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Chapter 134: Cutaneous Paraneoplastic Syndromes

Manasmon Chairatchaneeboon; Ellen J. Kim

CUTANEOUS PARANEOPLASTIC SYNDROMES

Paraneoplastic syndromes refer to the remote effects of underlying neoplastic diseases. The clinical syndromes can occur in various organ systems, including endocrine, neuromuscular, cardiovascular, cutaneous, hematologic, GI, and renal. The symptoms are not the direct effect of metastases or tumor invasion, but may result from substances produced by the tumor (eg. hormones, peptides, or cytokines) or from immunologic or inflammatory reactions between malignant and normal tissues.

Cutaneous paraneoplastic syndromes (Table 134-1) are diverse dermatologic entities that suggest the presence of a remote malignancy. In some instances, they may appear before a cancer diagnosis and contribute to the discovery of an occult cancer, or they may also indicate recurrence in a patient with prior remission of cancer. Consequently, prompt recognition of the cutaneous paraneoplastic syndromes may lead to the detection of an underlying malignancy at an early and highly-treatable stage. However, the paraneoplastic manifestations may predate the diagnosis of the related neoplasm by many months or years. If an underlying cancer is not detectible, careful followup should continue for several years.

Table 134-1Classic Paraneoplastic Dermatoses
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Table 134-1 Classic Paraneoplastic Dermatoses
PARANEOPLASTIC DERMATOSIS MAJOR INTERNAL MALIGNANCY PERCENT WITH CANCER UNIQUE FEATURES
Hyperkeratotic Diseases
Acanthosis nigricans Adenocarcinomas: intraabdominal; gastric cancer (CA) (60%) Unknown Older age, rapid course, extensive, and oral involvement indicate malignancy associations; more common in benign condition with insulin resistance and metabolic syndromes
Acquired ichthyosis Hodgkin lymphoma most common Unknown Spares flexures, palms, soles; can occur in several benign conditions
Pityriasis rotunda GI and hematologic malignancies; hepatocellular CA most common 30% of Type I

Type I: African/Asian; <30 lesions, high rate of malignancy

Type II: whites, familial; >30 lesions, cancer rare
Tripe palms Lung CA most common; gastric CA second >90% Coexists with acanthosis nigricans in 70%
Leser-Trélat sign Adenocarcinomas: GI (32%); lymphoproliferative disorders (20%) Unknown Seborrheic keratoses (SKs) with early onset, eruptive nature, pruritus; coexistence with acanthosis nigricans and/or tripe palms suggests paraneoplastic type
Bazex syndrome Squamous cell carcinoma (SCC) of upper aerodigestive tract Nearly 100% Acral papulosquamous lesions (ears, nose), paronychia, onychodystrophy
Collagen–Vascular Diseases
Dermatomyositis

Women: ovarian and breast CA

Men: GI, respiratory tract CA

Asians: nasopharyngeal CA

 18%-25% Older age, males predominate; refractory or flaring of previously well-controlled disease may signal malignancy
Progressive systemic sclerosis Lung CA 3%-11% Older age of onset, diffuse cutaneous disease, and RNA polymerase III autoantibodies
Reactive Erythemas
Erythema gyratum repens Lung CA 70% Migratory “wood-grain” pattern; erythema
Necrolytic migratory erythema Glucagonoma (pancreatic α cell tumor) Nearly 100% Can be first sign of glucagonoma syndrome; early recognition is important; most have metastatic tumors at the time of diagnosis
Neutrophilic Dermatoses
Sweet syndrome Acute myeloid leukemia (AML) most common 20% Bullous and ulcerated skin lesions, subcutaneous nodules, oral mucosal involvement, anemia, and abnormal platelet count are more common with malignancy
Pyoderma gangrenosum AML most common 4%-20% Atypical presentation more common with malignancy; may be on a ...

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