C. Hypertrichosis lanuginosa acquisita
A. Colonoscopy and chest radiograph
This interesting patient obviously suffers from excessive hair growth, which is most broadly deemed “hypertrichosis.” This phenomenon may involve lanugo hair, vellus hair, or terminal hair. Lanugo hairs are thin, soft, and unpigmented, similar to the fine hair normally found on a newborn. Terminal hairs are longer, thicker, and pigmented. Vellus hairs are somewhere in between the latter two hair types. In this case, since the type of excess hair resembles lanugo, the patient has hypertrichosis lanuginosa. Because the condition was not present at birth, but rather acquired later in life, it can be further classified as hypertrichosis lanuginosa acquisita (HLA). HLA has long been considered to be a paraneoplastic disorder.[1,2] Congenital hypertrichosis lanuginosa, which typically manifests as very widespread lanugo hair present at birth, may be due to prematurity or may be an autosomal dominant genodermatosis. Hirsutism, a subset of hypertrichosis, is typically characterized by the development of coarse terminal hairs in body areas with androgen-responsive hair follicles (eg, the chest, beard and mustache areas, and upper back). The development of hirsutism generally requires endocrinologic evaluation.
HLA was first reported by Turner in 1865 in a woman with breast cancer. Since that initial report, about 60 cases of HLA have appeared in the readily searchable medical literature. Women outnumber men by a 3 to 1 ratio and the mean age of onset is 54. Excessive lanugo hair growth may occur from several years before the primary associated tumor is identified until up to 5 years after the diagnosis of cancer has been established. Paraneoplastic HLA typically progresses in a craniocaudal direction, and usually spares the palms, soles, and genitalia. HLA may be accompanied by acanthosis nigricans, palmoplantar hyperkeratosis, and glossitis, as well as by disturbances of taste and smell, diarrhea, and weight loss. Patients with HLA often present with either symptomatic or occult metastatic disease, leading to an overall dismal prognosis. Successful antineoplastic therapy, however, has led to regression of hair growth. Absent hormonal abnormalities or drug ingestion, this association is so strong that the occurrence of lanugo-type hypertrichosis at skin sites previously perceived as being “hairless” should prompt a comprehensive evaluation for internal malignancy. Medications occasionally associated with HLA include: acetazolamide(Drug information on acetazolamide), cyclosporin A, diazoxide(Drug information on diazoxide), penicillamine(Drug information on penicillamine), phenytoin, prednisone(Drug information on prednisone)/prednisolone, and streptomycin(Drug information on streptomycin).
HLA has appeared in conjunction with a widely disparate group of internal cancers; however, lung and colorectal neoplasms are clearly the most common culprits. In patients who present with HLA without localized symptoms, in addition to a detailed history and meticulous physical examination, a chest radiograph and colonoscopy are mandatory. Because of its association with breast cancer, a mammogram would also be advisable in female patients who have not had this test done in a year or more. A variety of abnormal laboratory values have been noted in some case reports (eg, elevated levels of carcinoembryonic antigen, serum calcium and gastrin, and urinary cortisol; and low serum testosterone); however, these findings are far from consistent and should not be considered pathognomonic for this diagnosis.[6,10,11]
The precise pathogenesis of HLA is uncertain, although theoretically the underlying neoplasm likely produces one or more cytokines which act as growth factors and hair follicle stimulants.[4,10]
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2. Samson MK, Buroker TR, Henderson MD, et al. Acquired hypertrichosis lanuginosa: Report of two new cases and a review of the literature. Cancer. 1975;36:1519-1521.
3. Turner M. Case of a woman whose face and body in two or three weeks time became covered with a thick crop of short and white downy hair. Med Time Gaz. 1865;2:507.
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11. Toyoki Y, Satoh S, Morioka G, et al. Rectal cancer associated with acquired hypertrichosis lanuginosa as a possible cutaneous marker of internal malignancy. J Gastroenterol. 1998;33:575-577.