Florid Cutaneous Papillomatosis

Updated: Jul 19, 2017
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Overview

Background

Florid cutaneous papillomatosis (FCP) is a disorder consisting of the rapid onset of numerous warty papules on the trunk and the extremities that are clinically indistinguishable from viral warts. [1, 2, 3, 4] Florid cutaneous papillomatosis was first described and the name florid cutaneous papillomatosis was coined in 1978 by Schwartz and Burgess. [5]

Florid cutaneous papillomatosis usually occurs in association with malignant acanthosis nigricans and an internal malignancy (see Acanthosis Nigricans). In fact, florid cutaneous papillomatosis is believed to be an obligatory paraneoplastic syndrome in that it has always been associated with an internal malignancy (see Paraneoplastic Diseases and Paraneoplastic Syndromes). The cancer, which is usually intra-abdominal and most often gastric in origin, tends to evolve in parallel with florid cutaneous papillomatosis. The onset of florid cutaneous papillomatosis often becomes evident before or concurrent with the diagnosis of the internal tumor.

In a review of 46 patients, Stieler and Plewig [6] noted that each patient had an underlying cancer. The most common type was gastric adenocarcinoma, which occurred in more than half the patients. One patient had a gastric adenocarcinoma and a second malignancy, namely, breast adenocarcinoma. Other neoplasms that are reported to occur with florid cutaneous papillomatosis include other intra-abdominal tumors, such as cancer of the urinary bladder, biliary ducts, ovaries, and uterus; breast adenocarcinoma; squamous cell carcinoma of the lungs; and non-Hodgkin lymphoma.

Florid cutaneous papillomatosis occurs with signs of internal cancer. Although acanthosis nigricans and the sign of Leser-Trélat (ie, eruptive seborrheic keratoses) are most frequently linked to florid cutaneous papillomatosis, other signs, such as hypertrichosis lanuginosa acquisita, can be present. [7] See Sign of Leser-Trelat for more information on this topic.

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Pathophysiology

The etiology of florid cutaneous papillomatosis is not known, as is the cause of the other 2 eruptive paraneoplastic syndromes that occur with florid cutaneous papillomatosis: malignant acanthosis nigricans and the sign of Leser-Trélat. These 3 paraneoplastic syndromes are probably directly induced by their underlying neoplasms, most likely by means of a tumor-secreted growth factor. [1]

In 1976, Millard and Gould [8] found high levels of immunoreactive human growth hormone in 2 patients with tylosis, one of whom had the sign of Leser-Trélat. In another report, a patient was described as having a small cutaneous melanoma, acanthosis nigricans, the sign of Leser-Trélat, and multiple acrochordons. In this patient, the lesions of the cutaneous paraneoplastic syndrome were stimulated by the epidermal growth factor receptor. [9] Increased epidermal staining for the epidermal growth factor receptor was initially noted in skin specimens of acanthosis nigricans, seborrheic keratoses, and cutaneous papillomas. Both the staining and the urine level of alpha-transforming growth factor markedly decreased after the primary nonmetastatic melanoma was removed. Alpha-transforming growth factor is structurally related to epidermal growth factor but antigenically distinct from it.

Florid cutaneous papillomatosis, malignant acanthosis nigricans, and the sign of Leser-Trélat should be viewed as part of a continuum. These conditions develop by means of a common or similar pathogenic pathway due to an underlying malignancy that produces a factor possibly similar to human epidermal growth factor.

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Epidemiology

Frequency

Florid cutaneous papillomatosis is a rare disease. So far, only about 25 patients have been reported.

Sex

From the limited number of patients described to date, florid cutaneous papillomatosis is almost twice as common in men than in women.

Age

Florid cutaneous papillomatosis is usually diagnosed in individuals aged 53-72 years (mean patient age, 58.5 y).

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Prognosis

Florid cutaneous papillomatosis is an obligate paraneoplastic syndrome, the underlying case is most commonly of gastric origin as documented in the 9 patients described since 2000. [2]

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