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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?

List the autosomal dominant diseases that have prominent skin findings and internal cancer.

See Table 35-1.

Table 35-1. Autosomal Dominant Diseases with Skin Findings and Malignancy
	DISORDER	SKIN FINDINGS	CANCER	ASSOCIATIONS	AFFECTED GENE
	Cowden’s syndrome	Keratotic facial papules Acral keratosis Soft tissue tumors	Breast Thyroid	Mucosal papules Fibrocystic disease of the breast	PTEN
	Torre’s syndrome	Sebaceous tumors Keratoacanthomas	Colon	Colon polyps	MSH2, MLH1
	Gardner’s syndrome	Epidermoid cysts	Colon	Colon polyps Osteomas Desmoids Abnormal dentition	APC
	Peutz-Jeghers syndrome	Pigmented macules on mucosa, face, acral extremities	Intestinal	Intestinal polyps	STK11
	Multiple mucosal neuroma syndrome	Neuromas of lips, tongue, and oral mucosa	Thyroid	Pheochromocytoma Marfanoid habitus	RET
	Neurofibromatosis	Neurofibromas Café-au-lait macules	Neurofibrosarcoma (rare)	Lisch nodules Seizures Deafness	Neurofibromin
	Hereditary leiomyomatosis/renal cell cancer syndrome	Multiple leiomyomas of skin and uterus		Fumarate hydratase
	APC, Adenomatosis polyposis coli; MLH1, micronuclear linker histone; MSH2, melanocyte stimulating hormone; PTEN, phosphate and tension homologue deleted on chromosome 10; RET, RET protooncogene (rearranged during transfection); STK11, serine threonine kinase.