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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?

Can pyoderma gangrenosum be associated with internal malignancy?

Fig. 35.8 A young woman with acute-onset pyoderma gangrenosum.

Yes. Pyoderma gangrenosum (PG) is an ulcerative skin disease of unknown etiology. The lesions are painful, may rapidly enlarge, and are characterized by an erythematous or violaceous undermined border with a necrotic center (Fig. 35-8). The most common diseases associated with PG are inflammatory bowel disease, rheumatoid arthritis, and a small subset may have monoclonal IgA gammopathy. In a review of several studies, PG was associated with internal malignancy in 7.2% of patients. Leukemia is the most frequently reported malignancy with myelocytic and myelomonocytic leukemia accounting for the majority of cases. Other reported hematologic cancers are multiple myeloma, polycythemia vera, and lymphoma. In two thirds of the cases associated with myelocytic leukemia, the PG preceded or was concurrent with the diagnosis of the leukemia. There are rare sporadic reports of PG associated with solid tumors.

Callen J, Jackson JM: Pyoderma gangrenosum: an update, Rheumatol Dis Clin N Am 33:787–802, 2007.