A 62 year old Asian Male Sees you for sudden onset of the ... · What does this mean? Extensive...

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A 62 year old Asian Male Sees you for sudden onset of the following skin lesions on his face and back What are these lesions? Seborrheic keratoses What is this Called? The Sign of Leser-Trelat Concern Carcinoma of GI Tract (Also Lymphomas, Ovarian Cancers)

Transcript of A 62 year old Asian Male Sees you for sudden onset of the ... · What does this mean? Extensive...

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A 62 year old Asian Male Sees

you for sudden onset of the

following skin lesions on his

face and back

What are these lesions?

Seborrheic keratoses

What is this Called?

The Sign of Leser-Trelat

Concern

Carcinoma of GI Tract

(Also Lymphomas, Ovarian

Cancers)

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A 27 year old WM presents with a boil on his Left Leg that started

bleeding 2 weeks prior. Since that time, it has grown rapidly over 2

weeks. He complains of severe pain in the leg, fever, malaise and 10

pounds of weight loss during the past month. Thoughts??

Pyoderma Gangrenosum

Workup/Confirm with skin biopsy and culture

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Pyoderma Gangrenosum

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Pyoderma Gangrenosum

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Pyoderma Gangrenosum

Major:

• History of rapidly progressing, painful ulcer with irregular, violaceous,

undermined border

• Exclusion of other causes of ulcerations (typically vasculitis, neoplasm,

infection)

Minor:

• Skin biopsy showing sterile neutrophilic inflammation

• Presence of pathergy or healing with cribriform scarring

• Presence of systemic diseases associated with pyoderma gangrenosum

(inflammatory bowel disease, IgA paraproteinemia, internal malignancy,

systemic lupus erythematosus, or other autoimmune disease)

• Response to systemic glucocorticoid therapy (1-2 mg/kg/d, anticipate 50%

size decrease within 4 weeks)

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PG: What are the disease associations?

PG: What is the treatment (besides treating the underlying disorder?)

Inflammatory Bowel Disease (UC>CD)

Chronic Hepatitis

Rheumatoid Arthritis

HIV Infection

Leukemias (esp Myeloid)

Paraproteinemias

Wound Care (avoid debridement!!!!)

Topical/Intralesional steroids

Oral Steroids +/ Steroid Sparing Drugs & Cyclosporine/Infliximab

Evidence of utility with dapsone or colchicine

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What is this called when very pruritic in a patient

with diarrhea and iron deficiency anemia?

Dermatitis herpetiformis

Disease Association?

Celiac Disease

What are the other findings with Celiac disease?

Diarrhea, Iron Deficiency Anemia, Osteopenia,

Abnormal LFTs

How do you diagnose this disease?

Screen with tTG -> Small Bowel Bx -> If negative

or conflicting consider other markers/HLA

haplotyping

How do you treat patients?

Gluten-free diet. With Relapse consider other dx.

Complications?

Patients have increased risk of Lymphomas and

Adenocarcinomas of the GI tract.

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A 38 year old man with a history of

supernumerary teeth, s/p extraction in

childhood, and OMFS Surgery at the

age of 19 for osteoma of the jaw

presents to you for complaint of

dizziness, fatigue and dyspnea on

exertion. He has conjuctival pallor and

nail spooning. Skin Exam is shown.

Thoughts?

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Gardner syndrome.

Tell me about this…..

-Form of FAP with extensive

adenomatous polyps

-Epidermoid Cysts

-Lipomas

-Osteomas (over 50%)

-Supernumerary Teeth

What do You Refer this Patient for?

Colonoscopy - Will likely Need

Colectomy

Thyroid U/S

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You notice the following on a 18

year old HM that the ER at LAC-

USC is admitting for workup of

abdominal pain and

LGIB/melenda

What is your Concern?

Peutz-Jeghers syndrome

What does this mean?

Extensive hamartomatous polyps

throughout the GI tract (esp SI)What is the overall concern in these patients?

•Intuccesseption from the polyps

•Slightly Increased risk of malignant potential of these polyps

•Increased risk of pancreatic cancer, breast cancer, and cervical, ovarian, and testicular cancers

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What are these called?

Xanthelasmas

Associations/Concern?

Normal Finding

or

Type II/III

Hyperlipoproteinemia

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Eruptive Xanthomas Tuberous Xanthomas

Tendon Xanthomas

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A 22 year old Male

presents to you with

difficult to control HTN,

hypocalcemia, a distinct

non-tender hard thyroid

mass and >20 lbs weight

loss.

Facial exam :

What is your concern?

Medullary Thyroid Carcinoma +

Pheochromocytoma + Mucosal Neuromas

=MEN IIB

Other Findings?

Marfanoid Habitus

Intestinal ganglioneuromatosis

Hyperparathyroidism can be present

Workup in general MENIIB?

RET Oncogene Testing and genetic

counseling.

Screen for Pheochromocytoma

Thyroid Surgery

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A 23 year old noticed the acute

onset of these lesions on the

hand. They are “burning” and

not pruritic. He also complains of

low-grade fevers and fatigue

What is this called?

Erythema Multiform (Minor)

What two infections are associated with this skin condition?

HSV and Mycoplasms

What Drugs can cause this to occur?

Dilantin, NSAIDs, PCN, Sulfas

Treatment

Remove the offending agent. Acyclovir

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A 75 year old male presents with a chief

complaint of dizziness, dyspnea, as well as lower

extremity paresthesias and edema. Exam reveals

a blood pressure of 100/60 with a positive

orthostatic change. He has the skin finding to the

right. Noted is Hepatomegaly, bilateral crackles

as well as 2+ pitting edema.

Ua shows +1 protein with a Pr/Cr ratio of 4.7

CBC reveals a normocytic anemia

Alk Phos is 450 with Normal Bili and Transaminases.

Total Protein 8.5 with Albumin 2.6

BMP notable only for BUN 22 with Cr of 3.9 and

Ca 12.5

Echo reveals diastolic dysfunction and a thick

septum.

What is your suspicion?

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What are the causes?

AL: Light Chain Disease (Most Common)

AA: RA, TB, Osteo

What are the common clinical findings?

Nephrotic range Proteinuria

Decreased GFR

Restrive Cardiomyopathy

Hepatomegaly

Distal Sensimotor neuropathy/CTS/Orthostasis

GI Dysmotility

Malabsorption

Arthritis, Muscle Pain

Bleeding

Other Skin Manifestations…

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A 46 year old Man presents with a complaint of blistering when his skin has

prolonged exposure to the sun, and new hair growth in areas of prolonged

sun exposure, predominately on the extremities. He also states that any

trauma to his skin causes blisters that easily break.

What is your diagnosis?

Porphyria Cutanea Tarda (deficiency in hepatic uroporphyrinogen

decarboxylase)

What are the underlying

diseases that cause this?

Hepatitis (C>B)

Hemochromatosis

HIV

Alcoholism w/Cirrhosis

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A 46 year old Man presents with a complaint of blistering when his skin as

prolonged exposure to the sun, and new hair growth in areas of prolonged

sun exposure, predominately on the extremities. He also states that any

trauma to his skin causes blisters that easily break.

How to Dx?

24 Hour Urine porphyrins

are highly increased

uroporphyrins >

coproporphyrins

Skin biopsy is potentially

non-specific (pauci-

immune subepidermal

bullae)

Tx?

Tx Underlying Disease

Phlebotomy +/- HCQ

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Must distinguish PCT from this:

Skin complaints are exactly

the same as PCT, but patients

with will have other symptoms

such as abdominal pain,

nausea, vomiting and motor

neuropathy.

Variegate porphyria (aka

Mixed” porphyria)

Dx?

24 Hour Urine porphyrins are

only slightly increased

But Coproporphyrins>

uroporphyrins

Tx? IV Glucose/Hematin

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A 42 year old female with Graves Disease and notable ophthalmologic

involvement, is admitted to LAC for Atrial Fibrillation and symptoms of CHF.

What are Some of the cutaneous manifestations of Thyrotoxicosis?

Velvety Skin

Increasing Palm/Sole Sweating

Alopecia

Fast-Growing Nails

Distal Oncholysis

Pruritis

Urticaria

Palmar Erythema

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A 42 year old female with Graves Disease and notable ophthalmologic

involvement, is admitted to LAC for Atrial Fibrillation and symptoms of CHF.

What are Some of the cutaneous manifestions of Grave’s Disease?

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Switching Gears, what about cutaneous signs of Hypothyroidism?

Puffy facies with bepharoptosis

Dry skin that can progress to eczema

Nonpitting edema (early) of hand, face and

ankles that can progress to pitting edema

Pallor

“Yellowish” Skin secondary to carotonemia

Coarse brittle hair and hair loss

Brittle striated nails

Enlarged Tongue

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Which of the following is the most likely diagnosis?A) CalciphylaxisB) Ecthyma gangrenosumC) Necrotizing fasciitisD) Pyoderma gangrenosum

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Hematocrit 31%

Leukocyte count 13,000

Erythrocyte sedimentation rate 70

Serum creatinine 0.8

Urinalysis Negative

ANCA Screen Negative

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A 28 year old male presents with low back and bilateral knee pain 2 weeks after “food poisoning”

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Erythema gyratum repens

Virtually always associated

with internal malignancy,

usually lung, breast,

stomach, bladder

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