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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 are the clinical findings in Cowden’s syndrome (multiple hamartoma syndrome)?

Cowden’s syndrome. A, Typical small keratotic papules on the face. B, Characteristic cobblestone papules on the mucosal surface of the lower lip.

Fig. 35.7 Cowden’s syndrome. A, Typical small keratotic papules on the face. B, Characteristic cobblestone papules on the mucosal surface of the lower lip.

This syndrome is characterized by a triad of findings:
1. small keratotic facial papules (Fig. 35-7A),
2. cobblestoning of the oral mucosa (Fig. 35-7B), and
3. acral keratotic skin lesions.

These patients also have benign tumors of neural, fibrous, vascular, and epithelial origin. Multiple small tumors of facial hair follicles (trichilemmomas) are pathognomonic of this syndrome. Fibrocystic disease of the breast is common, and 30% of women will develop breast cancer. Many other associated cancers have been reported, with thyroid cancer being the second most common malignancy.