Cutaneous Manifestations w/ Systemic Disease Flashcards by Nadia Ahamed

MC associated malignancy w/ cryoglobulinemia?

Multiple Myeloma

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MC hepatitis associated w/ mixed cryoglobulinemia?

HCV

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A pt presents with fragile skin w/ sclerodermoid changes. What should be part of routine investigative work up in pts w/ porphyria cutanea tarda?

HCV serology

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A pt presents with purple, pruritic, polygonal papules and Wickham’s striae. most likely dx?

Lichen Planus

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MC hepatitis associated w/ polyarteritis nodosa?

HBV

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Excoriations, lichenification, and prurigo nodularis describe what?

Pruritis

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Classical PAN, renal vasculitis, ANCA negative all point to what dx?

Hep. B

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A pt presents w/ palmar erythema, gynecomastia, and multiple xanthomas in their palmar creases. What can this indicate?

Chronic liver disease

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which hormone plays a pivotal role in the growth and formation of hair and sebum production?

thyroid hormone

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What is the MC cystic abnormality of the neck? It presents as a mobile, nontender mass on the midline, anterior neck and is an embryonic duct remnant.

Thyroglossal duct cyst

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Skin is usually ___, ___, and ____

warm, moist, and smooth

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Hyperhydrosis, hyper pigmentation in the creases of palms and soles but no buccal pigmentation can point to?

hyperthyroidism

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most important non-specific cutaneous manifestations of hyperthyroidism?

1) localized/generalized hypertrichosis
2) plummer nails
3) vitiligo

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What disease is associated w/ thyroid dermopathy and symmetric, nonpitting yellow-brown waxy papules/plaques and is due to increased hyaluronic acid?

Graves disease

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What nail condition is described as concave contour and distal onycholysis?

Plummer’s nail in hyperthyroidism

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Clubbing of the fingers and toes in Graves’ disease associated w/ soft-tissue swelling of hands and feet and w/ periosteal new bone formation is termed:

thyroid acropachy

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What describes the process in which binding of excess organic iodide in the thyroid gland results in inhibition of thyroid hormone synthesis occurring in pts w/ erythema nodosum being tx w/ potassium iodide?

wolff-chaikoff effect

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Numerous firm white, yellow, or pink papules on face, trunk, axillae, and extremities describe..?

scleromyxedema in hyperthyroidism

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Non-specific cutaneous manifestations of hypothyroidism include:

1) yellowish hue secondary to carotenemia
2) generalized myxedema (swollen waxy appearance
3) Madarosis (loss of lat. 3rd of eyebrow)

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A pt presents with bruising, brittle nails, and purpura. What can this indicate?

Hypothyroidism

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What syndrome is autosomal dominant and is characterized by medullary thyroid carcinoma, pheochromocytoma, marfanoid features, and GI ganglioneuromatosis?

MEN 2b/III

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MC skin finding in MEN 2b/III?

mucosal neuromas

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What is known as sipple syndrome and is associated with macular amyloidosis?

MEN 2A

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Autosomal dominant and is associated w/ mucosal neuroma?

MEN 2b

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Earliest cutaneous finding seen in tubular sclerosis?

hypopigmented (ash-leaf) macules

MC renal finding in tubular sclerosis?

angiomyolipomas

MC ocular findings in tubular sclerosis?

Phakoma (retinal hamartoma)

MC cutaneous finding in Fabry's diease?

angiokeratomas

MC ocular finding in Fabry's disease?

whorl corneal opacities

nail manifestation of cirrhosis or hypoalbuminemia resulting in whitening of the proximal 2/3, while the distal 1/3 is red?

terry's nail

MC cutaneous manifestation of end-stage renal disease?

Pruritus

What is associated with high mortality and abnormally elevated levels of PTH? Pt would present with violaceous mottling of the skin that's acutely painful due to ischemia

Calcific Uremic Arteriolopathy | Calciphylaxis

Uremic frost consist of?

white or yellowish coating or urea crystals on the beard area & other parts face, neck, or trunk note: BUN would be increased

Nail manifestation of ESRD caused by edema of the nail bed and capillary network giving the proximal 1/2 of the nail on opaque white appearance?

Lindsay's nails

Dapsone & lasiks are usually the MC cause of this?

Pseudoporphyria

disease associated w/ malar erythema, discoid plaques, photosensitivity, raynaud's phenomenon, and periungal erythema?

SLE (systemic lupus erythematous)

which disease is associated w/ "mask like facies" and smoothening of lines of facial expressions?

systemic sclerosis

what disease presents with palpable purpura, gingival hyperplasia, and livedo reticularis?

wegener's granulomatosis

type 1 HHT is associated with a mutation in?

TGF-B

type 2 HHT is associated w/ a mutation in?

ALK-1

MC initial presenting sx in Osler-Weber-Rendu syndrome/HHT?

Epistaxis

What disease is associated with HHV-8 and primary effusion lymphoma, solid lymphoma, and castleman's disease?

Kaposi's sarcoma

What disease is associated with palpable purpura, mainly in children, and chronic kidney disease?

Henoch Sconlein purpura

MC location for hamartomatous polyps in the GI tract seen in Peutz-Jeghers syndrome?

Small intestine

Cutaneous findings in Peutz-Jeghers syndrome?

Lentigines on mucosa; periorificial skin, palms and digits

Autosomal dominant disorder and variant of Lynch syndrome?

Muir-Torre syndrome

MC malignancy associated w/ Muir-Torre?

Colon adenocarcinoma; | pts should be screened at 25 y/o, every 2-3 yrs

MC location of colon adenocarcinoma?

right-sided colon

symmetric, grouped vesicles on extensors and very pruritic and most commonly includes gluten sensitive enteropathy?

dermatitis herpetiformis

MC cutaneous manifestation of inflammatory bowel disease is?

Erythema nodosum

Tender red nodules on the anterior lower legs is known as?

erythema nodosum

MC IBD associated w/ erythema nodosum?

Ulcerative colitis; more common in females

Linear ulcerations, cobblestoned oral mucosa is indicative to?

oral crohn's

MC IBD associated with chronic apthous ulcers or pyostomatitis vegetans?

Ulcerative colitis

MC IBD associated with pyoderma gangrenosum?

Ulcerative colitis

What do you tx pyoderma gangrenosum with?

steroids

what disease is a defect in neurofibromin and has crowe's sign?

Neurofibromatosis 1 (AD)

disease associated with mutation in ATM gene and present w/ Ataxia?

Ataxia-Telangectasia (AR)

disease associated w/ premature aging, photosensitivity, and mutation in XP A-G?

Xeroderma Pigmentosum (AR)

Associated w/ mutation in NEMO gene involves peg-shaped teeth?

Incontinentia Pigmenti (XLD)

Associated w/ C-KIT, white forelock and impaired motor coordination?

Piebaldism (AD)

Syndrome that presents congenital ichthyosis, di/tri tetraplegia, MR. Also associated w/ a scaly, spastic, seizing swede?

Sjorgen-Larson Syndrome (AR)

Mutation in ATP7A, presents w/ doughy skin, kinky hair, severe psychomotor retardation and seizures?

Menke's disease (XLR)

difference in lesions for type I vs type II diabetes?

type I--> autoimmune lesions | type II--> cutaneous infxns

MC endocrine abnormality associated w/ acanthosis nigricans?

diabetes mellitus

MC associated malignancy w/ acanthosis nigricans?

gastric carcinoma

Skin disorder seen in DM type II chracterized by painless, symmetric, woody "peau d'orange" induration of the upper back and neck?

scleredema diabeticorum

Other than uncontrolled diabetes, what other settings may eruptive xanthomas occur?

- hypertriglyceridemia (>2000 mg/dl) - Meds: retinoids, estrogens - Alcohol

What are other conditions associated w/ diabetes?

- vitiligo - lichen planus - hemochromatosis

White, curd-like material adherent to erythematous, fissured oral commisure depicts?

angular cheilitis

What is a common skin infxn in DM and is caused by pseudomonas aeruginosa?

malignant otitis externa

What describes sharply demarcated erythematous patches and is caused by gram + corynebacterium minutissimum?

erythasma

syndrome associated w/ defect in fibrillin 1 and upward lens dislocation?

marfan's syndrome

Disease associated w/ downward lens dislocation and elevated homocysteine?

Homocystinuria (AR)

Disease associated w/ defect in COL3A1 and translucent skin/easy bruising?

Ehlers-Danlos IV (AD)

AD syndrome associated w/ defect in PTPN11. Presents w/ Lentinges, Ekg changes, Ocular hypertelorism, Pulmonic stenosis, Arterial abnormalities, Retarded growth, Deafness

LEOPARD syndrome (AD)

Paraneoplastic dermatoses associated w/ growth of fine lanugo hairs and is MC associated w/ lung carcinoma?

hypertrichosis lanuginosa

MC associated malignancy w/ acquired ichthyosis?

Hodgkins Lymphoma | - dx made after mlaignancy

MC associated malignancy w/ erythema gyratum repens:

lung carcinoma

"wood grain" concentric erythematous bands w/ trailing edge of scale describes what condition?

erythema gyratum repens

What syndrome is caused by prolonged exposure to elevated levels of either endogenous glucocorticoids or exogenous glucocorticoids? Pt presents w/ buffalo hump and striae!

Cushing syndrome

MC associated malignancy associated w/ cushing syndrome?

Oat cell lung carcinoma

Pt presents w/ flushing and erythema of head & neck and is dx with symptomatic carcinoid syndrome. MC associated malignancy?

ileal tumors

MC associated malignancy w/ asymptomatic carcinoid syndrome?

neoplasm originating in the endocrine argentaffin cells of the appendix

MC location of metastasis of carcinoid syndrome-causing tumors?

Liver

Syndrome MC associated w/ AML and presents w/ erythematous tender papules, nodules, and plaques with marked lesional edema?

sweet's syndrome

(T/F) Malignancy associated sweet syndrome occurs in individuals w/ previously undiagnosed or relapsing hematologic malignancies (10-20%) and solid tumors

TRUE

Associated malignancies associated w/ cryoglobulinemia?

multiple myeloma & waldrenstroms macroglobulinemia

sudden development of numerous seborrheic keratoses and MC associated malignancy is gastric or colon carcinomas?

Lesser Trelat sign

Which subtype of systemic amyloidosis is most commonly associated w/ malignancy (i.e: multiple myeloma) and presents w/ pinched purpura?

AL subtype

Seen in Sezary syndrome, lyphomas, and leukemias:

Erythrodema

condition associated w/ MGUS?

scleromyxedema

Can be a marker for internal neoplasia and most commonly associated w/ ovarian cancer (women)? Pt presents w/ Shawl and Holster sign

dermatomyositis

MC associated malignancy w/ dermatomyositis in men:

gastric carcinoma