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Visible Paraneoplastic Phenomena

Visible Paraneoplastic Phenomena: Excerpt from Field Guide to Bedside Diagnosis

Differential Overview

❑ Clubbing

❑ Cutaneous metastases

❑ Hyperpigmentation

❑ Cryoglobulinemia

❑ Acanthosis nigricans

❑ Acquired lanugo hair

❑ Generalized pruritus

❑ Carcinoid flush

❑ Dermatomyositis

❑ Seborrheic keratosis

❑ Neuromas

❑ Mucocutaneous nevi

❑ Palmar and plantar hyperkeratosis

❑ Thrombophlebitis migrans

❑ Amyloidosis

❑ Sweet syndrome

❑ Exfoliative erythroderma

❑ Gardner syndrome

❑ von Recklinghausen disease

❑ Erythema gyratum repens

❑ Necrolytic migratory erythema

❑ Acquired icthyosis

❑ Pancreatic panniculitis

❑ Tripe palm

Diagnostic Approach

Cutaneous manifestations may develop before an internal malignancy is recognized. Sometimes the skin lesion contains the tumor, through metastatic (lung cancer nodules) or local spread (Paget disease of the breast), direct infiltration by circulating tumor cells (lukemia cutis), or may be the primary site (Kaposi sarcoma in an HIV patient). At other times, findings are distant manifestations that provide clues to diagnosis.

Clinical Findings

Clubbing  The nail bed becomes elevated and spongy, with the nail bed angle expanding to more than 180 degrees. Associated cancers include bronchus, stomach, esophagus, thymus and mesothelioma, although clubbing is also commonly seen with nonmalignant cardiac and pulmonary conditions causing peripheral hypoxia. An extreme form, pachydermoperiostosis, has increased sebaceous activity with thickening of the skin on the forehead and scalp that gives the patient a leonine facial appearance.

Cutaneous metastases  Hard nodules in the scalp will have spread from lung, breast, or genitourinary cancers. Umbilical (Sister Mary Joseph) nodules appear with intra-abdominal malignancy.

Hyperpigmentation  Small cell lung carcinoma can produce circulating ACTH/MSH-like polypeptides, causing diffuse hyperpigmentation along with hypertension, muscle wasting, and edema.

Cryoglobulinemia  Hemorrhagic bullae or ulcers appearing in acral regions after cold exposure are seen in plasma cell dyscrasias (or more commonly in hepatitis C).

Acanthosis nigricans  Hyperpigmented, velvety, hyperkeratotic patches in the body folds, especially axilla, nipples, and umbilicus, are associated with gastric (most frequently), liver, and lung cancer. It may precede the clinical appearance of malignancy in 20%. Other causes, such as obesity or diabetes with insulin resistance, are more common. Malignancy-associated acanthosis nigricans is more florid, develops in unusual locations, and may be accompanied by the sudden appearance of multiple skin tags or seborrheic keratoses (Leser-Trelat sign).

Acquired lanugo hair  Downy hair appearing on the face and trunk may signal cancer of the bronchus, gallbladder, bladder, or rectum. More common causes are anorexia nervosa and Cushing syndrome.

Generalized pruritus  Pruritus associated with lymphoma (both Hodgkin and non-Hodgkin), leukemia and gastric carcinoids begins with local burning, which develops into intense pruritus.

Carcinoid flush  A deep red to purple flush appears over the face and neck, is associated with abdominal pain, diarrhea, edema, palpitations, wheezing, and dizziness, and is precipitated by food rich in tyramine or alcohol. It is seen in GI carcinoid tumors once metastatic to the liver.

Dermatomyositis  Heliotrope, ranging from a violet suffusion to a rash on the eyelids, and purplish Gottron papules over the knuckles have an association with gastrointestinal, breast, lung, and genitourinary cancers and lymphoma, especially in patients older than 60 years of age.

Seborrheic keratosis  The sudden development of a large number of inflamed/pruritic seborrheic keratoses (Leser-Trelat) is associated with carcinoma of the stomach, breast and lung, as well as lymphoma.

Neuromas  Pink nodules on the lips, eyelids, nares, and anterior two-thirds of the tongue are found in MEN III syndrome and in those with thyroid cancer and pheochromocytoma.

Mucocutaneous nevi  Peutz-Jeghers is associated with gastrointestinal malignancies occurring before the ligament of Treitz.

Palmar and plantar hyperkeratosis  Tylosis, a yellow, symmetrical thickening is associated with squamous cell cancer esophageal cancer and chronic arsenic poisoning.

Thrombophlebitis migrans  Recurrent episodes of thrombophlebitis, which are especially suspicious because they occur in the absence of precipitating factors, in unusual locations, or while on anticoagulation medications, signal an underlying adenocarcinoma of the pancreas, lung, colon, or stomach.

Amyloidosis  An enlarged tongue with dental indentations, and waxy purpuric periorbital plaques (“pinch purpura”) are visible manifestations of amyloidosis. Secondary amyloidosis may be caused by multiple myeloma.

Sweet syndrome  Painful bright red to red-brown plaques appear on the head, neck, and upper extremities, along with fever. About 10% are associated with cancer, most commonly acute myelogenous leukemia, but also with lymphomas and plasma cell disorders.

Exfoliative erythroderma  Diffuse generalized erythema of the skin is accompanied by intense pruritis, generalized lymphadenopathy, and ectropion. Most often this is due to atopic dermatitis, psoriasis, or drug hypersensitivity, but 10% of cases are associated with lymphoid malignancy, particularly cutaneous T-cell lymphoma (Sezary syndrome).

Gardner syndrome  Findings include sebaceous or epidermal cysts, lipomas, osteomas, supernumary teeth, and nasopharyngeal angiofibromas, associated with intestinal adenocarcinoma.

von Recklinghausen disease  Visible findings of cafe-au-lait macules and cutaneous neurofibromas are associated with optic pathway gliomas and other tumors involving the nervous system.

Erythema gyratum repens  Serpiginous bands with marginal desquamation in concentric rings that resemble burled wood are associated with breast and lung cancer.

Necrolytic migratory erythema  Bright erythematous areas with serpiginous outlines on the buttocks, intertriginous folds, and flexural aspects of the legs are characteristic of glucagonoma. Central blister formation occurs and is followed by hyperpigmentation with healing.

Acquired icthyosis  Prominent, generalized, large, adherent, fishlike scales, most commonly associated with Hodgkin disease, but also with cutaneous T-cell lymphoma, breast, lung or bladder cancer, or Kaposi sarcoma. It has also been seen in graft-versus-host disease following bone marrow transplantation. It should be differentiated from benign congenital icthyosis, which besides onset at an early age, affects the palms, soles, and flexor creases.

Pancreatic panniculitis  Tender erythematous to violaceous ulcerated nodules occur in association with fever and weight loss in pancreatic cancer.

Tripe palm  Velvety thickening of the palms with an exaggeration of normal skin markings is associated most prominently with stomach and bronchogenic cancer.

Book Source Details

  • Book Title: Field Guide to Bedside Diagnosis
  • Author(s): David S. Smith
  • Year of Publication: 2007
  • Copyright Details: Field Guide to Bedside Diagnosis, Copyright © 2007 Lippincott Williams & Wilkins.

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.




More About This Book:
Title: Field Guide to Bedside Diagnosis
Authors: David S. Smith
Publisher: Lippincott Williams & Wilkins
Copyright: 2007
ISBN: 0-78178-165-5

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