Paraneoplastic Syndromes

Question 1

Paraneoplastic Syndromes:

Answer 1

• Acanthosis Nigricans
• Leser-Trelat
• Tripe Palm
• Acquired Ichthyosis
• Acrokeratosis Paraneoplastica
• Papulosquamous
• Dermatomyositis
• Erythema Gyratum Repens
• Necrolytic Migratory Erythema
• Hypertrichosis lanuginosa acquisita

Question 2

Acanthosis Nigricans:

Answer 2

• Flexural folds
• New onset in non-obese adult or elderly may be sign of malignancy
• GI Adenocarcinomas

Question 3

Leser-Trelat:

Answer 3

• Abrupt increase in size/number of Seborrheic Keratoses

- Gastric/colon adenocarcinoma, breast carcinoma, lymphoma

Question 4

Tripe Palm:

Answer 4

• Form of palmoplantar keratoderma
• Thick skin of palms with rugose appearance
• Stomach, GU lung malignancies

Question 5

Acquired Ichthyosis:

Answer 5

• Generalized skin scaling

- Hodgkin Lyphoma

Question 6

Acrokeratosis paraneoplastica:

Answer 6

• Upper respiratory or GI tract malignancy
• Brittle nails with chronic paronychia
• Psoriatic like lesions on fingers

Question 7

Paget’s Disease:

Answer 7

• Malignant intraepithelial condition of nipple and skin around nipple
• BREAST CARCINOMA

Question 8

Erythema Gyratum Repens:

Answer 8

• Due to cross reactivity of tumor antigens with antigens in the skin
• Presents prior to internal malignancy detection
• Pruritic annular erythematous eruption whose edges advance at rapid rate

Question 9

Necrolytic Migratory Erythema:

Answer 9

• GLUCAGON PRODUCING TUMORS OF PANCREAS
• Weight loss fever abdominal pain
• Improves after surgery to remove pancreatic lesion

Question 10

Hypertrichosis Lanuginosa Acquisita:

Answer 10

• Malignant Down Hair
• Affecting areas of skin with no hair normally
• Lung Breast Colorectal Carcinoma

Question 11

Genodermatoses:

Answer 11

• Cowden’s Syndrome
• Gardner’s Syndrome
• Peutz-Jeuger’s Syndrome
• Xeroderma Pigmentosum
• Neurofibromatosis
• Tuberous Sclerosis

Question 12

Cowden’s Syndrome:

Answer 12

• Mutation of PTEN gene
• Multiple Hamartoma and neoplasia syndrome
• Skin lesions -> oral mucosa, face, acral area, palms, soles

Question 13

Gardner Syndrome:

Answer 13

• Mutation of APC gene
• AKA familial polyposis of colon
• Extraintestinal manifestations: Epidermoid cyst, osteoma, desmoid tumor, fibrous tumors
• Congenital hyperpigmentation of the retinal pigment

Question 14

Peutz-Jegher’s Syndrome:

Answer 14

• Autosomal dominant
• Perioral lentiginosis
• Permanent freckles around mouth lips buccal mucosa and multiple intestinal polyps

Question 15

Xeroderma Pigmentosum:

Answer 15

• Autosomal recessive
• Nucleotide Excision Repair disease
• Cannot repair UV-induced damage

Question 16

Neurofibromatosis Type 1:

Answer 16

• Tumor suppressor gene on chromosome 17 mutation
• Cafe-au-lait macules
• Axillary and groin freckling are pathognomonic
• Neurofibromas
• Lisch Nodules -> Iris hamartomas
• Sphenoid Dysplasia

Question 17

Neurofibramatosis Type 2:

Answer 17

• Tumor suppressor gene on chromosome 22 mutated
• Less cafe-au-lait macules
• NO LISCH NODULES IN IRIS
• Acoustic Neuromas

Question 18

Tuberous Sclerosis:

Answer 18

• Multi system autosomal dominant disorder
• Hamartin and Tuberin are the mutated genes
• Hamartomas in every organ
• Vogt Triad: Angiofibromas, Epilepsy, Intellectual Disability
• Shagreen patches: orange peel apperance
• White ash leaf macules
• Periungual fibromas
• Angiofibromas: pink or red papules with smooth surface located over nasolabial folds cheeks and chin
• Fibrous forehead plaque