Acknowledgements:
I would like to express my very great appreciation to Dr Aditi Mishra for her valuable and constructive suggestions during the planning and development of this research work. Her willingness to give time so generously has been very much appreciated.
I would also like to thank the Department of Pathology of Dhulikhel Hospital for their constant support.
CASE REPORT
BACKGROUND
Leiomyomas were first termed by Virchow in 1854 as “tuberculum dolorosum”.1 Cutaneous leiomyomas are rare, benign tumors that may be sporadic or inherited, originated from smooth muscle fibres and can be associated with various disorders.2It can be categorized into three subtypes depending upon the origin, arrector pili mucles (pilar leiomyoma), smooth muscle of blood vessels (angioleiomyoma) and tunica dartos or labia majora (dartoic or genital leiomyoma).3 cutaneous leiomyomas are painful tumors that are more common in adults than children.4 In 1880, Besnier classified leiomyomas as solitary or multiple.5 When multiple, these can be grouped, linear, segmental, zosteriform or dermatomal pattern.6Generally it affects extremities followed by trunk, face and neck and presents as firm skin colored to reddish brown papules or nodules. It is often associated with pain, spontaneous or induced by cold or pressure.7 The cause of pain is unknown but some suggest it to be due to cutaneous nerve compression, or by local ischemia caused by contraction of smooth muscle fibres of the tumor.7,8
Pilar leiomyomas can be transmitted as autosomal dominant trait and may be associated with uterine leiomyomatosis or renal cell cancer, also known as Reed syndrome or Multiple cutaneous and uterine leiomyomatosis (MCUL) or hereditary leiomyomatosis and renal cell cancer (HLRCC).8,9
OBSERVATION
A 36 year old, married female presented to us with multiple painful skin colored to brownish papules and nodules involving right upper extremity and right side of upper chest for the duration of three years. These lesions initially appeared over right forearm and gradually increased in number to involve right arm and right upper chest.(Figure 1,2) The lesions started as skin colored papules and few of them progressed in size to form a nodule. It was associated with pain, during touch and exposure to cold. Menstrual and bladder history did not show any abnormalities. General physical examination was unremarkable. Cutaneous examination revealed multiple tender skin colored to hyperpigmented firm papules and nodules varying in size from 0.5 cm to 2 cm, distributed in segmental pattern involving right upper extremity and right upper chest (C5 to C8) dermatome. Based upon history and clinical examination we made differential diagnosis of leiomyomas, neurofibromas, and angiolipomas. Routine hematological investigations including urea, creatinine and urine examination did not reveal any abnormalities. However, ultrasound examination of abdomen and pelvis revelead a lesion measuring 19x18x13 mm arising from posterior wall of uterine body suggestive of intramural leiomyoma. Serological tests were negative. Histopathological tests taken from right upper chest and right forearm showed well circumscribed tumor composed of smooth muscles arranged in interlacing fascicles. The tumor cells were spindle shaped with elongated nuclei with blunt ends with abundant amount of fibrillary eosinophilic cytoplasm confirming the diagnosis of pilar leiomyoma.(Figure 3, 4)
Patient was managed symptomatically for pain with Tab Nifedipine 10 mg twice daily and is kept on routine follow up for uterine leiomyoma accordingly.