skin; however, they can be moved easily over the deeper subcutaneous tissues. Piloleiomyomas can develop sporadically or be transmitted genetically.[6] They may even occur in a dermatomal or linear pattern.[7] Angioleiomyomas present as a solitary skin colored nodule on the lower extremities. They too are often painful. The pathogenesis of pain associated with these lesions is a mystery. Some authors suggest it is due to local pressure by the tumor on the neurofibrils or due to constriction of the vessels, causing local ischemia.[8] Genital leiomyomas arise from the dartos muscle of the scrotum or from the smooth muscle of the labia majora. They are usually asymptomatic and range in size from a few millimeters to a few centimeters.

Leiomyomas are benign tumors and medical management has a limited role. Calcium channel blockers, particularly nifedipine, relieve pain associated with piloleiomyomas.[9] They act by inhibiting the movement of extracellular calcium ions across the cell membrane into the smooth muscle cell, thereby inhibiting muscular contraction. Besides this, phenoxybenzamine[10] and gabapentin[11] have also been tried for piloleiomyomas associated pain. Surgical excision is effective in solitary cases. CO2 laser ablation has been tried in multiple leiomyomas.[12]



 
  References Top

1.Garcia MP, Pujol RM, Alomar A. Familial leiomyomatosis cutis et uteri (Reed's syndrome). Arch Dermatol Res 1988;280:29-32.  Back to cited text no. 1      
2.Fearfield LA, Smith JR, Bunker CB. Association of multiple familial cutaneous leiomyoma with a uterine symplastic leiomyoma. Clin Exp Dermatol 2000;25:44-7.  Back to cited text no. 2      
3.Fernandez MA, Delgado V. Familial multiple cutaneous leiomyomas. Dermatology 1995;191:295-8.  Back to cited text no. 3      
4.Martinez MA, Glaser B, Chuang GS. Germline fumarate hydratase mutations in families with multiple cutaneous and uterine leiomyomata. J Invest Dermatol 2003;121:741-4.  Back to cited text no. 4      
5.Cislo M, Szepietowski F, Wasik JC. Familial cutaneous and uterine leiomyomas: case report. Acta Derm Venerol Croat 2003;11:212-6.  Back to cited text no. 5      
6.Cairey S, Salard D, Algros MP, Laurent R. Multiple familial cutaneous leiomyoma. Ann Dermatol Venereol 2003;130:1017-20.  Back to cited text no. 6      
7.Sahoo B, Radotra BD, Kaur I, Kumar B. Zosteriform pilar leiomyoma. J Dermatol 2001;28:759-61.  Back to cited text no. 7      
8.Archer CB, Whittaker S, Greaves MW. Pharmacological modulation of cold induced pain in cutaneous leiomyomata. Br J Dermatol 1988;118:255-60.  Back to cited text no. 8      
9.Laporte M, Achten G, Gheeraert P. Multiple leiomyoma treated with nifedipine: association with an astrocytoma. Dermatologica 1985;171:486-90.  Back to cited text no. 9      
10.Venencie PY, Bigel P. Multiple cutaneous leiomyomatosis. Treatment with phenoxybenzamine. Ann Dermatol Venereol 1982;109:819-20.  Back to cited text no. 10      
11.Alam M, Rabinowitz AD, Engler DE. Gabapentin treatment of multiple piloleiomyoma related pain. J Am Acad Dermatol 2002;46:S27-9.  Back to cited text no. 11      
12.Christenson LJ, Smith K, Arpey CJ. Treatment of multiple cutaneous leiomyomas with C02 laser ablation. Dermatol Surg 2000;26:319-22.  Back to cited text no. 12      


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]



 

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