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, C.A. Seldenrijk§ and M.A.M. van Steensel– Departments of Internal Medicine, *Dermatology, Obstetrics and Gynaecology, Gastroenterology and §Pathology, St Antonius Hospital, PO Box 2500, 3430 EM Nieuwegein, the Netherlands –Department of Dermatology, University Medical Hospital Maastricht, the Netherlands
Summary
Correspondence Acquired hypertrichosis lanugo-type or hypertrichosis lanuginosa acquisita (HLA) P.H.T.J. Slee. is often associated with metabolic and endocrine disorders and use of certain E-mail: [email protected] 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 Accepted for publication 13 August 2007 women and in men. Sometimes HLA occurs concurrent with acanthosis nigri- cans, papillary hypertrophy of the tongue, and glossitis. The predominance of Key words female cases is striking. Malignancy-associated HLA seems to occur especially in acquired hypertrichosis lanugo-type, hypertrichosis the age group 40–70 years. In women with HLA the most frequent malignancy lanuginosa acquisita, malignancy, paraneoplastic is colorectal cancer, followed in order by lung cancer and breast cancer; in men Conflicts of interest lung cancer is the malignancy most frequently associated with HLA, followed by None declared. colorectal cancer. In 3 years we saw 10 patients with HLA, in whom the malig- nancy 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 Lanugo hairs physiologically grow in utero, contain no that may present itself in various clinical patterns. Recognition medulla, are not pigmented, are long and are shed during the of the type of hypertrichosis is very important for the diagno- last months of pregnancy up to the first months after birth.1 sis and therapy of the underlying disease. In contrast to hirsutism, lanugo-type hypertrichosis does not show a gender-specific distribution pattern. Patients with Hypertrichosis acquired hypertrichosis lanugo-type or hypertrichosis lanugin- osa acquisita (HLA) grow lanugo-type hair near their eye- Hypertrichosis may involve lanugo hair, vellus hair or terminal brows and on their forehead, ears and nose. Some patients hair.1 Lanugo hairs are long, thin and unpigmented, like have extensive involvement that includes the extremities, axil- wool. Vellus or intermediate hairs are short, unpigmented lae and trunk, but the palmoplantar, suprapubic and genital hairs; they are variably medullated. Terminal hairs contain a areas are rarely involved.2 medulla, and are longer, thick, and pigmented. These hairs Hypertrichosis lanuginosa may occur as congenital or are involved in hirsutism, which is defined as an adult male- acquired forms. Only the lanugo-type of hypertrichosis will pattern hair overgrowth in children or women. Most authors be discussed here. For the other types excellent reviews are consider hirsutism as a subclass of hypertrichosis. This type of available.1 hypertrichosis appears predominantly on body areas with androgen-sensitive follicles, e.g. the chest, beard and mous- Hypertrichosis lanuginosa congenita tache regions. Hirsutism occurs as a result of androgen activ- ity: either as a consequence of elevated systemic androgen Hypertrichosis lanuginosa congenita or congenital lanugo levels, or by means of local elevated sensitivity for androgens, hypertrichosis (CLH) often represents a normal variation in or a combination of both mechanisms. In hirsutism endocrine premature neonates that have not gone through the in utero evaluation is warranted. shedding phase because of premature birth, but is rarely