ARTICLE
Cutaneous leiomyomas are benign neoplasms of smooth muscles that sometimes
occur in a multiple fashion. There are five types of cutaneous leiomyomas
proposed; 1) multiple piloleiomyomas, 2) solitary piloleiomyoma, 3) solitary
genital leiomyoma, 4) solitary angioleiomyoma, and 5) leiomyoma with additional
mesenchymal elements [1]. The origin of both multiple piloleiomyomas and
solitary piloleiomyoma is referred to the arrector pili muscle, that of
solitary genital leiomyoma to the dartoic, vulvar, or mammillary muscle,
and that of solitary angioleiomyoma to the muscle of the vein. We report
a case of multiple piloleiomyomas of ipsilateral and segmental distribution
associated with a solitary angioleiomyoma of the thigh.
Case report
In April 1997, a 58-year-old man presented with tender papules on the
right side of his upper back and the nape of his neck, his right shoulder,
and his right upper arm (Fig.
1). In addition, a tender subcutaneous nodule was noted on his
left thigh. The multiple papules first developed on the right upper back
more than 10 years ago and then gradually spread onto the right side of
the nape of his neck, his right shoulder and his right upper arm. The
nodule on his left thigh, which measured 6 mm in diameter, had appeared
5 years ago. His late mother seemed to have similar papules on her back
in a similar distribution. The patient was otherwise healthy and serologically
normal, in particular, the serum estrogen level was not high. The multiple
papules on the upper back were histologically composed of interlacing
eosinophilic bundles of smooth muscles arranged in a whorled fashion in
the dermis. The tumor cells were slightly wavy in shape, and contained
thin, long and blunt-edged nuclei. A direct connection between the tumor
nest and the arrector pili muscle was evident in the lesion (Figs.
2, 3). The papules were therefore diagnosed as multiple piloleiomyomas.
In contrast, the nodule of his left thigh was a solitary angioleiomyoma
demonstrating a well-circumscribed nodule containing numerous blood vessels
and intervening smooth muscle bundles (Figs.
4, 5). The tumor cells of both lesions were immunohistochemically
positive for alpha-smooth muscle actin.
Discussion
Piloleiomyomas are clinically manifested as solitary or multiple firm,
erythematous, tender nodules or papules. They usually appear on the face,
back and the dorsal aspect of the limbs. A high likelihood of heredity
is indicated in the cases of multiple piloleiomyomas. Koepfler et al.
[2], in 1958, suggested that the multiple form of cutaneous piloleiomyomas
is an inherited disorder transmitted by an autosomal dominant gene with
incomplete penetrance. Examples of familial multiple piloleiomyomas have
also appeared in the literature [3]. In our case, the nevoid character
was suggested by the ipsilaterality and the segmentality of the distribution
of piloleiomyomas and by positive family history. On the other hand, solitary
angioleiomyoma is usually located subcutaneously and does not exceed 4
cm as the maximum diameter. The lower extremities are the most common
site. Pain and tenderness are evoked by most, but not all, angioleiomyomas.
Trauma is considered to be one of the causes,
although our patient did not recall it. Histopathologically, angioleiomyomas
differ from other types of cutaneous leiomyomas in that they are well-circumscribed
and embrace a component of numerous blood vessels. The vessels vary in
size and have muscular walls of varying thickness. On the basis of types
of blood vessels proliferated, angioleiomyomas are subdivided into three
types, namely, a capillary or solid type, a cavernous type, and a venous
type [1]. Our case is classified as an example of the venous type based
upon the histological findings. Piloleiomyomas were often reported to
be associated with leiomyoma of the uterus or other organs [4, 5]. However,
to our knowledge, there is no case of multiple piloleiomyomas associated
with solitary angioleiomyoma reported so far in the literature. This association
is considered to be fortuitous because both leiomyomas are not exceedingly
rare.
REFERENCES
1. Ragsdale BD. Leiomyoma. In: Elder D, Elenitsas R, Jaworsky C, eds.
Lever's Histopathology of the Skin. 8th ed. Philadelphia: Lippincott-Raven,
1997: 955-9.
2. Koepfler-HW, Krafchuk J, Derbes V, et al. Hereditary multiple
leiomyoma of the skin. Am J Hum Genet 1958; 10: 48-52.
3. Fernández-Pugnaire MA. Familial multiple cutaneous leiomyomas.
Dermatology 1995; 191: 295-8.
4. Reed WB, Walker R, Horowitz R. Cutaneous leiomyomata with uterine
leiomyomata. Acta Derm Venereol 1973; 53: 409-16.
5. Suenaga Y, Yamamoto O, Nishio K. A case of multiple piloleiomyoma
associated with submucosal tumor of the stomach. Sangyo Ika Daigaku
Zasshi 1987; 9: 321-7.
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