Post on 13-Oct-2020
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)
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
Pyoderma Gangrenosum
Pyoderma Gangrenosum
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)
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
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.
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?
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
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
What are these called?
Xanthelasmas
Associations/Concern?
Normal Finding
or
Type II/III
Hyperlipoproteinemia
Eruptive Xanthomas Tuberous Xanthomas
Tendon Xanthomas
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
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
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?
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…
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
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
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
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
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?
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
Which of the following is the most likely diagnosis?A) CalciphylaxisB) Ecthyma gangrenosumC) Necrotizing fasciitisD) Pyoderma gangrenosum
Hematocrit 31%
Leukocyte count 13,000
Erythrocyte sedimentation rate 70
Serum creatinine 0.8
Urinalysis Negative
ANCA Screen Negative
A 28 year old male presents with low back and bilateral knee pain 2 weeks after “food poisoning”
Erythema gyratum repens
Virtually always associated
with internal malignancy,
usually lung, breast,
stomach, bladder