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Acquired Hypertrichosis Lanuginosa: A Rare Cutaneous Paraneoplastic Syndrome

Diakonessenhuis Utrecht, Department of Internal Medicine, Division of Medical Oncology, Utrecht, the Netherlands

A 57-year-old woman with a history of carcinoma of the breast presented with fine, lanugo-type hair on the cheeks and chin. Infiltrating ductal carcinoma of the right breast (pT1N1M0, estrogen receptor positive, and progesterone receptor positive) had been diagnosed 5 years before presentation and was treated by modified radical mastectomy with total axillary lymph node dissection and adjuvant tamoxifen. One year after surgery a local recurrence was excised and irradiated and tamoxifen was replaced with letrozole. In the subsequent 2 years, she developed metastatic disease in subclavicular and contralateral axillary lymph nodes, bone, and liver for which she received six cycles of cyclophosphamide, methotrexate, and fluorouracil followed by several hormone therapies. Because of progressive disease 6 months after the completion of therapy, she was treated with six cycles of doxorubicin. Three months thereafter she presented with excessive fine, unmedullated hair on her cheeks and chin (Figs 1 and 2). In addition, progression of supra- and infraclavicular and contralateral axillary lymphadenopathy were noted. No signs of virilization or hirsutism were seen and axillary and pubic hair appeared normal. Laboratory investigations, including extensive endocrinologic studies, did not reveal any abnormalities other than a previously recognized elevation in liver function tests, most probably caused by liver metastases. Hypertrichosis lanuginosa acquisita was diagnosed. Computed tomography scan of the thorax confirmed progression of lymphangitis carcinomatosa and clavicular and contralateral axillary lymphadenopathy. With docetaxel and local radiotherapy, a partial response was obtained and coincided with disappearance of the hypertrichosis. Our patient passed away within 2 years after diagnosing this rare cutaneous paraneoplastic syndrome.

View larger version (88K): [in this window] [in a new window] [PowerPoint Slide for Teaching]   Fig 1.

View larger version (73K): [in this window] [in a new window] [PowerPoint Slide for Teaching]   Fig 2.

Acquired hypertrichosis lanuginosa (AHL) is a rare cutaneous paraneoplastic syndrome characterized by the development of excessive, long, fine, nonpigmented hairs, preferentially located on the face.^2,3 So far, only 56 cases of AHL have been reported,^2-7 of which five cases were associated with breast carcinoma. AHL is most commonly associated with adenocarcinoma of the lung and colon (27% and 24%, respectively^1,4,5), and is also described in other solid tumors of the pancreas, uterus, ovary, kidney, bladder, and gallbladder in addition to leukemia and Ewing's sarcoma. The syndrome is more often observed in women than in men (70% and 30%, respectively)^3,4 and usually occurs when the tumor is disseminated.⁸ In women, AHL should be differentiated from hirsutism, a subclass of hypertrichosis defined by excessive androgen-sensitive hair growth distributed in an adult male pattern.⁹ The differential diagnosis includes other causes of acquired hypertrichosis, such as anorexia nervosa, AIDS, brain injury, hypothyroidism, polymyositis, porphyria cutanea tarda, primary biliary cirrhosis, shock, systemic lupus erythematosus, and specific drugs (corticosteroids, cyslosporine, diazoxide, interferon-, minoxidil, penicillin, phenytoin, spironolactone, streptomycin, and zidovudine).^2,3,5,10-12 In these associated cases, hypertrichosis is characterized by terminal-type hair, which is mostly coarse and dark.^2,8

Our patient had normal adrenal and thyroid function and was not using any of the aforementioned drugs. She had been treated with doxorubicin a few months before presentation with hypertrichosis. Gaffney et al¹² suggest that cytotoxic chemotherapy itself might induce AHL, but in the majority of reported cases no association has been described. In our patient the lanugo-type hair disappeared during docetaxel treatment together with regression of metastasis.

The mechanism responsible for excessive lanugo-type hair growth is still unclear.^2,3 It has been suggested that an as yet unidentified tumor-derived humoral factor may lead to a prolongation of the anagen phase (the active growth phase) of vellus hair follicles, resulting in hypertrichosis.^3,13

AHL is commonly associated with symptoms like burning glossitis, papillary hypertrophy of the tongue, taste or smell disturbances, diarrhea, lymphadenopathy, and weight loss.^4,14 Our patient presented with abnormal hair growth and progressive lymphadenopathy; however, no other clinical features were noted.

Although higher levels of gonadotropin, carcinoembryonic antigen, and urinary cortisol were found in some patients, no consistent biochemical abnormalities have been reported.^2,8

The occurrence of AHL is usually associated with a poor prognosis, as most patients succumb within 3 years of initial diagnosis.³ Treatment of AHL is focused on eradication of the cause, which frequently results in its resolution. In our patient, a partial regression of metastasis was noted during second-line chemotherapy together with the disappearance of hypertrichosis.

AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST

The authors indicated no potential conflicts of interest.

REFERENCES

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7. Lorette G, Maruani A: Images in clinical medicine: Acquired hypertrichosis lanuginosa. N Engl J Med 354:2696, 2006[Free Full Text]

8. Hovenden AL: Acquired hypertrichosis lanuginosa associated with malignancy. Arch Intern Med 147:2013-2018, 1987[Abstract/Free Full Text]

9. Vulink AJ, Vermes I, Kuijper P, et al: Steroid cell tumour not otherwise specified during pregnancy: A case report and diagnostic work-up for virilisation in a pregnant patient. Eur J Obstet Gynecol Reprod Biol 112:221-227, 2004[CrossRef][Medline]

10. Quesada Charneco M, Fernandez Soto ML, Penafiel J, et al: Acquired hypertrichosis lanuginosa in primary biliary cirrhosis. Med Clin (Barc) 103:318, 1994[Medline]

11. Roca-Tey R, Segura Garcia A, Gonzalez Atienza J, et al: Acquired hypertrichosis lanuginosa and polymyositis. Int J Dermatol 32:227-228, 1993[Medline]

12. Gaffney CC, Roberts JT: Hypertrichosis lanuginosa acquisita following cytotoxic chemotherapy. Clin Oncol (R Coll Radiol) 4:267-268, 1992[Medline]

13. Wendelin D, Pope D, Mallory S: Hypertrichosis. J Am Acad Dermatol 48:161-179, 2003[CrossRef][Medline]

14. Samson MK, Buroker TR, Henderson MD, et al: Acquired hypertrichosis languiginosa: Report of two new cases and a review of the literature. Cancer 36:1519-1521, 1975[CrossRef][Medline]

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