|LETTER TO THE EDITOR
|Year : 2021 | Volume
| Issue : 3 | Page : 80-82
Segmental leiomyoma: A report of two cases
Arumugam Iswarya1, Palaniappan Vijayasankar1, Kaliaperumal Karthikeyan1, Vijayaraghavan Sriram2
1 Department of Dermatology, Venereology, and Leprosy, Sri Manakula Vinayagar Medical College and Hospital, Puducherry, India
2 Department of Pathology, Sri Manakula Vinayagar Medical College and Hospital, Puducherry, India
|Date of Submission||15-Jun-2021|
|Date of Decision||05-Aug-2021|
|Date of Acceptance||09-Aug-2021|
|Date of Web Publication||29-Sep-2021|
Dr. Kaliaperumal Karthikeyan
Department of Dermatology, Venereology, and Leprosy, Sri Manakula Vinayagar Medical College and Hospital, Puducherry.
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Iswarya A, Vijayasankar P, Karthikeyan K, Sriram V. Segmental leiomyoma: A report of two cases. Turk J Dermatol 2021;15:80-2
| Introduction|| |
Leiomyomas are a rare form of benign smooth muscle tumors. The most common site of occurrence is the uterus (95%), followed by the skin. Cutaneous leiomyoma accounts for 75% of the extrauterine leiomyomas. The pathogenesis of cutaneous leiomyoma is unknown. Based on their site of origin, they are classified as piloleiomyoma, angioleiomyoma, and genital leiomyoma. Piloleiomyoma is the most common variant arising from the arrector pili muscle. Segmental leiomyomas represent the mosaic manifestation of cutaneous leiomyomas. We present two cases of Type 1 segmental leiomyoma, which are rare and unique.
| Case Report 1|| |
A 27-year-old man presented with a history of multiple intermittently painful skin-colored raised lesions limited to the right side of the back region for 3 years. The patient experienced increased pain whenever lesions came in contact with cold water. Cutaneous examination revealed multiple, firm, tender, skin-colored to pale red papules and nodules. The lesions are grouped over the right side of the posterior trunk in a segmental distribution [Figure 1]a. Incisional skin biopsy revealed a well-circumscribed lesion composed of spindle cells arranged in a whorled pattern in the deep dermis [Figure 2]a. Masson trichrome stained the smooth muscle bundles in the dermis as red color [Figure 2]b. He was started on oral nifedipine and advised regular follow-up.
|Figure 1: (a, b) Multiple skin-colored to red-colored papules and nodules in a segmental distribution|
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|Figure 2: (a) HPE 10×—A circumscribed tumor with fascicles of smooth muscle bundles. (b) Masson trichrome stain (4×)—Red color highlights the smooth muscle bundles in the dermis|
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| Case Report 2|| |
A 26-year-old man presented with painful raised lesions over his left upper arm since 1 year. He experienced an increased intensity of pain upon friction. Cutaneous examination showed three well-defined, firm, tender, skin-colored to erythematous nodules of varying sizes. These nodules were present over the antero-medial aspect of the upper left arm [Figure 1b]. Based on the distinct clinical and pathological findings, he was diagnosed to have segmental leiomyoma and the lesions were excised.
| Discussion|| |
Piloleiomyomas commonly manifest between the first and third decades of life with no gender predilection. It clinically presents as skin-colored to red-brown dermal papules or nodules, mainly distributed over the proximal extremities, followed by trunk, face, and neck. The pain in leiomyoma may either be spontaneous or induced, secondary to cold, pressure, emotional stress, and friction. The pain may be of burning, stabbing, or pinching in nature which could be attributed to pressure of the tumor on the underlying nerves or smooth muscle fiber contraction and infiltration of mast cells. Pain is more commonly observed in diffuse and segmental pattern.
Piloleiomyomas can either be solitary or multiple. Multiple leiomyomas are more common and are termed as leiomyomatosis. When the leiomyoma lesions are more than 5000, it is known as myomatosis cutis miliaris. Usually, multiple piloleiomyomas are commonly observed between 10 and 30 years of age, whereas solitary piloleiomyomas are seen with advancing age. Multiple piloleimyomas are transmitted in an autosomal dominant fashion, which may be associated with uterine leiomyomas (Reed’s syndrome/MCUL—multiple cutaneous and uterine leiomyomatosis) and hereditary leiomyomatosis and renal cell carcinoma (HLRCC). A predisposing factor to MCUL and HLRCC is the gene encoding fumarate hydratase mutation on chromosome 1q42.3–43. Multiple lesions in the leiomyoma may present in different patterns, such as disseminated/diffuse, blaschkoid, and segmental/zosteriform., The other reported forms are grouped, linear, and symmetrical pattern.
Segmental leiomyomas are of two types: (i) Type 1 and (ii) Type 2. Both are inherited as autosomal dominant pattern of inheritance. The salient features of the two types have been summarized in [Table 1]. Cutaneous leiomyomas have to be differentiated from other painful cutaneous tumors such as the dermatofibroma, angiolipoma, eccrine spiradenoma, schwannoma, and neuromas. Other painful conditions in segmental distribution are given in [Table 2].,,,,,
The piloleiomyoma is histopathologically characterized by poorly demarcated interlacing bundles of smooth muscle cells with varying amounts of intermingling collagen fibers in a low-power field. The muscle fibers are usually straight, with centrally located long, thin, “eel-like nuclei.” The histopathological differential diagnosis includes other spindle tumors such as dermatofibroma, leiomyosarcoma, neurofibroma, and schwannoma. Masson trichome stain and immunohistochemistry are helpful in differentiating piloleiomyomas from other spindle cell tumors.,
The treatment aspects for leiomyomas are not satisfactory. Avoiding trauma and exposure to cold can prevent the pain in leiomyoma. Surgical excision can be done when lesions are few; however, recurrence is common. In case of multiple lesions, where excision is not possible, liquid nitrogen cryotherapy, electrocoagulation, and CO2 laser ablation can be considered. Drugs such as nifedipine, doxazosin, gabapentin, antidepressants, and topical 9% hyoscine hydrobromide are helpful in relieving the pain. Recently, botulinum toxin has been used for pain management.
To conclude, leiomyoma cutis Type I is a rare but painful tumor that can affect the quality of life. The cornerstone of management is planning the treatment appropriate to the patient.
Arumugam Iswarya has contributed to the content, literature search, manuscript preparation, and submitted the article.
Palaniappan Vijayasankar has contributed to the design and literature search of the article.
Kaliaperumal Karthikeyan has planned the concept, manuscript preparation, manuscript editing, manuscript review, and is the guarantor of this article.
Vijayaraghavan Sriram has contributed to the histopathology part.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]
[Table 1], [Table 2]