Chapter 35 Cutaneous manifestations of internal malignancy
1. List the five criteria that establish an association between a skin disease and internal malignancy.
In the late 1950s, Helen Curth, while evaluating acanthosis nigricans, established five criteria necessary to make this association. These five criteria are called Curth’s postulates:
1. Concurrent onset of the cutaneous disease and internal malignancy—or at the onset of the cutaneous disease, the internal malignancy is recognizable
4. Sound statistical evidence that the malignancy is more frequent in patients with the skin disease than in age- and sex-matched controls
2. What is Sweet’s syndrome?
Sweet’s syndrome occurs mostly in women 30 to 60 years of age and consists of characteristic skin lesions, fever, malaise, and leukocytosis. Less commonly, there is involvement of the joints, eyes, lungs, kidneys, and liver. Approximately 20% of cases have an association with a hematopoietic malignancy or, more rarely, a solid tumor.
3. Describe the cutaneous lesions of Sweet’s syndrome.
The clinical hallmark of Sweet’s syndrome is the presence of sharply demarcated, painful plaques on the face, neck, upper trunk, and extremities (Fig. 35-1). The surface of the plaques has a mammillated (nipple-like) appearance and often shows papulovesicles and pustules. Some lesions have a target-like appearance, and lesions on the lower extremities may resemble erythema nodosum. Oral mucous membrane and eye lesions can be seen. Skin lesions may develop at the site of minor skin trauma or needle-sticks in a small subset of patients. This phenomenon is called pathergy and is also seen in pyoderma gangrenosum and Behçet’s syndrome.
4. Are any laboratory abnormalities found in Sweet’s syndrome?
Leukocytosis. Ten thousand/mm3 is present in 60% of patients. Elevated sedimentation rates, increased numbers of segmented neutrophils, lymphopenia, anemia, thrombocytopenia, and increased C-reactive protein levels can be seen. A handful of cases have been reported with antineutrophilic cytoplasmic antibodies.
5. What cancers are associated with Sweet’s syndrome?
The most commonly associated malignancy is acute myelogenous leukemia, but chronic myelogenous leukemia, lymphocytic leukemia, T- and B-cell lymphomas, polycythemia, and, rarely, solid tumors also have been reported. There are no clinical or histopathologic differences between patients with and without associated cancer. Patients with persistent laboratory abnormalities, especially anemia, thrombocytosis, and thrombocytopenia, require close observation and thorough diagnostic evaluation.
Figure 35-1. Sweet’s syndrome demonstrating painful red indurated plaques on on the hand and arm.
(Courtesy of James E. Fitzpatrick, MD.)
Bae-Harboe Y-SC, Salter SA, Kimball A: Acute febrile neutrophilic dermatosis, eMedicine Online August 2009. Available at: http://www.emedicine.com.
7. What clinical disease states are associated with acanthosis nigricans?
Acanthosis nigricans is a common skin finding, reported in 7.1% of children aged 11 to 16, and is frequently associated with obesity. It is commonly found in association with diabetes and other endocrinopathies with insulin resistance. Paraneoplastic acanthosis nigricans is rare and is most commonly associated with gastrointestinal cancer, especially gastric carcinoma. When associated with malignancy, it is usually abrupt in onset, severe, and may involve mucous membranes and palmar skin. Findings of tripe palms (resembling rugose bovine intestine) and the sign of Leser-Trélat, discussed later in this chapter, are often seen in patients with paraneoplastic acanthosis nigricans.
Figure 35-2. Acanthosis nigricans with hyperpigmented velvety skin lesions and small tags on the proximal thigh and groin.
Miller J, Rapini R: Acanthosis nigricans, eMedicine Online, June 2002. Available at: http://www.emedicine.com/derm/topic1.htm.
8. What is necrolytic migratory erythema?
This characteristic skin eruption is associated with an α-cell tumor of the pancreas. It presents as erythema with superficial pustules and erosions, typically involving the face, intertriginous skin, and acral extremities (Fig. 35-3). Alopecia, weight loss, glossitis, stomatitis, nail dystrophy, anemia, and diabetes are frequent associations. The eruption tends to migrate and desquamate, and most patients have elevated glucagon serum levels (glucagonoma syndrome). Skin biopsy shows necrosis of the upper portion of the epidermis and is usually diagnostic. This unique skin disease is probably related to low serum amino acid levels.
Zettouni N, Harvey N: Glucagonoma syndrome, eMedicine Online, http://www.emedicine.com. May 2008.
9. What is hypertrichosis lanuginosa?
Hypertrichosis lanuginosa (malignant down) is an acquired excessive growth of lanugo hair. It usually begins on the face, neck, and ears and eventually can involve most hair-bearing skin. Glossitis is frequently an associated finding. If drug-related causes (such as minoxidil, diazoxide, and cyclosporine) can be excluded, there is a high association with internal malignancy. The most common associated cancers are lung, breast, gastrointestinal, and carcinoid. This rare cutaneous finding has also been reported in patients with anorexia nervosa.
10. What is Trousseau’s sign?
Trousseau’s sign consists of recurrent and migratory superficial thrombophlebitis, affecting both large and small cutaneous veins, which is associated with an internal cancer. Crops of oval to linear, erythematous, tender skin lesions are seen most commonly on the arms, legs, flanks, and abdomen. Thrombosis of internal veins can also occur and lead to a variety of symptoms. Men are more commonly affected. The most commonly associated cancers are lung and pancreatic carcinoma.
11. Describe the classical skin lesions of dermatomyositis.
The classic eruption of dermatomyositis is a reddish-purple erythema involving the face, typically the eyelids (heliotrope sign). The rash may be faint or quite inflamed and edematous (Fig. 35-4A).
In addition to the facial rash, lesions on the scalp, neck, upper trunk, and extensor extremities are common. As the lesions mature, scaling and atrophy may develop. The erythema on the hands occurs over the knuckles rather than over the phalanges, as is typical of lupus erythematosus. Cuticular telangiectasias can be seen in both lupus erythematosus and dermatomyositis. Frequently, flat-topped, red-to-violaceous papules known as Gottron’s papules develop over the knuckles of patients with dermatomyositis (Fig. 35-4B).
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