Case for diagnosis

Abstracts

O piloleiomioma é uma neoplasia benigna originada do músculo eretor do pelo. Atinge adultos jovens, sem predileção por sexo. As lesões podem ser solitárias ou múltiplas, acometem mais frequentemente as extremidades e costumam apresentar dor espontânea ou após estímulos físicos. Descreve-se um caso de piloleiomioma múltiplo unilateral em paciente jovem do sexo feminino, com queixa de prurido nas lesões.

Adulto; Leiomiomatose; Músculo liso; Neoplasias cutâneas

WHAT IS YOUR DIAGNOSIS ?

Case for diagnosis: (Unilateral multiple piloleiomyoma)

Luciana Maria Leão Parreira^I; Juliana Muggiati Sípoli^II; Ana Maria da Cunha Mercante^III; Raquel Leão Orfali^IV; Jacob Levites^V

^ISpecialist by the Brazilian Society of Dermatology, Hospital Heliópolis - Sao Paulo(SP), Brazil

IISpecialist by the Brazilian Society of Dermatology, Hospital Heliópolis - Sao Paulo(SP), Brazil

IIIPathologist of Hospital Heliópolis - Sao Paulo(SP), Brazil. MSc in Sciences, Hospital do Câncer de São Paulo (SP), Brazil

IVAttending physician of the dermatology service of the Hospital Heliópolis - Sao Paulo (SP), Brazil. MSc in Sciences, Faculdade de Medicina da Universidade de São Paulo (FMUSP) – Sao Paulo (SP), Brazil

VHead of the dermatology service of the Hospital Heliópolis. MSc in Sciences, Universidade Federal de São Paulo (UNIFESP) – Sao Paulo (SP), Brazil

Mailing Address

ABSTRACT

Piloleiomyoma is a benign neoplasm arising from the erector pilorum muscle in the skin. It occurs in young adults of both genders. Lesions can be single or multiple and more frequently involve extremities. Pain may occur spontaneously or after physical stimulation. We describe a case of unilateral multiple piloleiomyoma in a young woman, complaining of itching lesions.

Keywords: Adult; Leiomyomatosis; Muscle, smooth; Skin neoplasms

DISEASE BACKGROUND

White, female, 21 year-old patient presented with a painful to trauma, itchy, initially single lesion for two and a half years, located on the outside of the left lower limb. Throughout the two years new grouped lesions on the left thigh, left breast and left arm emerged. The patient did not present any co-morbidity, denied using any medication as well as a family history of a similar clinical picture. Her mother had been submitted to a hysterectomy due to a uterus leiomyoma. Physical examination revealed multiple skin color and other brownish-red papules on the left breast and arm (Figure 1), left thigh and lower limb (Figure 2). Histopathology with hematoxylin eosin (HE) staining revealed a well circumscribed, benign mesenchymal neoplasm comprised of fusiform cell bundles, without atypia (Figure 3). An immunohistochemical study with anti-smooth muscle actin and desmin antibodies was performed in order to characterize the origin of cells. There was a cytoplasm marker for both antibodies, confirming the smooth muscle origin of the neoplasm, allowing for the diagnosis of piloleiomyoma. Trans-vaginal pelvic ultrasound was normal as was blood work.

Treatment with 25mg a day, oral hidroxizine was initiated to relieve itching.

COMMENTS

Skin leiomyomas are divided into three categories: piloleiomyomas, originating from erector pilorum muscle, angioleiomyomas originating from vascular smooth muscle and dartoid leiomyomas from the dartos muscles of genitals, areola and nipple.¹ The piloleiomyoma is a benign tumor of the skin described originally by Virchow (1854). ¹ It can affect both genders and any age group, but it is more frequent between the second and fourth decades of life; lesions may be multiple or single. ² They generally affect extremities mainly extensor surfaces, followed by trunk, face and neck. Lesions may be grouped, linear or accompany dermatomes. They present as firm red or brownish-red intradermal nodules fixed to the skin, but not to deep tissue.³

Multiple piloleiomyomas may be painful. Crises of acute pain may occur spontaneously or after physical stimuli or friction. Pain can be worsened by contact with cold objects or even in winter.⁴ Multiple piloleiomyoma, the most common type of skin leiomyoma, is the only one with a multinodular aspect, and can be unilateral, although it tends to be bilateral in most cases described. The combination of familial occurrence multiple piloleiomyoma with uterus leiomyoma is called leiomyomatosis cutis et uteri or Reed Syndrome.⁵ In these cases there may be erythrocytosis resulting from erythropoietic tumor activity.³

Treatment is based on patients' symptoms. Most of the time camouflage with cosmetics and avoiding cold and trauma suffice. Excision of painful or non-esthetical lesions, when in a small number, may be performed. In more extensive and symptomatic cases, drugs that block calcium inflow to smooth muscle, such as nifedipine (initial dose of 30 mg, divided in TID schedule). The alpha adrenergic blocker fenoxibenzamine may be another alternative.² Gabapentin used to relieve chronic neuropathic pain, has been a good treatment option with less side effects.⁴ Cryotherapy and eletrocoagulation have shown to be less effective. Recurrences are frequent, particularly in patients with multiple lesions. It is not known, however, if those are new lesions or result from the growth of partially excised lesions.³

REFERENCES

• 1. Stone JA, Morrison AL. Piloleiomyoma mistaken for postacne scarring. Cutis. 2004;73: 335-7.
• 2. Arrua GGA, Cadena GMM, Filippo AA, Azulay DR, Azulay RD. Piloleiomioma múltiplo: relato de tręs casos tratados com nifedipina. An Bras Dermatol. 1991;66:3003-5.
• 3. Holst VA, Junkins-Hopkins JM, Elenitsas R. Cutaneous smooth muscle neoplasms: clinical features, histologic findings, and treatment options. J Am Acad Dermatol. 2002; 46:477-90.
• 4. Alam M, Rabinowitz AD, Engler DE. Gabapentin treatment of multiple piloleiomyoma-related pain. J Am Acad Dermatol. 2002;46:S27-9.
• 5. Pacheco AP, Ramos AMO, Rolim MLM, Oliveira FM, Lopes JG, Rocha KF. Piloleiomioma múltiplo: relato de caso com diagnóstico diferencial. An Bras Dermatol. 1995;70:43-46.

*

Trabalho realizado no Complexo Hospitalar Heliópolis – São Paulo (SP), Brasil.

Publication Dates

• Publication in this collection
  03 June 2009
• Date of issue
  Apr 2009

History

• Accepted
  20 Oct 2008
• Received
  07 Feb 2007