Malignant Acanthosis Nigricans, Sign of Leser–Trélat, and Tripe Palms

Abstract

G.Y. Wu et al. (eds.), Atlas of Dermatological Manifestations of Gastrointestinal Disease, DOI 10.1007/978-1-4614-6191-3_32, © Springer Science+Business Media New York 2013 Clinical signs and features include: Malignant acanthosis nigricans, sign of Leser–Trelat, and • tripe palms are often found together Malignant acanthosis nigricans is rare: in total, world• wide, only 1,000 reported cases [ 1 ] Involves symmetric hyperpigmented plaques with – variable amounts of epidermal hypertrophy, creating a velvety texture; ranges in color from yellow to gray/ black; often has overlying scaling/papillomas (if extensive then cutaneous papillomatosis) Progressive—generally advances with cancer, begins – with increased pigmentation and advances to hypertrophy with accentuation of the skin lines; maybe associated with pruritus Most commonly found on fl exural areas, posterior neck, – or mucosally (particularly the mouth) ( see Fig. 32.1 ) Tripe palms: <100 cases reported, men affected more • commonly than women [ 1 ] Condition involves hypertrophy of the palms and soles – with papillations creating a velvety, rugose appearance often with a yellow hue and exaggeration of the skin lines (they can become distorted with extensive epidermal hyperkeratosis), associated with clubbing and pruritus Leser-Trelat: rare, <100 cases reported worldwide [ • 1 ] Numerous eruptive seborrheic keratoses on trunk and – extremities, possible in Christmas tree like pattern, often with pruritus Controversial whether this is a valid sign as both – malignancies and seborrheic keratoses are common in the elderly (see Fig. 32.1 ) In general about 60% of the time these conditions precede • diagnosis of cancer, 10% are with diagnosis, and 30% follow diagnosis [ 1 ] Associated with gastric, intestinal, pulmonary, uterine, • liver, ovarian, renal, and lymphoproliferative neoplasms; malignancies are often aggressive with poor prognosis The differential diagnosis should include: Benign acanthosis nigricans • Nonmalignant causes of seborrheic keratosis: HIV, trans• plants, erythroderma, acromegaly, age Keratoderma • Pathogenesis of this disease is unclear but hypotheses include: May be related to release of growth factors (e.g., growth • factors tumor necrosis factor alpha [TNFa ] and epidermal growth factor [EGF]) from the neoplasm that act on receptors, particularly EGF receptor [ 1 ] Histopathological features include: Malignant acanthosis nigricans: hyperkeratosis and • papillomatosis with minimal or slight and irregular acanthosis; often alternating acanthosis with atrophy; none or occasionally only slightly increased basal layer hyper pigmentation (see Fig. 32.2 ) Tripe palms: hyperkeratosis, acanthosis, and papillomato• sis; increased dermal mucin with an increased number of mast cells can be seen Leser–Trelat, seborrheic keratosis: varying hyperkerato• sis, papillomatosis, acanthosis often with horn pseudocysts (see Fig. 32.3 ) Malignant Acanthosis Nigricans, Sign of Leser–Trelat, and Tripe Palms