Paraneoplastic hypertrichosis lanuginosa acquisita: uncommon or overlooked?

TOPICAL REVIEW DOI 10.1111/j.1365-2133.2007.08253.x Paraneoplastic hypertrichosis lanuginosa acquisita: uncommon or overlooked? P.H.T.J. Slee, R.I.F. van der Waal,* J.H. Schagen van Leeuwen, R.A. Tupker,* R. Timmer,à C.A. Seldenrijk§ and M.A.M. van Steensel– Departments of Internal Medicine, *Dermatology, Obstetrics and Gynaecology, àGastroenterology and §Pathology, St Antonius Hospital, PO Box 2500, 3430 EM Nieuwegein, the Netherlands –Department of Dermatology, University Medical Hospital Maastricht, the Netherlands Correspondence P.H.T.J. Slee. E-mail: [email protected] Accepted for publication 13 August 2007 Key words acquired hypertrichosis lanugo-type, hypertrichosis lanuginosa acquisita, malignancy, paraneoplastic Conflicts of interest None declared. Summary Acquired hypertrichosis lanugo-type or hypertrichosis lanuginosa acquisita (HLA) is often associated with metabolic and endocrine disorders and use of certain drugs. The occurrence of HLA with malignancy was first noted in 1865, and it has since been described in 56 patients as a paraneoplastic syndrome both in women and in men. Sometimes HLA occurs concurrent with acanthosis nigri- cans, papillary hypertrophy of the tongue, and glossitis. The predominance of female cases is striking. Malignancy-associated HLA seems to occur especially in the age group 40–70 years. In women with HLA the most frequent malignancy is colorectal cancer, followed in order by lung cancer and breast cancer; in men lung cancer is the malignancy most frequently associated with HLA, followed by colorectal cancer. In 3 years we saw 10 patients with HLA, in whom the malignancy was usually metastasized. Only one patient had local disease; after removal of the primary tumour it took 2 years before the lanugo hair recurred. The aeti- ology of the syndrome is not clear: no specific hormonal or biochemical abnor- malities have been identified as yet. The difference between hirsutism and lanugo-type hypertrichosis is discussed. It is stressed that the appearance of lanugo-type hypertrichosis in body areas previously perceived by patients as ‘hairless’ is highly indicative of internal malignancy. Hypertrichosis, defined as excessive hair growth, is a problem that may present itself in various clinical patterns. Recognition of the type of hypertrichosis is very important for the diagno- sis and therapy of the underlying disease. Hypertrichosis Hypertrichosis may involve lanugo hair, vellus hair or terminal hair. 1 Lanugo hairs are long, thin and unpigmented, like wool. Vellus or intermediate hairs are short, unpigmented hairs; they are variably medullated. Terminal hairs contain a medulla, and are longer, thick, and pigmented. These hairs are involved in hirsutism, which is defined as an adult male- pattern hair overgrowth in children or women. Most authors consider hirsutism as a subclass of hypertrichosis. This type of hypertrichosis appears predominantly on body areas with androgen-sensitive follicles, e.g. the chest, beard and mous- tache regions. Hirsutism occurs as a result of androgen activ- ity: either as a consequence of elevated systemic androgen levels, or by means of local elevated sensitivity for androgens, or a combination of both mechanisms. In hirsutism endocrine evaluation is warranted. Lanugo hairs physiologically grow in utero, contain no medulla, are not pigmented, are long and are shed during the last months of pregnancy up to the first months after birth. 1 In contrast to hirsutism, lanugo-type hypertrichosis does not show a gender-specific distribution pattern. Patients with acquired hypertrichosis lanugo-type or hypertrichosis lanuginosa acquisita (HLA) grow lanugo-type hair near their eye- brows and on their forehead, ears and nose. Some patients have extensive involvement that includes the extremities, axil- lae and trunk, but the palmoplantar, suprapubic and genital areas are rarely involved. 2 Hypertrichosis lanuginosa may occur as congenital or acquired forms. Only the lanugo-type of hypertrichosis will be discussed here. For the other types excellent reviews are available. 1 Hypertrichosis lanuginosa congenita Hypertrichosis lanuginosa congenita or congenital lanugo hypertrichosis (CLH) often represents a normal variation in premature neonates that have not gone through the in utero shedding phase because of premature birth, but is rarely Ó 2007 The Authors Journal Compilation Ó 2007 British Association of Dermatologists • British Journal of Dermatology 2007 157, pp1087–1092 1087

Paraneoplastic hypertrichosis lanuginosa acquisita: uncommon or overlooked?

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