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Florid Cutaneous Papillomatosis

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: May 29, 2009

Introduction

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 It was first observed in 1891.2 Florid cutaneous papillomatosis was first described and the name florid cutaneous papillomatosis was coined in 1978 by Schwartz and Burgess.3

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 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 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.5 See Sign of Leser-Trelat for more information on this topic.

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 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.

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.

Frequency

United States

Florid cutaneous papillomatosis 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, 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).

Clinical

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 florid cutaneous papillomatosis.
    • It can occur in the affected regions, or it can be generalized.
  • The unsightly clinical appearance of florid cutaneous papillomatosis may result in social exclusion.9

Physical

  • Clinically, the papulonodules of florid cutaneous papillomatosis 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.
  • Florid cutaneous papillomatosis 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 florid cutaneous papillomatosis or so-called tripe palms, mandates a thorough search for malignancy.10,11,12,13
  • Florid cutaneous papillomatosis may be seen in patients with multiple visceral carcinomas14 or with an adenocarcinoma of unknown primary site.15

Causes

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

More on Florid Cutaneous Papillomatosis

Overview: Florid Cutaneous Papillomatosis
Differential Diagnoses & Workup: Florid Cutaneous Papillomatosis
Treatment & Medication: Florid Cutaneous Papillomatosis
Follow-up: Florid Cutaneous Papillomatosis
References
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References

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  2. Pollitzer S. Unna PG, Morris M, Leloir H, Duhring LA, eds. International Atlas of Rare Skin Diseases. London, England: HK Lewis; 1891:1-3.

  3. Schwartz RA, Burgess GH. Florid cutaneous papillomatosis. Arch Dermatol. Dec 1978;114(12):1803-6. [Medline].

  4. Stieler W, Plewig G. [Acanthosis nigricans maligna and Leser-Trelat sign in double malignancy of the breast and stomach]. Z Hautkr. Mar 1 1987;62(5):344-66. [Medline].

  5. Dingley ER, Marten RH. Adenocarcinoma of the ovary presenting as acanthosis nigricans. J Obstet Gynaecol Br Emp. Dec 1957;64(6):898-900. [Medline].

  6. Millard LG, Gould DJ. Hyperkeratosis of the palms and soles associated with internal malignancy and elevated levels of immunoreactive human growth hormone. Clin Exp Dermatol. Dec 1976;1(4):363-8. [Medline].

  7. Ellis DL, Kafka SP, Chow JC, et al. Melanoma, growth factors, acanthosis nigricans, the sign of Leser-Trélat, and multiple acrochordons. A possible role for alpha-transforming growth factor in cutaneous paraneoplastic syndromes. N Engl J Med. Dec 17 1987;317(25):1582-7. [Medline].

  8. Singhi MK, Gupta LK, Bansal M, Jain V, Kachhawa D. Florid cutaneous papillomatosis with adenocarcinoma of stomach in a 35 year old male. Indian J Dermatol Venereol Leprol. May-Jun 2005;71(3):195-6. [Medline].

  9. Csete B, Moezzi M, Lengyel Z, Hodosi B, Zombai E, Battyani Z. Florid cutaneous papillomatosis leading to social exclusion. Br J Dermatol. Sep 2005;153(3):667-9. [Medline].

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  11. Bottoni U, Dianzani C, Pranteda G, et al. Florid cutaneous and mucosal papillomatosis with acanthosis nigricans revealing a primary lung cancer. J Eur Acad Dermatol Venereol. May 2000;14(3):205-8. [Medline].

  12. Jung DS, Oh CK, Lee JB, Kim MB, Jang HS, Kwon KS. Three Cases of Cutaneous Paraneoplastic Syndromes: Coexistence of Acanthosis Nigricans, Tripe Palms and Cutaneous Florid Papillomatosis. Korean J Dermatol. 2003;41:1364-1369. [Full Text].

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  14. Weger W, Ginter-Hanselmayer G, Hammer HF, Hodl S. Florid cutaneous papillomatosis with acanthosis nigricans in a patient with carcinomas of the lung and prostate. J Am Acad Dermatol. Nov 2007;57(5):907-8. [Medline].

  15. Yoo KH, Rho YK, Kim JY, Li K, Seo SJ, Hong CK. Cutaneous paraneoplastic syndrome in a patient with adenocarcinoma of unknown primary site syndrome. J Clin Oncol. Jan 10 2009;27(2):309-11. [Medline].

  16. Pentenero M, Carrozzo M, Pagano M, Gandolfo S. Oral acanthosis nigricans, tripe palms and sign of leser-trélat in a patient with gastric adenocarcinoma. Int J Dermatol. Jul 2004;43(7):530-2. [Medline].

  17. Jablonska S, Kozminska A, Chorzelski T. [An unusual variant of acanthosis nigricans. (Relations of acanthosis nigricans to generalized verrucosis)]. Dermatol Wochenschr. Feb 13 1965;151:177-85. [Medline].

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  39. Weger W, Ginter-Hanselmayer G, Hammer HF, Hodl S. Florid cutaneous papillomatosis with acanthosis nigricans in a patient with carcinomas of the lung and prostate. J Am Acad Dermatol. Nov 2007;57(5):907-8. [Medline].

  40. White H. Acanthosis nigricans and wart-like lesions associated with metastatic carcinoma of the stomach. Cutis. May 1976;17(5):931-3. [Medline].

  41. Worret WI, Mayerhausen W, Emslander HP. Hypertrichosis lanuginosa acquista associated with florid cutaneous papillomatosis. Int J Dermatol. Jan 1993;32(1):56-8. [Medline].

  42. Yeh JS, Munn SE, Plunkett TA, Harper PG, Hopster DJ, du Vivier AW. Coexistence of acanthosis nigricans and the sign of Leser-Trelat in a patient with gastric adenocarcinoma: a case report and literature review. J Am Acad Dermatol. Feb 2000;42(2 Pt 2):357-62. [Medline].

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Further Reading

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Keywords

florid cutaneous papillomatosis, Schwartz-Burgess syndrome, FCP, paraneoplastic disease, paraneoplastic syndrome, warts with underlying visceral cancer, warts, malignant acanthosis nigricans, sign of Leser-Trélat, eruptive seborrheic keratoses, hypertrichosis lanuginosa acquisita

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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, UMDNJ-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.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

Jeffrey J Miller, MD, Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center
Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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