Chapter 7 Flashcards
Objective-not fail
Which of the following can induce menopausal related flushing: A. Oophorectomy B. Tamoxifen C. Leuprolide Acetate D. Hysterectomy E. A-C F. All the above
E
List medications associated with flushing:
Niacin CCB Cyclosporine Chemo Vancomycin Bromocriptine IV contrast ED medications Methylprednisolone Combo of SSRI + MOI (Serotonin Toxicity) Anesthesia Meds: Thiopental and Muscle Relaxants
What labs should be ordered if an endogenous cause is suspected in flushing case?
- Urine catecholamins 2. Serotonin 3. Histamine metabolites
Treatment for idiopathic flushing
Avoid triggers and sipping iced water to break flush -Avoid alcohol, caffiene, and spicy foods
Treatment for menopausal flushing
Low dose oral/transdermal estrogen
List endogenous causes of flushing
- Carcinoid Syndrome 2. Mastocytosis 3. Medullary thyroid carcinoma 4. Pheo
Niacin induced flushing is mediated by _____
Prostaglandin D2 -Should respond to ASA or laropiprant
Diagnosis.
Erythema Palmare
AKA Persistent palmar erythema
Note: Usually ost marked on the hypothenar areas and associated with elevated estrogen levels
List common causes of erythema palmare
- Cirrhosis
- Hepatic metastases
- Pregnancy
What labs should be performed to confirm diagnosis of erythema toxicum neonatorum
Smears of the pustules will demonstrate eosinophils
-Biopsy RARELY REQUIRED (would show folliculitis containing eosinophils and neutrophils)
Diagnoses
Erythema toxicum neonatorum
Describe typical presentation of erythema toxicum neonatorum
2nd or 3rd of newborns life–present with broad erythematous flare on face, trunk, and proximal extremities
- Confluent erythema on the face
- small folicular papules/pustules
*usually disappears by tenth day
Diagnosis?
Erythema Toxicum neonatorum
Describe the 3 zones seen in the classic EM lesion
- Central dusky purpura
- Elevated edematous, pale ring
- Surrounding macular erythema
42 y/o female presents with a solitary unilateral erythematous plaques with a dusky center. The plaques are about 14 cm in diameter. Pt also c/o new bumps on her lower extremities. On exam the nodules are deep seated and tender.
What is the diagnosis?
Atypical variant of EM
Note: can present with erythema nodosum like lesions
True or False?
Acyclovir suppression increases the frequency of lesions, while prednisone therapy prevents the lesions
FALSE.
Correct response is: Acyclovir suppression prevents the lesions and prednisone therapy seems to increase the frequency of attacks
From a histology standpoint, what factors determine the extent of epidermal involvment of EM
- Duration of lesion
- Where in lesion biopsy was taken
Treatment for HSV associated EM
- Antivirals
- Sunscreen/Sunscreen lip balm daily to prevent UVB induced outbreaks
- (If above txt fails or genital HSV is the cause) Chronic suppressive doses of an oral antiviral drug should be used
-Valacyclovir 1g/day
-Famiciclovir 250mg/day
- If above dose is ineffective–double it
Treatment for EM pt who fail to respond to antiviral suppression
- Dapsone
- Cyclosporine
- Thalidomide
Note: Most cases of HAEM are self limited and symptomatic treatment may be all that is required
Treatment for oral lesions of EM
Topical “swish and spit” of mixtures containing lidocaine, diphydramine, and kaolin
How to treat widespread EM unresponsive to first/second line therapies?
Management is same as for severe drug-induced SJS
Diagnosis
Urticaria multiforme
How to distinguish EM from SJS based on morphology presentation
SJS presents with purpura or bullae in macular lesions of the trunk vs. erythematous macules that evolve over 24-48h into edematous papules/Centrally lesions become flatter, more purpuric and dusky forming the classic target lesions of EM
Diagnosis
EM secondary to sulfa
Diagnosis
EM
Diagnosis
EM
Diagnosis
Diagnosis
EM
Diagnosis
Diagnosis?
EM
10 y/o pt presents with recurrent episodes of hive like lesions. Per mom the lesions change colors in the middle. See attached image.
Urticaria Multiforme–it is a type of urticaria, histology never shows EM
-commonly misdiagnosed as EM
41 y/o female presents to clinic with cc of sore in mouth. She has previously been seen and treated with unknown meds by PCP and the lesions cleared but they have come and gone several times. On exam, pt has erythematous, eroded ulcers on the tongue and buccal mucosa. What is the treatment for her diagnosis?
Diagnosis is Oral EM
Treatment: Treat like HAEM with antivirals, sunscreen if lip involvment, and swish-spit with lidocaine/diphenydramine/kaolin for symtompatic relief
What should you counsel patient on if you rx swish-spit treatment for EM?
Advise pt to chew carefully because the anesthetic effect ay dampen their gag reflex aka choking hazard
True or False: In 45% of oral EM cases the oral cavity is the only site involved and 30% of cases both the oral cavity and lip are involved
True
Note: In 25% of oral EM cases the skin is involved
Diagnosis
Recurrent oral EM
Diagnosis
Recurrent oral EM
Based on the pictures, what portions of the oral cavity are MC involved in this diagnosis?
Tongue, gingiva, and buccal mucosa
What is the typical histologic findings for EAC
Epidermis: mild focal spongiosis and parakeratosis
Dermis: Lymphocytes are organized tightly around blood vessels forming a pattern known as coat sleeve arrangement
Pt presents to clinic with c/o rash. States the rash is on his stomach and top of his thighs and has been “off and on” over the past 7 months. On exam, pt has multiple annular erythematous that are about 5-8cm in diameter. Pt denies any symptoms related to rash. What is the appropriate treatment?
Diagnosis: EAC
Txt: While active, will respond to steroids or calcipotriol. Eventually spontaneously resolves
When diagnosing a patient with EAC what important considerations should you make?
Most cases are idiopathic. Some are associated with dermatophytosis or ingestion of molds (think blue cheese and tomatoes). Can also be due to medications and internal malignancy
Diagnosis
EAC
What is the diagnosis based on the picture? What is the characteristic finding with this disorder
EAC; trailing scale at the inner border of the annular erythema
Diagnosis
EAC
What is the MC malignancy associated with the rash seen in picture?
Lung Cancer
Note: Eruption precedes the detection of malignancy be anaverage of 9 months
treatment for erythema gyratum repens
If carcinoma is treated, lesions clear. Otherwise, eruption generally resistant to treatment .
Have been cases with Cetirizine and Topical corticosteroids helping.
Besides internal malignancy, what other etiologies have been implicated for EGR? And how do you treat EGR when it is caused by them.
- Drug induced
- Secondary to pulm TB
- D/c medication or treat underlying condition to resolve rash
Pt presents with new onset rash that he says has been moving daily. On exam pt has erythematous concentric lesions with a trailing scale over his entire body. Pt c/o of severe itching. What is the diagnosis?
Erythema Gyratum Repens
Note: “Characteristically concentric, giving the skin a “wood grain” appearance
38 y/o female presents to office with rash. She has no pertinent personal, social, or family history. On exam, you see the attached picture of the patients trunk. What is the diagnosis
Eosinophilic annular erythema
Note: Usually presents on trunk and is symmetric; favors females
What conditions are associated with eosinophlic annular erythema?
None
What is the histology findings in eosinophilic annular erthema
dense perivascular and interstitial lymphocytic infiltrate with many eosinophils (without flame figures)
What is the treatment for eosinophilic annular erythema?
Spontaneous resolution can occur.
Hydroxychloroquine and/or prednisone when needed
What is the classic finding on histology for well’s syndrome and what causes it?
Flame figures (dermal collagen with adherent eosinophil granules) due to dermal eosinophil degranulation
What is believed to cause MOST cases of eosinophilic cellulitis
Arthropod reactions
-Also been associated with various disease states such as: varicella, mumps, parasties, immunizations, drug reaction, IBD, etc
How would you treat this case? See pic.
Well’s Syndrome AKA Eosinophilic Cellulitis
- Any triggering factor (arthropods) should be eliminated
- Topical/Intralesional steroids
- PO antihistamines
- Tacrolimus ointment
- Minocycline
- UVB or PUVA
- Dapsone
- Low dose prednisone
- Note: Although some cases may be due to exposure to TNF-a inhibitors–reports have shown adalimumab to work well
Being such a smart PA you know that patients that present with reactive neutrophilic dermatoses tend to have simultaneous or sequential appearance of 2 or more of the conditions. What is the MC overlap?
Combo of typical sweet syndrome on upper body and erythema nodosum-like lesions on the legs
-In these patients, histo often enables a diagnosis of subq sweet’s syndrome on the EN like lesions
What is another name for Sweet’s Syndrome?
Acute Febrile Neutrophilic Dermatosis
Of the 4 subtypes of sweet’s syndrome which is the second most frequently occuring?
Associated with inflammatory disease (16%)
Note: MC is Classic type (71%); Associated with neoplasia in 11% of cases, and associated with prego in 2%
50% of patients with Pyoderma gangrenosum have an associated dz. What is the most common dz and tx of PG?
IBD (both crohns & uc)
Tx: surgical removal of diseased intestine
Most common types of PG associated with IBD
ulcerative and pustular
What is bullous sweet syndrome?
more superficial and less destructive type of PG (bullous PG)
seen in pts with leukemia or PCV
red plaques become dusky and develop superficial erosions
50 year old man comes into RDA with this on his arm. His disease seems to be progressing agressively. How do you diagnose and treat?
PG is a diagnosis of exclusion
(bx shows supperative folliculitis- NOT diagnostic)
Agressive dz = start strong tx with immunosuppressive agent
cyclosporine 5-10mg/kg/d
infliximab IV 5mg/kg
What is the most common inherited autoinflammatory syndrome
FMF = familial mediterranean fever
autosomal recessive
recurrent attacks of 12-72h fever and monoarthritis with erythema
mutation in MEFV gene
mainstay tx: colchicine
resistant cases: rilonacept
PAPA syndrome stands for
Pyogenic sterile Arthritis, PG, and Acne
autosomal dominant
caused by PSTPIP1 or CD2BP1 gene mutations
TRAPS is similar to FMF but shows autosomal dominant inheritance, has longer bouts and does not respond to colchicine. Having eaten a smartie this morning you know that TRAPS responds to?
- acute treatment = NSAIDs or prednisone
- prevention = anti-TNF receptor antagonists or anakinra
What is the difference between annular and nummular
annular = ring with central clearing
nummular = coin shaped lesion
A 29 year old patient comes in with urticaria and a fever. After telling the front desk that you hate rashes, you find out that the urticaria has occured for 7 weeks, doesn’t itch, and has disabiling bone pain. Diagnose and treat.
Schnitzler syndrome
anakinra
rituximab
tocilizumab
rilonacept
canakinumab
Urticaria d/t food allergens is seen in which foods. (Name 3)
- chocolate
- shellfish
- nuts / peanuts
- tomatoes, strawberries, melons, pork, cheese, garlic, eggs, milk, spices
Which foods are shown to have cross-reaction with latex allergy
True or False: In sweet’s syndrome, lesions that present as dusky bullous and necrotic lesions that overlap pyoderma gangrenosum are more common in patients with associated lymphomas
False
-MC in patients with associated leukemias
What is the treatment for Sweet’s Associated with Pregnancy and what patient education should you provide?
Treatment: Can spontaneously resolve or clear with topical/systemic steroids
Education: No risk to fetus and could recur in future prego
A 42 y/o female presents to the office with a c/o a rapidly changing painful rash. When you start to interview the pt. she states that she has been “feeling feverish recently” and complains of redness and pain that feels like pressure in her right eye.
On exam: Pt has multiple erythematous elevated plaques ranging from 3-8 cm in size located on her neck, chest, and arms. Some of the lesions are violaceous in color and have vesicles on the surface. Pt’s right eye is erythematous and edematous.
What is your diagnosis?
Classic Type Sweet’s Syndrome
Hints to Dx: Primary lesions are sharply marginated erythematous/violaceous and rapidly extending. Lesions are tender/painful.
- 3/4 of patients have systemic findings. MC is fever (which she has). Can have various ocular findings–one of which is Dacyroadenitis (her secondary/assoc dx)
- Sweet’s is MC in adult females
What are some associated systemic findings with Sweets Syndrome?
- MC is fever
- Musculoskeletal: Arthritis, Arthralgia, Myalgia
- Ocular: MC are conjuctivitis and episcleritis
- Oral: Apthae ulcers
- Pulmonary: Cough, dyspnea, pleuritis
True or False: Patients with Sweet’s Syndrome associated with neoplasia, specifically hematologic malignancy, are more likely to have oral lesions resembling apthae.
True
-Lesions are found in 10% of these pts vs. 2-3% in classic type
In Sweets Syndrome, patients presenting with pulmonary complaints will likely have what on their CXR?
Effusions and pulmonary infiltrates
Pt presents to clinic for f/u 7 weeks after diagnosis of classic sweet’s syndrome. Patient is upset because she is still getting new lesions before the old lesions have a chance to clear. What should you tell this patient about her disorder?
Persistent cases of Sweet’s (which typically have new lesions eruption before old lesions resolve) may continue for YEARS.
Note: Most cases resolve in 3-6 weeks but can recur due to same precipitating cause (example-URI)
What is the histo hallmark of Sweet’s Syndrome?
Nodular and diffuse dermal infiltrate of neutrophils with karyorrhexis and massive papillary dermal edema
If pathology reveals a histologic variant of Sweet syndrome what test can you order to confirm Sweets Syndrome as the diagnosis?
Myeloperoxidase stains–will be + if Sweets
True or False: Most cases of Sweets Syndrome are acute and self-limited, typially following a URI
True
What 3 inflammatory disorders are associated with Sweet’s Syndrome?
- IBD
- Behcet Syndrome
- Peripheral ulcerative keratitis
In neoplasia associated sweet’s syndrome, what common solid tumors do patients have?
GI (Men)
Breat (Women)
GU
What criteria must be met to diagnose patient with sweet’s syndrome?
2 Major + 2/4 of Minor criteria
What are the 2 major criteria that must be met to diagnose Sweets?
- Lesions presentation: Abrupt onset of erythematous plaques/nodules (occasionally with vesicles, pustules, bullae) presence of red edematous plaques
- Biopsy results: neutrophils, karyorrhexis, and marked papillary edema
Name the 4 minor criteria of Sweet’s Syndrome
- Associated Symptoms/Conditions: Rash preceded by respiratory infection, GI infection, or vaccination OR associated with–Inflammatory dx or infection, Myeloproliferative d/o or other malignancy, or pregnancy
- Malaise and Fever
- Abnormal Labs (_>_3): ESR>20, elevated CRP, Leukocytosis>8000, or left shift wiht >70% neutrophils
- Excellent response to treatment
What is the treatment for Sweets Syndrome
Systemic Steroids
oral prednisone 1mg/kg/day
-Continue for several weeks to prevent relapse
Name 3 of the 8 medications listed as possible txts for chronic or refractory sweets syndrome
- NSAIDS
- Dapsone
- Colchicine
- Doxy
- CLofazimine
- Cyclosporine
- Potassium Iodide
- Sulfapyridine
What is the treatment for this condition?
Prednisone and Dapsone
You are seeing a new patient for recurring rash on hands. On exam you note the palmar side of their hand is clear but the dorsal side appears edematous with numerous pustules and plaques. See attached pic. What is your diagnosis?
Neutrophilic dermatosis of the dorsal hands
Note: Best considered a localized variant of Sweet Syndrome
Diagnosis?
Neutrophilic Dermatosis of the Hands
diagnosis?
Histiocytoid Sweets Syndrome
Diagnosis?
Lymphocytic Sweets Syndrome
diagnosis?
Sweets in AML patient
Diagnosis?
Sweet’s in an SLE pt
Diagnosis?
Sweets in a Leukemia Patient
A 9 year old boy is seen in clinic for a worsening rash. Mom states the rash started off as small red bumps but has progressively gotten worse. On exam, pt has large urticarial, targetoid plaques with hypopigmented centers. This morphology is characteristic of what disease?
Marshall Syndrome
Note: Characterized by skin lesions resembling sweet’s syndrome which are followed by acquired cutis laxa
What 2 organs should you be considered about in a patient diagnosed with Marshall Syndrome and why?
heart and lungs
In this syndrome, elastic tissue is destroyed
What associated deficiency is seen in Marshall Syndrome?
alpha1 antitrypsin deficiency
A patient previously diagnosed with Marshall syndrome is seen in your office. What would you expect of the now healed lesions
As lesions resolve the elastic tissue is destroyed. This produces soft, wrinkle, skin-colored protuberant plaques that can be pushed into the dermis
Describe the histology of marshall syndrome
Neutrophilic dermatosis (identical to sweet’s)
Occasionally an eosiniphilic infiltrate is seen
Diagnosis?
angioedema
Note: eyelid, lips, earlobes, external genitalia, mucous membranes of mouth, tongue, larynx
Diagnosis?
exercise-induced urticaria
Note: appear 5-30 minutes after start of exercise (some pts only have this after eating celery before exercise)
suppressed by antihistamines + H2 antihistamine
Diagnosis?
Cholinergic Urticaria
Note: produced by acetylcholine on mast cell.
Spare palms/soles.
Diagnosis?
Dermatographism
(MC type of physical urticaria)
Diagnosis?
Urticaria secondary to Hep B
Diagnosis?
Acute urticaria
Diagnosis?
enlarging ulcer of PG
Diagnosis?
Cold urticaria
- after provacative test of ice cube x 3 minutes
Diagnosis?
annular and polycclic urticaria
Associated diseases?
dermatographism may occur in:
- hypo/hyperthyroid
- infectious dz
- diabetes mellitus
- onset of menopause
What induces this rash?
increasing body temp
- exercise
- warm bath
–> cholinergic urticaria
Diagnosis?
positive ice cube test =
- primary cold urticaria
- familial cold autoinflammatory synd.
Note: develops on rewarming
