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Cutaneous Manifestations of Internal Malignancy

»	List the five criteria that establish an association between a skin disease and internal malignancy.
»	What is Sweet’s syndrome?
»	Describe the cutaneous lesions of Sweet’s syndrome.
»	Are any laboratory abnormalities found in Sweet’s syndrome?
»	What cancers are associated with Sweet’s syndrome?
»	Describe the clinical appearance of acanthosis nigricans.
»	What clinical disease states are associated with acanthosis nigricans?
»	What is necrolytic migratory erythema?
»	What is hypertrichosis lanuginosa?
»	What is Trousseau’s sign?
»	Describe the classical skin lesions of dermatomyositis.
»	Is dermatomyositis associated with internal malignancy?
»	What are the three components of Sézary’s syndrome?
»	What is paraneoplastic pemphigus?
»	Discuss the laboratory findings in patients with paraneoplastic pemphigus.
»	What is the characteristic finding in erythema gyratum repens?
»	How do the lesions in Bazex syndrome (acrokeratosis paraneoplastica) progress?
»	Where does Paget’s disease most commonly occur?
»	Which disorder of protein metabolism is associated with skin lesions and malignancy?
»	List the autosomal dominant diseases that have prominent skin findings and internal cancer.
»	Describe the cutaneous features of Gardner’s syndrome.
»	What are the clinical findings in Cowden’s syndrome (multiple hamartoma syndrome)?
»	When do the characteristic skin lesions of Peutz-Jeghers syndrome appear?
»	How does multiple mucosal neuroma syndrome typically present?
»	What is Torre’s syndrome (Muir-Torre’s syndrome)?
»	Is the sign of Leser-Trélat (eruptive seborrheic keratoses) associated with internal malignancy?
»	Is dry scaly skin associated with internal malignancy?
»	Which recessively inherited diseases have skin findings and associated internal malignancy?
»	Can pyoderma gangrenosum be associated with internal malignancy?
»	What is erythromelalgia?
»	Can pruritus be a sign of malignancy?
»	Is vasculitis associated with malignancy?
»	What is multicentric reticulohistiocytosis?
»	What is carcinoid syndrome? Does it have prognostic significance?
»	What is FAMM syndrome?

What is FAMM syndrome?

FAMM is the familial atypical multiple mole melanoma–pancreatic cancer syndrome, and it appears to be related to germline mutations in cyclin-dependent kinase inhibitor 2A (CDKN2A). Affected patients have numerous clinically atypical nevi, increased lifetime risk for melanoma, and a family history of melanoma and/or pancreatic cancer. Affected families benefit from screening for early detection of pancreatic cancer.

Lynch HT, Fusaro R, Lynch JF, et al: Pancreatic cancer and the FAMM syndrome, Fam Cancer 7:103–112, 2008.