AUTHOR AND EDITOR INFORMATION

Section 1 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

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 Pollitzer² first described this condition in 1891. The name florid cutaneous papillomatosis was coined in 1978.³

FCP usually occurs in association with malignant acanthosis nigricans and an internal malignancy (see Acanthosis Nigricans). In fact, FCP 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 FCP. The onset of FCP often becomes evident before or concurrent with the diagnosis of the internal tumor.

In a review of 46 patients, Stieler and Plewig⁴ 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 FCP 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.

FCP occurs with signs of internal cancer. Although acanthosis nigricans and the sign of Leser-Trélat (ie, eruptive seborrheic keratoses) are most frequently linked to FCP, other signs, such as hypertrichosis lanuginosa acquisita, can be present.⁵ See Sign of Leser-Trelat for more information on this topic.

Pathophysiology

The etiology of FCP is not known, as is the cause of the other 2 eruptive paraneoplastic syndromes that occur with FCP: 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 Gould⁶ 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.⁷ 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.

FCP, 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.

Frequency

United States

FCP is a rare disease. So far, only about 25 patients have been reported. Pollitzer² described the first case in 1891.

Sex

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

Age

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

CLINICAL

Section 3 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

History

• The patient usually reports an eruption of wartlike growths. Unusually marked verrucous changes may cause disfigurement of the hands and feet.⁸
• Pruritus is an important sign that is evident in about one half of patients.
• • Pruritus may precede the onset of FCP.
  • It can occur in the affected regions, or it can be generalized.
• The unsightly clinical appearance of FCP may result in social exclusion.⁹

Physical

• Clinically, the papulonodules of FCP are usually indistinguishable from common viral warts.
• • The papulonodules begin on the extremities, particularly on the dorsa of the hands and the wrists.
  • The papules may spread to involve the entire body, including the face.
  • The papulonodules can be as large as 1 cm in diameter, or they can be as small as 2-3 mm in diameter.
• Pruritus may be associated.
• FCP is usually associated with signs of internal cancer. Malignant acanthosis nigricans develops eventually, many times with the sign of Leser-Trélat.
• Marked acanthosis nigricans, especially when associated with either FCP or so-called tripe palms, mandates a thorough search for malignancy.¹⁰
• FCP may be seen in patients with multiple visceral carcinomas.¹¹

Causes

• The cause of FCP is not known.
• FCP may be due to a tumor-secreted growth factor, although, to the author's knowledge, none has been identified (see Pathophysiology).

DIFFERENTIALS

Section 4 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Verrucous mycosis fungoides may occasionally require distinction. Verrucous mucocutaneous acanthosis nigricans of the lips and buccal mucosa may represent, as described by Pentenero et al¹² in 2004, a patient with underlying cancer also beginning to develop FCP.

WORKUP

Section 5 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Histologic Findings

The cutaneous papillomas are uniform with pronounced hyperkeratosis, acanthosis, and papillomatosis. They lack evidence of epidermal vacuolization, parakeratosis, or eosinophilic inclusions suggestive of viral warts; however, patients in one study¹³ did have these findings.

Results of ultrastructural evaluation, immunofluorescence tests, viral serologic tests, and DNA hybridization analyses to detect human papillomavirus in skin papillomas have been negative.¹ Accordingly, a viral origin is unlikely.

The principle concern in the differential diagnosis of FCP is viral warts. FCP may morphologically resemble viral warts, but the microscopic changes in the granular layer of FCP are not present as they are in verruca vulgaris. The verrucous velvety pattern of malignant acanthosis nigricans facilitates the distinction, although acanthosis nigricans and FCP often occur together.

The sudden eruption of multiple seborrheic keratoses or the sign of Leser-Trélat may occur with acanthosis nigricans and FCP. Each seborrheic keratosis is a discrete verrucous nodule that appears as if it has been stuck on the skin. This nodule is easily distinguished from viral warts after careful scrutiny. However, patients have been reported to have oral acanthosis nigricans with multiple verrucouslike nodules on all of the extremities or rice- to pea-sized nodules on the trunk; these findings do not allow the reader to distinguish between the sign of Leser-Trélat and FCP.^{14, 15}

TREATMENT

Section 6 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Medical Care

• Although follow-up evaluation has been limited in most reported cases, marked improvement of the cutaneous lesions was observed in 7 patients after they underwent surgical or chemotherapeutic treatment of the internal cancer.
• • In at least 1 patient, local recurrence of the tumor was associated with a recurrence of FCP.
  • In other cases, generalization of the cancer was associated with severe worsening of the cutaneous lesions.
• To the author's knowledge, the palliative treatment of cutaneous papillomas has not produced any significant improvement, except in one patient in whom topical 5-fluorouracil was used.
• • Such palliative treatments include systemic therapy with oral retinoids, radiation, or smallpox vaccination.
  • Topical treatments with steroids, vitamin A acid, urea, salicylic acid, podophyllotoxin,⁹ and liquid nitrogen have also been tried.

FOLLOW-UP

Section 7 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Further Outpatient Care

• Monitor patients for any recurrence of FCP (assuming that it regresses after tumor therapy) because the reappearance of FCP can indicate a new tumor.

MISCELLANEOUS

Section 8 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Because FCP is a sign of internal cancer, failure to identify it may result in a delay in the diagnosis of the visceral malignancy.

REFERENCES

Section 9 of 9

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

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Article Last Updated: May 13, 2008