21 - 134 - CUTANEOUS PARANEOPLASTIC SYNDROMES Flashcards
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Elenita is a 50 year old female who came to you for consult of her skin lesions. You assessed that she has multiple acrochordons, fibrofolliculomas and trichodiscomas.
- What is the major associated internal malignancy associated with her skin lesions?
a. breast cancer
b. colorectal cancer
c. renal cancer
d. medullary thyroid cancer p.2444 table 134-2 - What other associated conditions should you investigate for?
a. adrenocortical disease
b. esophageal papillomas
c. cerebellar dysfunction
d. emphysema
p. 2444 table 134-2
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c. renal cancer
d. emphysema
Diagnosis is Birt-Hogg-Dube syndrome
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Rolando is a 40 year old male who came to you for consult for his skin lesions. You assessed that he has heliotrope rash, gottron papules, gottron rash as well as muscle pains.
- Which is true regarding his disease?
a. In adult patients with DM, the risk of malignancy is highest in the first 3-6 months after diagnosis
b. It is associated with GI and respiratory tract cancer in females
c. It is associated with nasopharyngeal cancer in Caucasians
d. Its course does not always follow the course of malignancy
p. 2453 - Which of these organ malignancies is most commonly associated with the patient’s gender?
a. hematologic
b. renal
c. GI
d. lymphatic
p. 2442 table 134-1
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d. Its course does not always follow the course of malignancy
c. GI
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Necrolytic Migratory Erythema is associated with which major internal malignancy?
a. multiple myeloma
b. glucagonoma
c. Hodgkin lymphoma
d. hepatocellular carcinoma p.2442 table 134-1
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b. glucagonoma
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Which set of paraneoplastic associated with lung cancer?
a. tripe palms, systemic sclerosis, erythema gyratum repens
b. erythema gyratum repens, scleromyxedema, sweet syndrome
c. sweet syndrome, tripe palms, erythema gyratum repens, multicentric reticulohistiocytosis
d. dermatitis herpetiformis, tripe palms, pyoderma gangrenosum, necrolytic migratory erythema p.2442 table 134-1
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a. tripe palms, systemic sclerosis, erythema gyratum repens
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Which is true regarding Gorlin syndrome? a. associated with esophageal papillomas
b. can be associated with meningiomas and ovarian fibromas
c. associated with defect in the PRKAR1A gene
d. presents with epidermoid cysts, pilomatricomas and lipomas
p. 2442 table 134-2
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b. can be associated with meningiomas and ovarian fibromas
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Which of these syndromes have an X-linked recessive inheritance?
a. xeroderma pigmentosum
b. maffucci syndrome
c. dyskeratosis congenita
d. Werner syndrome
p. 2446 table 134-2
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c. dyskeratosis congenita
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What is the most common site for cutaneous metastasis?
a. scalp
b. face
c. genitals
d. thorax
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d. thorax
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What is the most frequent clinical manifestation of Trousseau syndrome?
a. venous thromboembolism
b. pulmonary embolism
c. superficial migratory thrombophlebitis
d. marantic endocarditis
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a. venous thromboembolism
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# Name the major internal malignancy associated with the following disease
Acanthosis nigricans
gastric CA
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# Name the major internal malignancy associated with the following disease
Acquired ichthyosis
Hodgkin lymphoma
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# Name the major internal malignancy associated with the following disease
Pityriasis rotunda
hepatocellular CA
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# Name the major internal malignancy associated with the following disease
Tripe palms
Most common: Lung CA
2nd: Gastric CA
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# Name the major internal malignancy associated with the following disease
Leser-Trélat sign
Most common: GI CA
2nd: lymphoproliferative disorders
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# Name the major internal malignancy associated with the following disease
Bazex syndrome
Squamous cell carcinoma (SCC) of upper aerodigestive tract
## Footnote
* The second most common is lung cancer.
* Other less-common tumor locations are genitourinary and lower GI tract
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# Name the major internal malignancy associated with the following disease
Progressive systemic sclerosis
Lung CA
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# Name the major internal malignancy associated with the following disease
Dermatomyositis
Women: ovarian and breast CA
Men: GI, respiratory tract CA
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# Name the major internal malignancy associated with the following disease
Erythema gyratum repens
Lung CA
## Footnote
Among patients with associated carcinoma, almost half of cases have lung cancer, whereas 8% have stomach cancer, 7% have esophageal cancer, and 5% have breast cancer.
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# Name the major internal malignancy associated with the following disease
Necrolytic migratory erythema
Glucagonoma (pancreatic α cell tumor)
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# Name the major internal malignancy associated with the following disease
Sweet syndrome
Acute myeloid leukemia (AML)
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# Name the major internal malignancy associated with the following disease
Pyoderma gangrenosum
Acute myeloid leukemia (AML)
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# Name the major internal malignancy associated with the following disease
Necrobiotic xanthogranuloma
Monoclonal gammopathy of undetermined significance (MGUS)
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# Name the major internal malignancy associated with the following disease
Paraneoplastic pemphigus
Most common: non-Hodgkin lymphoma
2nd most common: chronic lymphocytic leukemia (CLL)
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# Name the major internal malignancy associated with the following disease
Dermatitis herpetiformis
Non-Hodgkin lymphoma
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# Name the major internal malignancy associated with the following disease
Hypertrichosis lanuginosa acquisita
Men: lung, colorectal CA
Women: colorectal, lung, or breast CA
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# Name the major internal malignancy associated with the following disease
Trousseau syndrome
Pancreatic, brain, lung CA
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features suggestive of malignancy-associated acanthosis nigricans
* malignancy-associated acanthosis nigricans usually appears abruptly and extensively
* Mucosal involvement and generalized pruritus are more common compared to benign acanthosis nigricans
* co-occurrence with florid cutaneous papillomatosis and other paraneoplastic skin lesions, such as tripe palms and Leser-Trélat sign
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In malignancy-associated acanthosis nigricans, it is proposed that the tumor produces what substance that is similar in structure to epidermal growth factor and binds to epidermal growth factor receptors and stimulates keratinocyte proliferation, which leads to development of acanthosis nigricans
transforming growth factor-α
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Histopathology of acanthosis nigricans
* Histopathology of acanthosis nigricans classically shows hyperkeratosis and epidermal papillomatosis.
* There is only slight acanthosis and usually no hyperpigmentation.
* Horn pseudocyst formation and increased melanin pigmentation can be observed in some cases.
* The brown color of the lesion is caused by hyperkeratosis rather than melanin.
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* Skin manifestations are characteristic, consisting of perfectly round or oval, asymptomatic, well-defined, hypopigmented or hyperpigmented ichthyosiform scaly patches that appear on the trunk and proximal extremities
Pityriasis rotunda
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This type of pityriasis rotunda is associated with underlying** malignant or systemic disease** and presents with <30 skin lesions. It is more common in black and East Asian patients.
Type 1 pityriasis rotunda
## Footnote
It has been observed in association with chronic diseases, infections, and malignancies. GI (most commonly hepatocellular carcinoma) and hematologic malignancies are frequently reported among the various types of associated malignancies.
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This type of pityriasis rotunda occurs in white patients and presents with more than 30 lesions (usually hypopigmented), and is **not associated with any underlying disease.**
Type 2 or familial pityriasis rotunda
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* The palms are rough, thickened, and velvety with exaggerated dermatoglyphics.
* The texture of the ventral surface of hand and fingers may be **moss-like, cobbled, or honeycombed**
Tripe palms
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In patients with only tripe palms, what is the most common associated malignancy?
pulmonary/lung carcinoma
## Footnote
especially the squamous cell type
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In patients with both tripe palms and acanthosis nigricans, what is the most common carcinoma associated?
Gastric CA
2nd MC: lung CA
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characterized by the sudden increase in size and number of seborrheic keratoses (SKs) related to internal malignancy
Leser-Trélat sign
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Leser-Trélat sign often coexists with what skin condition?
malignant acanthosis nigricans
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history suggesting presence of seborrheic kerratosis is associated with an underlying malignancy
The rapid occurrence of pruritic, eruptive SKs, especially in the setting of acanthosis nigricans should alert the clinician to a potential internal malignancy
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pseudosign of Leser-Trélat is associated with what drugs?
cytarabine and tumor necrosis factor-α inhibitors
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aside from malignancy, Leser-Trélat sign has been reported in what conditions?
A Leser-Trélat–like eruption or eruptive SKs have been rarely reported in the setting of benign conditions, such as **pregnancy, HIV, heart transplantation, acromegaly, and erythroderma, **and in association with drugs, such as **cytarabine and tumor necrosis factor-α inhibitors.**
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* The characteristic cutaneous findings are symmetrical erythematous to violaceous **scaly patches or plaques over the acral extremities, ears, and bridge of the nose**
* Hyperpigmentation tends to appear in dark-skinned individuals.
* Vesicles and bullae are occasionally observed on the hands and feet.
* A bulbous enlargement of the distal phalanges has been described.
* The most common sites of involvement are the **nails** (77%), ears (76%), fingers (65%), nose (62%), palms (56%)/ hands (51%), and soles (49%)/feet (44%).
Acrokeratosis paraneoplastica of Bazex
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Most common site of involvement of Acrokeratosis paraneoplastica of Bazex
nail
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first sign of nail involvement in Acrokeratosis paraneoplastica of Bazex
Paronychia
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The distinguishing clinical feature, which is nearly always present in acrokeratosis paraneoplastica
involvement of the helices of the ears and the tip of the nose
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most effective therapy for Acrokeratosis paraneoplastica of Bazex
Treatment of the primary neoplasm
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These antibodies have been reported as a promising cancer marker in adult patients with DM
anti-p155 antibodies
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malignancies reported in association with dermatomyositis
* Lung, breast, ovarian, cervical, pancreatic, gastric, colorectal, and prostate malignancies are frequently reported;
* Hematologic malignancies may also occur in association with DM.
* It has been noted that nasopharyngeal carcinoma is more common in the Asian population
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* Numerous serpiginous bands are arranged in a parallel configuration of concentric red swirls over most of the body. This presentation is occasionally referred to as a “wood-grain” appearance.
* Even more striking is the relatively rapid rate at which lesions migrate (repens is Latin for creeping), estimated at 1 cm per day.
* A fine scale may be found along the trailing edge of erythema
* The hands, feet, and face are commonly spared, except for occasional volar hyperkeratosis.
Erythema gyratum repens.
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a very rare paraneoplastic skin disorder that is considered a hallmark of glucagonoma and is present in more than two-thirds of patients at the time of tumor diagnosis
necrolytic migratory erythema
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most common presenting sign of glucagonoma syndrome
Weight loss
## Footnote
* Glucagonoma syndrome is characterized by NME, weight loss, sore mouth, diarrhea, diabetes mellitus, deep vein thrombosis, normochromic normocytic anemia, and neuropsychiatric disorders
* The glucagonoma tends to grow slowly.
* Metastasis to the liver, regional lymph nodes, and bone is common, but appears in late stage of the disease.
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Monoclonal gammopathy of undetermined significance has been described by several authors as an associated condition with pyoderma gandrenosum.
What is the most ?
IgA
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* rare, systemic disease presenting with **destructive polyarthritis **and typical cutaneous lesions.
* The isolated or grouped **reddish brown to skin-colored papules and nodules** are seen predominantly on the **face and hands** with a characteristic** “coral bead” appearance **of periungual papules.
Multicentric reticulohistiocytosis (MRH)
## Footnote
* There is no predominant type of associated cancer in this disease
* The course of the disease may be self limtied without joint deformity; waxing and waning; or aggressive with mutilating arthritis.
* Spontaneous remission usually occurs after many years.
* It does not run a parallel course with the associated malignancy in the majority of patients.
* Treatment of the accompanying cancer will resolve joint and skin disease only in some reported cases.
* It is still debated among several authors whether to label multicentric reticulohistiocytosis as a paraneoplastic disorder.
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* rare, nonLangerhans cell histiocytosis with a strong association with hematologic disorders
* The skin is the most common site of involvement and more than 80% of patients have **periorbital** lesions.
* The lesions appear as yellowish to red-orange or violaceous papules, plaques, or nodules, with areas of ulceration, telangiectasia, or atrophy.
Necrobiotic xanthogranuloma
## Footnote
* The most common associated plasma cell dyscrasias are monoclonal gammopathies of undetermined significance, smoldering multiple myeloma, and multiple myeloma.
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most common associated malignancy, and is seen in approximately 20% of patients with AL amyloidosis
Multiple myeloma
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most common form of systemic amyloidosis associated with malignancy
AL amyloidosis
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* rare condition characterized by the relatively-sudden appearance of long, fine, nonpigmented lanugo (Latin for down) hairs
* The lanugo hairs most frequently appear on the face and ears early in the course
* The hairs may grow to an impressive length; eyebrows and eyelashes may grow to inches long.
* The long fine hairs also may be seen on the trunk and limbs, including the axillae, but the palms, soles, suprapubic, and genital areas are usually spared
* develops in a cephalocaudal direction and may be accompanied by glossitis, hypertrophy of tongue papillae, oral hyperpigmentation, disturbances of taste and smell, acanthosis nigricans, diarrhea, adenopathy, and weight loss
Hypertrichosis lanuginosa acquisita
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most frequent clinical manifestation of Trousseau syndrome
Venous thromboembolism (VTE)
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* occurrence of migratory thrombophlebitis associated with underlying gastric cancer
* cancer-associated hypercoagulability with a wide range of clinical manifestations, including abnormal coagulation tests without clinical symptoms, superficial migratory thrombophlebitis, deep vein thrombosis, marantic endocarditis, pulmonary embolism, and massive thromboembolic phenomenon associated with disseminated intravascular coagulation
Trousseau syndrome
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The combination of abnormal blood flow or stasis, vessel wall injury, and blood hypercoagulability contributes to thrombus formation. What do you call this triad?
Virchow triad
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The most common malignancies to metastasize to the skin in women
1. breast cancer
2. colon cancer
3. melanoma
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The most common malignancies to metastasize to the skin in men
1. Lung CA
2. Colon CA
3. Melanoma
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clinical variant of cutaneous metastasis that presents with warm, tender erythematous patches or plaques resembling erysipelas or cellulitis, but without fever or leukocytosis
carcinoma erysipelatoides
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clinical variant of cutaneous mets characterized by leather-like skin changes of sclerosing metastatic breast cancer, which may later present as nodules and ulceration
carcinoma en cuirasse
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most common site for cutaneous metastasis, as a result of the high frequency of metastatic breast and lung cancers
thorax
## Footnote
The scalp is another common site, for metastases from lung, kidney, and breast tumors
