This article may be too technical for most readers to understand.(April 2018) |
Florid cutaneous papillomatosis | |
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Other names | Schwartz–Burgess syndrome |
Specialty | Dermatology |
Florid cutaneous papillomatosis (FCP), is an obligate paraneoplastic syndrome.
FCP begins as the sudden onset of numerous cutaneous papillomas that are clinically indistinguishable from viral warts. The papillomas range from 1 to 3 mm in diameter may spread to involve the entire body, including the face. Pruritus, which may sometimes precede the onset of FCP, is evident in the affected regions in about half of patients. Evaluation of a skin biopsy clearly distinguishes FCP from viral warts.
FCP is associated with underlying cancer of the breast, bladder, ovary, uterus, prostate, and lung. Other associated underlying malignancies include squamous cell carcinomas and lymphomas such as non-Hodgkin's lymphoma.
FCP is sometimes seen together with other signs of internal cancer, especially malignant acanthosis nigricans, tripe palms, Leser–Trélat sign, and hypertrichosis lanuginosa acquisita. FCP tends to improve in association with surgical or chemotherapeutic therapy of the underlying internal cancer. A recurrence or exacerbation of FCP may be linked with tumor regrowth or metastatic spread.
The characteristic eruption is of multiple warty papules and nodules beginning on acral skin, especially the hands and wrists, and disseminating onto the skin of the entire body. These skin lesions develop on the trunk, extremities, and face. [1] : 63 Pruritus is also associated.
The etiology of florid cutaneous papillomatosis is unknown. It is likely directly induced by an underlying neoplasm secreting a growth factor. One candidate may be alpha-transforming growth factor, structurally related to epidermal growth factor, but antigenically distinct from it. The underlying cancer is most often gastric adenocarcinoma [1] : 63 [2] [3] but also with breast cancer, bladder cancer, hepatobiliary cancer, ovarian cancer, uterine cancer, [4] prostate cancer [ citation needed ], lung cancer [5] and cervical cancer. [6] Other associated underlying malignancies include squamous cell carcinomas and lymphomas. [7]
Florid cutaneous papillomatosis, malignant acanthosis nigricans, and the sign of Leser-Trélat may be highlighted as part of a continuum, with each having a common or similar pathogenic pathway due to an underlying malignancy that produces a factor epidermal growth factor-like activity.[ citation needed ]
The sudden eruption of papulonodules usually indistinguishable from common viral warts should suggest this diagnosis. These papulonodules begin on the extremities, especially on the dorsa of the hands and the wrists and may disseminate to involve the entire body, including the face. [8] The papulonodules may vary in size from 2–3 mm to 10 mm in diameter. Pruritus is often associated.
Florid cutaneous papillomatosis is linked with an underlying cancer. Malignant acanthosis nigricans may also become evident, many times with the sudden eruption of multiple seborrheic keratoses, known as the sign of Leser-Trélat. [9] [10] [11] Florid cutaneous papillomatosis mandates a search for an underlying malignancy, recognizing that it may be seen in patients with multiple visceral carcinomas. Histologic examination shows uniform and pronounced hyperkeratosis, acanthosis, and papillomatosis without epidermal vacuolization, parakeratosis, or eosinophilic inclusions suggestive of viral warts. [12]
The sudden quality of the eruption of florid cutaneous papillomatosis and its anatomic distribution should facilitate distinction from widespread common warts and from epidermodysplasia verruciformis.[ citation needed ]
Identifying and treatment the underlying malignancy constitutes an uptime approach. Topical 5-fluorouracil may occasionally be help, as may oral retinoids, topical steroids, vitamin A acid, urea, salicylic acid, podophyllotoxin, and cryodestruction employing liquid.[ citation needed ]
Improvement usually parallels that of the cancer, whether surgical or chemotherapeutic. Generalization of the associated visceral malignancy may worsen the eruption.
Florid cutaneous papillomatosis is almost twice as common in men than in women, and is usually diagnosed in individuals aged 53–72 years (mean patient age, 58.5 years). [1] : 66
Florid cutaneous papillomatosis was discovered by Robert A. Schwartz and Gordon H. Burgess. [13] The original description was published in the Archives of Dermatology, [14] which is published by American Medical Association. It has since been called Schwartz–Burgess syndrome. [15] [16]
Patients may have their unaesthetic appearance resulting in isolation from their community, feeling or being unwelcome in public places. [17]
Stomach cancer, also known as gastric cancer, is a cancer that develops from the lining of the stomach. Most cases of stomach cancers are gastric carcinomas, which can be divided into a number of subtypes, including gastric adenocarcinomas. Lymphomas and mesenchymal tumors may also develop in the stomach. Early symptoms may include heartburn, upper abdominal pain, nausea, and loss of appetite. Later signs and symptoms may include weight loss, yellowing of the skin and whites of the eyes, vomiting, difficulty swallowing, and blood in the stool, among others. The cancer may spread from the stomach to other parts of the body, particularly the liver, lungs, bones, lining of the abdomen, and lymph nodes.
Acanthosis nigricans is a medical sign characterised by brown-to-black, poorly defined, velvety hyperpigmentation of the skin. It is usually found in body folds, such as the posterior and lateral folds of the neck, the armpits, groin, navel, forehead and other areas.
Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, and is only named that because it looks like one. It is characterized by polygonal, flat-topped, violaceous papules and plaques with overlying, reticulated, fine white scale, commonly affecting dorsal hands, flexural wrists and forearms, trunk, anterior lower legs and oral mucosa. The hue may be gray-brown in people with darker skin. Although there is a broad clinical range of LP manifestations, the skin and oral cavity remain as the major sites of involvement. The cause is unknown, but it is thought to be the result of an autoimmune process with an unknown initial trigger. There is no cure, but many different medications and procedures have been used in efforts to control the symptoms.
A seborrheic keratosis is a non-cancerous (benign) skin tumour that originates from cells in the outer layer of the skin. Like liver spots, seborrheic keratoses are seen more often as people age.
Gastrointestinal cancer refers to malignant conditions of the gastrointestinal tract and accessory organs of digestion, including the esophagus, stomach, biliary system, pancreas, small intestine, large intestine, rectum and anus. The symptoms relate to the organ affected and can include obstruction, abnormal bleeding or other associated problems. The diagnosis often requires endoscopy, followed by biopsy of suspicious tissue. The treatment depends on the location of the tumor, as well as the type of cancer cell and whether it has invaded other tissues or spread elsewhere. These factors also determine the prognosis.
The International Classification of Diseases for Oncology (ICD-O) is a domain-specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. This classification is widely used by cancer registries.
The Leser–Trélat sign is the explosive onset of multiple seborrheic keratoses, often with an inflammatory base. This can be a sign of internal malignancy as part of a paraneoplastic syndrome. In addition to the development of new lesions, preexisting ones frequently increase in size and become symptomatic.
Verrucous carcinoma (VC) is an uncommon variant of squamous cell carcinoma. This form of cancer is often seen in those who chew tobacco or use snuff orally, so much so that it is sometimes referred to as "Snuff dipper's cancer".
Necrolytic migratory erythema is a red, blistering rash that spreads across the skin. It particularly affects the skin around the mouth and distal extremities; but may also be found on the lower abdomen, buttocks, perineum, and groin. It is strongly associated with glucagonoma, a glucagon-producing tumor of the pancreas, but is also seen in a number of other conditions including liver disease and intestinal malabsorption.
A paraneoplastic syndrome is a syndrome that is the consequence of a tumor in the body, specifically due to the production of chemical signaling molecules by tumor cells or by an immune response against the tumor. Unlike a mass effect, it is not due to the local presence of cancer cells.
Lymphomatoid papulosis (LyP) is a rare skin disorder. The overall prevalence rate of lymphomatoid papulosis is estimated at 1.2 to 1.9 cases per 1,000,000 population. [This is a widespread misinterpretation of a 1992 study saying "the period prevalence rate of lymphomatoid papulosis was estimated to be 1.9 per 1,000,000 population for Massachusetts and 1.2 per 1,000,000 population for Pennsylvania". The authors of that study said clearly "Our estimate of 1.2-1.9 cases per 1,000,000 population should be considered a minimum estimate of the prevalence rate". That estimate was based on the 78 patients involved in the study, not the LvP population. The study recruited 11 patients from Massachusetts and 15 from Pennsylvania ]. This rare condition has only been studied in depth since 1968.
Cutaneous horns, also known by the Latin name cornu cutaneum, are unusual keratinous skin tumors with the appearance of horns, or sometimes of wood or coral. Formally, this is a clinical diagnosis for a "conical projection above the surface of the skin." They are usually small and localized but can, in very rare cases, be much larger. Although often benign, they can also be malignant or premalignant.
The book Skin Cancer: Recognition and Management by Robert A. Schwartz is a clinical reference covering skin and accessible mucosal disorders, premalignant and malignant cutaneous disorders, including melanoma, Kaposi's sarcoma and other sarcomas, cutaneous lymphoma, cutaneous metastatic disease and cutaneous markers of internal malignancy. It emphasizes skin cancer prevention, as well as recent advances in diagnosis and management. It has a chapter exploring dermoscopic evaluation of skin cancer and a chapter on oral cancer.
Robert Allen Schwartz is an American physician, biomedical researcher, university professor, and government official. He is Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, and Professor of Preventive Medicine and Community Health at the Rutgers New Jersey Medical School, Visiting Professor and Scholar of Public Affairs and Administration at the Rutgers School of Public Affairs and Administration, and serves on the Rutgers University Board of Trustees. He has made seminal contributions to medicine, including the discovery of AIDS-associated Kaposi sarcoma (KS-AIDS) and Schwartz–Burgess syndrome. In 2019 Schwartz joined the Trump administration as a member of the Presidential Advisory Council on HIV/AIDS.
Dermatosis neglecta is a skin condition in which accumulation of sebum, keratin, sweat, dirt and debris leads to a localized patch of skin discoloration or a wart-like plaque. It is caused by inadequate hygiene of a certain body part, usually due to some form of disability or a condition that is associated with pain or increased sensitivity to touch (hyperesthesia) or immobility.
An arsenical keratosis is a growth of keratin on the skin caused by arsenic, which occurs naturally in the earth's crust and is widely distributed in the environment, Arsenical compounds are used in industrial, agricultural, and medicinal substances. Arsenic is also found to be an environmental contaminant in drinking water and an occupational hazard for miners and glass workers. Arsenic may also causes other conditions including: Bowen's disease, cardiovascular diseases, developmental abnormalities, neurologic and neurobehavioral disorders, diabetes, hearing loss, hematologic disorders, and various types of cancer. Arsenical keratoses may persist indefinitely, and some may develop into invasive squamous cell carcinoma. Metastatic arsenic squamous cell carcinoma and arsenic-induced malignancies in internal organs such as the bladder, kidney, skin, liver, and colon, may result in death.
Eccrine carcinoma is a rare skin condition characterized by a plaque or nodule on the scalp, trunk, or extremities. It originates from the eccrine sweat glands of the skin, accounting for less than 0.01% of diagnosed cutaneous malignancies. Eccrine carcinoma tumors are locally aggressive, with a high rate of recurrence. Lack of reliable immunohistochemical markers and similarity to other common tumors has made identification of eccrine carcinoma difficult.
Howel–Evans syndrome is an extremely rare condition involving thickening of the skin in the palms of the hands and the soles of the feet (hyperkeratosis). This familial disease is associated with a high lifetime risk of esophageal cancer. For this reason, it is sometimes known as tylosis with oesophageal cancer (TOC).