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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?

 
 
 

Discuss the laboratory findings in patients with paraneoplastic pemphigus.

Skin and oral mucous membrane biopsy specimens reveal epidermal acantholysis, epidermal spongiosis, suprabasilar clefts, basal cell vacuolar changes, and dyskeratotic keratinocytes. Direct immunofluorescent examination reveals IgG and, less commonly, IgA with or without complement in the intracellular spaces and C3, IgG, or IgM at the basement membrane zone. Antibodies have been demonstrated against desmoplakins, proteins in keratinocyte attachment plaques (desmosomes), and a 230-kDa protein in the basement membrane (bullous pemphigoid antigen). Rat bladder is a useful substrate for indirect immunofluorescent examination and shows positive staining with serum from patients with paraneoplastic pemphigus but will be negative with serum from patients with classic pemphigus vulgaris.

Zhu X, Zhang B: Paraneoplastic pemphigus, J Dermatol 34:503–511, 2007.