Sign of Leser-Trelat Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 18, 2012
 

History

A complete medical history should be obtained.

The sudden eruption of multiple seborrheic keratoses is diffusely evident on any body surface, most strikingly on the trunk. The face, the neck, the axillae, the groin, and the extremities may also be affected. The abruptness of the eruption is noteworthy; it should not be confused with generalized seborrheic keratoses of gradual onset. Nevertheless, for the purposes of diagnosis, precise quantification of the length of the eruptive period and the number of seborrheic keratoses is lacking. In the author's experience, many but not all elderly patients are able to provide a clear history.

Pruritus may be the only symptom of the sign of Leser-Trélat. Pruritus occurs in almost one half of patients, but it may also occur with eruptive seborrheic keratoses unassociated with malignancy.

Seborrheic keratoses are common findings. However, the early appearance of these keratoses in individuals aged 0-30 years and their eruptive nature are not common. In a few families, seborrheic keratoses were autosomal dominant, and the lesions were rarely present at birth or in individuals aged 10-30 years. Members of a family from India had multiple seborrheic keratoses since birth.[26] In another family, a mother and 3 daughters had generalized seborrheic keratoses that developed in their teens and 20s.[27] Seven families had multiple seborrheic keratoses. In 3, the investigators were able to trace the tumors through 3 generations.[28] In 3 generations of a family, multiple seborrheic keratoses appeared during or near puberty.[29]

Rarely, overlap may occur between the body areas involved by pulmonary adenocarcinoma and the seborrheic keratoses of the sign of Leser-Trélat.[30]

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Physical

A complete physical examination should include an examination of the lymph nodes; the liver; the spleen; the prostate in men; the rectum; the breasts; and the pelvic area, including the ovaries, in women.

Warty nodules that proliferate cutaneously are evident. Each nodule usually appears distinct, with its characteristic warty stuck-on morphologic features and brownish black or yellowish coloration. The author recommends that a biopsy specimen be obtained to confirm the diagnosis.

The eruption may appear in a Christmas tree or splash pattern, although these patterns are not specific for this sign and may occur with eruptive seborrheic keratoses unassociated with malignancy. Note the image below.

Middle-aged woman with a Christmas tree-like distrMiddle-aged woman with a Christmas tree-like distribution of seborrheic keratoses on her back with no evidence of internal cancer.

Malignant acanthosis nigricans is frequently evident with the sign of Leser-Trélat. Malignant acanthosis nigricans occurs in about 35% of patients. Florid cutaneous papillomatosis is less common.[31]

Other rarely associated paraneoplastic entities include the following:

  • Acquired hypertrichosis lanuginosa[32]
  • Acquired ichthyosis[33]
  • Cowden disease[34]
  • Tylosis[11]
  • Acrokeratosis of Bazex[35]
  • Tripe palms[36]
  • Simultaneous paraneoplastic manifestations, including both ichthyosis acquisita and Bazex syndrome[37]

The sudden eruption and the rapid increase in the number and the size of freckles, along with seborrheic keratoses, may occur.

A proliferation of papillary angiomas may occur.

Eruptive capillary angiomas have been described as a paraneoplastic sign,[38] although this sign is not widely accepted. The author has observed 1 patient with eruptive capillary angiomas and a visceral malignancy in whom no eruptive seborrheic keratoses were present.

The sign of Leser-Trélat, malignant acanthosis nigricans, and florid cutaneous papillomatosis are 3 paraneoplastic entities that constitute a continuum. These conditions more commonly accompany each other than any other paraneoplastic disorder. Florid cutaneous papillomatosis is a sudden and widespread eruption of what appears to be common viral warts on clinical examination. Acanthosis nigricans is a widespread dermatosis with a verrucous tendency anywhere on the surface of the body, especially around the lips, the eyes, and the axillae.

Patients with erythroderma may develop a widespread eruption of multiple seborrheic keratoses without associated malignancies. In the author's experience, these patients usually have atopic dermatitis complicated by erythroderma.

The sudden eruption of hundreds of typical seborrheic keratoses confined to the skin of the breasts and a small area below them is described in association with pregnancy.

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Causes

The cause of the sign of Leser-Trélat is unknown. It may be due to the same unknown factors that induce malignant acanthosis nigricans. These paraneoplastic syndromes are probably directly caused by their underlying neoplasms, presumably from a tumor-secreted growth factor; however, evidence of this concept is lacking. The sign of Leser-Trélat is usually linked with visceral cancer, but it has also been described in metastasized malignant melanoma.[39]

The sign of Leser-Trélat may be linked with cytokine alterations (see Pathophysiology).

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Takeji Nishikawa, MD  Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Warren R Heymann, MD  Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Middle-aged woman with a Christmas tree-like distribution of seborrheic keratoses on her back with no evidence of internal cancer.
 
 
 
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