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.