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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?

Describe the classical skin lesions of dermatomyositis.

Fig. 35.4 Dermatomyositis. A, Facial erythema and heliotrope sign. B, Classic hand lesions and Gottron’s papules on the knuckles.

The classic eruption of dermatomyositis is a reddish-purple erythema involving the face, typically the eyelids (heliotrope sign). The rash may be faint or quite inflamed and edematous (Fig. 35-4A).

In addition to the facial rash, lesions on the scalp, neck, upper trunk, and extensor extremities are common. As the lesions mature, scaling and atrophy may develop. The erythema on the hands occurs over the knuckles rather than over the phalanges, as is typical of lupus erythematosus. Cuticular telangiectasias can be seen in both lupus erythematosus and dermatomyositis. Frequently, flat-topped, red-to-violaceous papules known as Gottron’s papules develop over the knuckles of patients with dermatomyositis (Fig. 35-4B).

The skin lesions of dermatomyositis may precede clinical or laboratory evidence by weeks, months, or years. A few patients may never develop muscle dysfunction. The skin lesions are notoriously resistant to topical steroid therapy.