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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?

 
 
 

Which recessively inherited diseases have skin findings and associated internal malignancy?

 

See Table 35-2.
Table 35-2. Recessively Inherited Diseases with Skin Findings and Malignancy
  DISORDER INHERITANCE CLINICAL FINDINGS CANCER AFFECTED GENE
 
Wiskott-Aldrich syndrome
 
X-linked
recessive
 
Chronic dermatitis
Thrombocytopenia
Recurrent infections
 
Lymphoma, especially non-Hodgkin’s
 
WAS
 
Bloom’s syndrome
 
Autosomal
recessive
 
Photosensitivity
Telangiectasia of sun-exposed skin

Short stature
Decreased serum immunoglobulins

Recurrent infections
Sister chromatid exchange
 
Lymphomas
Leukemias
 
RecQ3
 
Ataxia-telangiectasia (Louis-Bar syndrome)
 
Autosomal
recessive
 
Progressive cerebellar ataxia

Telangiectasia
Recurrent sinus and pulmonary infections

Decreased/absent serum IgA
 
Lymphomas (increased cancer risk also seen in heterozygotes)
 
ATM
 
Dyskeratosis
congenita
 
X-linked recessive
Autosomal dominant
 
Skin atrophy and hyperpigmentation

Nail dystrophy
Oral precancerous leukokeratosis
 
Oral cancers
Other malignancies
 
DKC1
TERC
  WAS, Wiskott-Aldrich syndrome; RecQ3, DNA helicase Rec Q protein-like-3; ATM, ataxia telangiectasia mutated; DKC1, dyskerin;
TERC, telomerase RNA component.