10/2/2013 - emdsjhc.com€¦ · 10/2/2013 2 4 Severe nodular acne conglobata cystic Acne conglobata...
Transcript of 10/2/2013 - emdsjhc.com€¦ · 10/2/2013 2 4 Severe nodular acne conglobata cystic Acne conglobata...
10/2/2013
1
What’s up with Acne?
Jeanne Zeller MDDermatology
St Joseph ‘s Health centerPediatric Grand rounds June 28 2013
At what age should I do a screening?
At what age should I do a screening?
• 6‐7 or under for females
• 7‐8 or under for males
• Check for Tanner stages
• Send to endocrine if any doubt
How would you classify an acne?
• Comedonal
• Mild moderate papulopustular acne ( 0‐5)
• Severe papulopustular acne (6‐20), moderate nodular acne
• Severe nodular acne, Conglobata acne
How would you classify an acne?1‐Comedonal
Closed comedons White heads Open comedons “ black heads”
Papulo pustular
2‐Mild papulopustular 3‐Severe papulopustular
10/2/2013
2
4 Severe nodular acneconglobata cystic
Acne conglobata cystic Scars
Treatment depends ontype of acne
• Comedonal
• Mild moderate papulopustular acne ( 0‐5)
• Severe papulopustular acne (6‐20), moderate nodular acne
• Severe nodular acne, Conglobata acne
Treatment depending on prognosis
• Postinflammatory hyperpigmentation will disappear Good Prognosis long term
• Scars
Show increased collagen (hypertrophic and keloidscars) or be associated with collagen loss
Scarring Aggressive management
Therapy should be commenced early in the disease process.
Treatment depends on Psychological impact
• Not always proportional to the severity of the Acne
• Consider psychological impact in the therapeutic decision
Associated signs
• Virilism:– Hirsutism– Androgenetic alopecia
• Other signs of PolyCystic Ovarian Syndrome (PCOS)– Irregular cycles– Acanthosis nigricans– Obesity– Infertility
• Think of Hypercortisolism too: Cushing
1‐Comedonal AcneClosed comedons White heads Open comedons “ black heads”
10/2/2013
3
Treatment for Comedonal acne
• Retinoid topical– Stieva A: the cheapest
– Retin A micro :better tolerated
– Differin, Differin XP the best tolerance‐effect
• How supplied:– Stieva A, ( 0.01,, 0.05, 0.1 CREAM)(0.05 GEL) 0.025 Solution
– Retin A micro( 0.04,0.1)gel
– Differin0.1% gel or cream, Differin XP 0.3% gel
Treatment of comedonal acne
• Teen are lazy: Make it SIMPLE!
– Stievamycin ? PB with antibiotic resistance NO
– Prefer to alternate one day a Vit A ( stieva , Retin A Differine), one day a benzoyl peroxide ( Benzaclin, Clindoxyl)
– Or if good drug coverage Tactuo* : Differine plus benzoyl peroxide, once a day
– Or give a benzoyl peroxide wash10% ( Benzac*)
And Vit A topically.
Benzoyl peroxide and Clindamycin
• Clindoxyl :
– cheaper,
– ODB covered
• Benzaclin:
– more convenient : a pump
– More expensive
– Partly prepared by Pharmacist , some discrepancy with consistency.
Only indication for Benzamycin
• Benzoyl peroxyde and Erythromycin
• Pregnant woman
• Erythromycin advised instead of Clinda
Any Questions? Papulo‐pustular Acne
2‐Mild papulopustular 3‐Severe papulopustular
10/2/2013
4
Treatment of moderate papulopustular acne
• Retinoid:ALWAYS– Stieva A, ( 0.01, 0.05, 0.1 CREAM)(0.05 GEL) 0.025 Solution
– Retin A micro( 0.04,0.1)GEL– Differin, Differin XP,
• Benzoyl peroxide : Often,prevents antibiotic resistance, good anti inflammatory effect
• Dapsone? I have no experience yet• Topical Antibiotic if associated with peroxyde: Clindoxyl Benzaclin, not alone.
• Oral AB: if more severe
Antibiotics
• Anti inflammatory effect
• More than P acnes treatment effect
• Topical AB : Clindamycin preferred than Erythromycin , always with Benzoyl peroxide
Oral Antibiotics
• Stop Minocycline ( Minocin)!!!– too many auto immune side effects!
• Tetracyclin: – 500mg po TID far from food!!– Cheap , ODB covered
• Doxycycline: best dose <50– Non antibiotic dose is preferred to avoid the resistance: 20 mg BID
– Doxycyclin Hyclate 20mg tablets: Periostat * – Apprilon 40mg : Doxycyclin 40 OD– If Back order, Half a Doxy 100mg OD
• In Europe Lymecycline ..Hopefully one day in Canada..?
Explain!!!
• How they will hate their retinoid
• How long it takes to work
• How it can flare the first month
• How they will be red , itchy scaly
• How they will like their Benzaclin
• If no explanation , no compliance
• Reexplain when reassessed at 3 months
When to choose a contraceptive
• Female
• Acne variable with cycles
• Non smoker, no thromboembolic episodes in family
• Explain prevention of HIV and other sexually transmitted diseases
• Explain it may recur at the end of treatment
• Explain the delay to be efficient!!
What contraceptive to use?
• Alesse*, Tricyclen* efficient in studies
• We used to prescribe YAZ, but new side effect
– Higher risk of blood clot
• Diane 35 if hyperandogenism..
• Spironolactone : 200mg per day
– Safe and efficient in PCOS
10/2/2013
5
Any Questions? Treatment of severe acne
• Resist to the treatment ( at least 3 months)
• Severe papulopustular or scarring, conglobata
Papulo‐pustular Acne
2‐Mild papulopustular 3‐Severe papulopustular
4 Severe nodular acneconglobata cystic
Acne conglobata cystic Scars
First line therapy
• Three months of conventional therapy first
– Topical retinoid always, topical benzoyl peroxydeand antibiotic, oral antibiotic
– (Contraceptive pill)
– Can work !!
– Will prevent the flare with Accutane
– Will cover if it fails to justify the use of Accutane
Isotretinoin
• Yes if scarring
• Yes if other treatment failed
• Always precede with topical vit A for at least 3 months, and manual treatment of cysts.
• Easy for males
• If too early in puberty, need for a second course
• Treatment to reach 120 to 150 mg/kg total:
• about 6 months of 0.5 mg/kg
10/2/2013
6
Isotretinoin‐ female
• At least two visit to initiate
• First visit :
– Explain side effects
– Explain follow up monthly, BW
– Begin contraceptive
– Give hand out
– Give BW prescription Beta HCG blood, AST ALT, ALP Bili CBC Chol TG
Isotretinoin female
• Second visit
– After one full month of contraceptive
– Explain two methods contraceptive needed
– Review side effects
– Check BW
– Then initiate RX: 0.5 mg per kilo per day
Isotretinoin Follow up Females
• Every months females,
• Liver Chol Tg CBC at 1 month , then every three months
• beta HCG every month
• Side effects to reassess
Isotretinoin follow up Males
• Liver Chol Tg CBC at 1 month , then every three months
• Side effects to reasess
Isotretinoin Side Effects
• Dryness
– Skin, even patches of eczema , hands mostly
– Eyes ( caution with contact lens, risk keratitis)
– Lips
• Headaches: risk Pseudotumor cerebri: this is why CI with Cyclines
• Muscle pain, very frequent , can limit competitive sport
Isotretinoin Side Effects
• Trouble with night vision: Caution with drivers
• Flare of acne at beginning: very frequent
– Risk Acne Fulminans : very rare
• Depression: Yes , idiosyncratic. Mood changes Suicidal risk.
– To assess at each visit!
• Sleep disorder
• Relation with Inflammatory Bowel disease?
10/2/2013
7
Isotretinoin and pregnancy
• Teratogenicity +++
– 1 month of contraceptive pill before treatment begins
– 2 contraceptive ways, add spermicide or preservatives.
– Check Beta HCG every 4 weeks.
– Have the hand out read twice , and signed before treatment
Side effectsWhen to wean , when to stop?
Side effectsWhen to wean , when to stop?
• High Cholesterol?
• High triglycerides?
• Depression?
• Back pain?
• Eczema?
• Keratitis?
When to wean, When to stop
• High Cholesterol? NO
• High triglycerides? YES IF RISK PANCREATITIS
• Depression? YES
• Back pain? NO ,
• Eczema? NO
• Keratitis? YES UNTIL HEALED, STOP CONTACTS
Acne fulminans Acne fulminans
• Can appear spontaneously
• Or after initiating Accutane
– Fever
– terrible inflammation/pain
– erythema nodosum
• STOP ACCUTANE
• ORAL PREDNISONE ,topical as well
• Accutane can be given at very low dose
10/2/2013
8
scars Cheloids
• Family History to look for
• Complication of acne, NOT a complication of Accutane
• Use silicone dressings or creams for about a year
• Then inject the residual scars with Triamcinolone 5 to 10 mg/ml
• Can be devastating.
Hyperpigmentation Reassure
• Responds well to treatment and time
• Sun protection++
• Vitamine A 0.1%
Conclusion
• Retinoids ALWAYS :The only way to change the skin!
• Benzoyle Peroxyde avoids AB resistance
• Low dose oral AB avoids antibiotic resistance
• No mynocyclin anymore !!
• Scars indicate stronger RX
02/10/2013
1
Pediatric Derm. Cases6 months review
Jeanne Zeller, MD
St Joseph’s Health Center
Pediatric Dermatology
Objectives
• Review the main referred diagnosis
• Update on these in recent meetings
• Share a few interesting cases
• And leave with learning points
No conflict of interest
Pictures taken from Dermatlas.
+ Some patient pictures
Atopic dermatitis
• High proportion of the cases in Pediatric derm.
Learning points
• Current management to review :– Water is good not bad
– Baths 5‐10 min lukewarm water +/‐ oil– Room humidifier
– Topical steroids to apply first,– on wet skin, easier, keeps the water in– Ointments better than creams– Not sparingly, 2 to 3 times a day
– Treat aggressively to win quickly– Fight against fear of steroids , parents and Pharmacists
– Teens after 10 , risk increased for striae– Think of alternative Rx: Protopic, oral Rx for severe cases.
– Asthma associated, long term and severe eczema
We just explain
• Take time to explain– “We have NO CURE
– We can only treat the flares
– It will come and go
– You are not responsible for the flares”
• Reassure:– “Kids usually outgrow it “
• Discuss side effects of steroids , and black box for Tacrolimus– Pharmacist will warn, and parents will not use it!
02/10/2013
2
Unwell Atopic Kids
Red excoriated Honeycomb crusts
Supra‐infection of Atopic Dermatitis
• IMPETIGO
– More crusts
– More redness
– Oozing
– Yellowcomb crusts : Staphylococcus
– Bright red and pain : Streptococcus
Learning points
• Treatment:– Keep topical steroids
– Associated with AD, use Oral Antibiotics
• Keflex (because Oxacillin very bad taste)• If allergy , Erythromycin ( bad GI tolerance)
– Baby, use oral AB
– Not AD, Not baby
• Topical AB often enough• Or Medi‐honey
Other infection?
Eczema herpeticum
• 1 patient seen in ward
• Worse despite
– good use of topical steroids
– IV antibiotics
• Clue: Parents thought he had “ chicken pox”
• Dramatic improvement with IV Acyclovir 20mg/kg/q8h (60mg/kg/day) (+1.5l drinks)
• Think about it when does not respond
Eczema Herpeticum
• Small vesicles grouped or isolated , small pinpoint erosions
• Eczema ”spreads”, generalized, unresponsive
• patient uncomfortable, ,appearance of bacterial infection
• systemic symptoms.
02/10/2013
3
Treatment• Hospitalization advised for:
– young children<1 year of age
– Male sex
– Fever
– Systemic symptoms at presentation
– IV Acyclovir ( 4‐6 days),60mg/kg/day
– Then oral(30 mg/kg/day) ( lower bioavailability‐15‐30%) total duration 12‐15 days
(SPD I Lara‐Corrales , resident fellow research award 2010)( printed algorythm)
Learning point
• Stop topical steroids for 48 hours ,
– until Acyclovir has shown some effect.
– Not too long or eczema will flare
• Eczema herpeticum is a contraindication for further use of Tacrolimus and Pimecrolimus (Protopic and Elidel)
Kids return from camp or cottage in the summer
Phyto‐Contact Dermatitis
• Many cases in the ward:• Some look like cellulitis, but itchy, will not respond well to antibiotics
• Summer bilateral legs or feet blisters or ?“cellulitis”
• Ask where they are coming from, what they did during the week end
• Burnt leaves in neighborhood: face
Learning points:• The clue of treatment : strong topical steroids, dramatic improvement in most of the cases.
• Oral steroids only if very severe…always think of risk and benefit of the oral steroids (hip necrosis).
• AB rarely necessary
• Sunprotection of healed lesions: Risk of hyperpigmentation++
Culprits
Poison Ivy Poison Sumac
02/10/2013
4
Other culprits
Poison OakHog weed
“Useful experience”
• Dr Lefevre Psychiatrist in Sick Kids advises the kids with a chronic visible condition:
• “Do a science project about it !!
• Become the School expert about the condition! “
• Hanging a poster at school may help peers!
13 years old male, 10 years of topical steroids for “eczema”. No effect.
Ichthyosis“ichthus”: fish in greek
Ichthyosis vulgaris
• Often associated with AD
–hyperlinearity of palms,
– fine scales on extensor surfaces.
X linked ichthyosis
• Recessive X linked:
– mother carrier
–maternal grand father affected
• Thick brownish scales,
– spare the folds and the face,
–no hyperlinearity of palms.
Learning points X linked
• Risk of testicle cancer: cryptorchidism
– Look for it
– ++ Send to surgeon
• Eye exam R/U corneal opacities, retinal AN
• Treatment :– Emollients, keratolytics, humidifier.
– No use for Steroids
02/10/2013
5
Nevi check‐Melanoma
• Increase incidence of Pediatric Melanoma
• Update on ABCDE rules in melanoma pediatrics (<10yo)
• A Amelanotic 76%
• B Bleeding 54%
• C Color Single 87%
• D Diameter<6mm 72%
• E Elevation 91%
Research report Dept DermSan Francisco
• Evidence for Modification of the ABCDE Criteria for Pediatric MelanomaDeepti Gupta, MD1, Ilona J. Frieden, MD1, Mohammed Kashani‐Sabet, MD 2, Timothy H. McCalmont, MD1, Kelly Cordoro, MD11University of California ‐ San Francisco, 2California Pacific Medical Center
• Study 70 patients,death:
– 6% prepub
– 12% pub
Learning point
• “Pseudo pyogenic granuloma”
– Send all pyogenic granuloma to pathology,
– React in front of new red tumor.
– Know the new ABCDE for kids
• Spitz nevus• controversial risk of Melanoma
• but usually keeps growing ,
• so excision is preferred to not RX
• In SPD study 26% prepubertal melanoma were “spitzoid”
Acne
• Reassure:– 95% teenagers have acne
• Explain :– Role of vit A
• Irritant• Long time to be efficient• Keep using it even every other day • Stieva A, Stievamycin, Retin A micro , Differin, Differin XP.
– Anti‐inflammatory• Peroxyde ( proactive very weak peroxyde %)• Topical antibiotics• Oral antibiotics• Will not change the skin, only a “ concealer”.
Learning points• Don’t use Mynocin.
– There are alternative AB (TTC, Doxycyclin, Erythromycin)
• Risk autoimmune disease (Ochsendorf F.. Am J Clin Dermatol. 2010;11(5):327‐41.
• Minocycline in acne vulgaris: benefits and risks.)
• Isotretinoin– When resists to other therapy or risk of scarring.– Discuss side effects
• Teratogenicity +++• Dryness• Muscle pain• Liver , lipids• Depression: Idiosyncratic , reversible.• Controversial association with IBD ( recent litterature)
– Booklet to read before the treatment , – Contraception.– Bloodwork, monthly F/U
References
• Ochsendorf F.. Am J Clin Dermatol. 2010;11(5):327‐41.
• Minocycline in acne vulgaris: benefits and risks.
• Source
• Department of Dermatology and Venereology, University of Frankfurt, Germany. [email protected]‐frankfurt.de
• Abstract
• Minocycline is a semi‐synthetic, second‐generation tetracycline. It was introduced in 1972 and has both antibacterial and anti‐inflammatory properties. Minocycline is used for a variety of infectious diseases and in acne. Even today, new indications beyond the antibacterial indications are being investigated such as its use in neurologic diseases. Formerly, minocycline was thought to have a superior efficacy in the treatment of inflammatory acne, especially with respect to antibacterial‐resistant Propionibacterium acnes. A thorough review of the literature, however, shows that minocycline is not more effective in acne than other tetracyclines. Compared with first‐generation tetracyclines, minocycline has a better pharmacokinetic profile, and compared with doxycycline it is not phototoxic. However, minocycline has an increased risk of severe adverse effects compared with other tetracyclines. It may induce hypersensitivity reactions affecting the liver, lung,kidneys, or multiple organs (Drug Reaction with Eosinophilia and Systemic Symptoms [DRESS] syndrome) in the first weeks of treatment and, with long‐term treatment, may cause autoimmune reactions (systemic lupus erythematosus, autoimmune hepatitis). In addition, CNS symptoms, such as dizziness, are more frequent compared with other tetracyclines. Long‐term treatment may induce hyperpigmentation of the skin or other organs. Resistance of P. acnes to minocycline also occurs, dependent on the prescribing behavior. Considering the aspects of efficacy, its adverse effect profile, resistance, price, and alternatives, minocycline is no longer considered the first‐line antibacterial in the treatment of acne.
02/10/2013
6
Red tumor in a newborn Hemangioma
• Natural history: will regress
• Perineal lesion: look for association
– Imperforated anus, genitourinary anomalies
• When to treat?
– Impair a function: eye , lips, throat,
– Ulceration ( folds, periorificial)
– Liver : risk compartment syndrome, hypothyroidism
– Hemorrhage : GI
– Cosmetical risk
How to treat?• Steroids were first intention
• Propranolol (July 2008)2mg/kg
• Protocol of use
– Weight, Blood pressure, Heart rate,
– F/U 1 week, 3 weeks then monthly
– Picture each time
• Dose
– 0.1mg/kg TID 3 days
– 0.3mg/kg TID 3 days
– 0.7mg/kg TID ( = 2 mg/kg)
Propranolol Side effects
• Low BP, cold or blue extremities
• Wheezing (CI if asthma)
• Low sugar: give before the bottle
• potassium? Recently reported
• Sleep trouble
• Globally very well tolerated, compared with Steroids+++
Other treatments
• Nadolol ,
– probably the best
– but not enough published studies yet,
– less side effects,
– does not pass the brain barrier.
• Timolol topically (0.5% like the eyedrops for Glaucoma)
– for flat small on face
Experience in Day care for new born
• 29 weeks premature twin male.
• Painful ulcerated hemangioma
• Topical care:
– Ihle’s paste mixed with Stoma powder
– Non adherent dressing when possible ( mepilex border‐opsite postop)
• Organized day‐admission for each new dose
• Until reached 2mg per kilo
02/10/2013
7
1 month treatment1 week at 2mg/kg
Sept 14 open wound Oct 12, healed , no pain
Risk of special locations
• PHACES ?(No Propranolol!)• P for Posterior malformation
• H for Hemangioma
• A for Arterial anomalies
• C for Cardiovascular anomalies
• E for Eye and Endocrin anomalies
• S for Sternal defect
• Pediatr Dermatol. 2011 Aug 19.
• Propranolol for Recalcitrant Ulcerated Hemangioma of Infancy.
• Hong E, Fischer G.
• Source
• Department of Dermatology, Royal North Shore Hospital, Sydney, NSW Discipline of Dermatology, University of Sydney, NSW, Australia.
• Abstract
• Hemangioma of infancy (HOI) is a common, self‐resolving, benign tumor that frequently requires no intervention. Ulceration is one of the main complications of HOI, and active treatment is usually required to manage pain, potential scarring, and occasionally bleeding and infection. Since the discovery that oral propranolol is an effective treatment for complicated HOI, it has been replacing systemic corticosteroids as first‐line therapy, and early recommendations for treatment protocols have been devised. We report the successful treatment of six children with recalcitrant ulcerated HOI using propranolol. In all cases, complete, rapid healing occurred. Two children experienced recurrence of ulceration after ceasing propranolol, but this was rapidly controlled by restarting treatment. No adverse effects were encountered. We propose that ulceration in HOI is an indication for propranolol treatment if more conservative therapies have failed.
• Br J Dermatol. 2010 Aug;163(2):269‐74. Epub 2010 May 8.
• Propranolol for infantile haemangiomas: insights into the molecular mechanisms of action.
• Storch CH, Hoeger PH.• Source
• Department of Clinical Pharmacology and Pharmacoepidemiology, University Hospital of Heidelberg, Im Neuenheimer Feld 410, D‐69120 Heidelberg, Germany.
• Abstract
• Infantile haemangiomas (IH) are the most common benign tumours of infancy. Although most IH are innocuous and 85‐90% regress spontaneously, some may become life‐ or function‐threatening and require immediate treatment. Previous standard therapeutic options include physical measures (laser surgery, cryosurgery) and systemic corticosteroids, in severe cases also vincristine, alpha‐interferon or cyclophosphamide, all bearing the risk of serious side‐effects. Oral propranolol is a very recent therapeutic option for complicated IH with impressive efficacy and generally good tolerance. The effects of propranolol on IH were discovered by chance, and very little is known about its mechanisms of action in IH. Here we present a summary of current knowledge of how propranolol interferes with endothelial cells, vascular tone, angiogenesis and apoptosis. Early, intermediate and long‐term effects of propranolol on IH can be attributed to three different pharmacological targets. Early effects (brightening of the haemangioma surface within 1‐3 days after start of therapy) are attributable to vasoconstriction due to decreased release of nitric oxide. Intermediate effects are due to the blocking of proangiogenic signals (vascular endothelial growth factor, basic fibroblast growth factor, matrix metalloproteinase 2/9) and result in growth arrest. Long‐term effects of propranolol are characterized by induction of apoptosis in proliferating endothelial cells, and result in tumour regression
• Arch Dis Child. 2011 Sep;96(9):890‐3. Epub 2011 May 28.
• Propranolol for infantile haemangiomas: a review.
• Starkey E, Shahidullah H.
• Source
• Derbyshire Children's Hospital, Uttoxeter Road, Derby DE22 3NE, UK. [email protected]
• Abstract
• Infantile haemangiomas are the most common benign tumour of infancy. However the majority are self‐resolving and only a small minority of cases require treatment, with various different medications being used in the past. Over the last few years, propranolol, a non‐selective β‐blocker, has become a popular and successful treatment for infantile haemangiomas. However, further research on its safety is needed if it is going to be used more frequently. This article summarises the current literature on propranolol for haemangioma treatment with emphasis on its toxicity and adverse event profile.
New born with a rash
02/10/2013
8
1 day Female on term
• Normal delivery
• No fever, no sign of distress
• Drinking well
• Vesicules, pustules papules:polymorphic
• No pattern
• Eosinophils Tzank smear ( F Vincent)
• Eosinophils in blood
Differential
• Vesicules R/U Herpes, treated
• Pustules R/U Bacteria, treated ( Candida )
• Eosinophils Differential:
– Scabies
– Erythema toxicum neonatorum
– Incontinentia pigmenti
Was too well to stay in NICU
1 week
Linear pattern
Firm pustules, crusts
Incontinentia pigmenti ( IP)
• Blashkoid rash
– Vesicules ( first weeks)
– Crusts ( first months)
– Hyperpigmentation (1st years)
– Hypopigmentation (teen to adult)
• Teeth,
– conic, missing
• Sparse Hair,
– eybrows,eyelashes
– nails
• Risk to the eye and brain
– Send to Eye exam
• Can lead to blindness
• retina
– Risk 10‐20% CNS • Seizures
• Developmental delays
02/10/2013
9
SPD July 2011 BaltimoreJohn Hopkins University: Buddy Cohen, David Wolf
– Patient with brain involvement :
• Early <6 mo
• Vascular anomalies, “ vasculitis”?
• ? Sytemic steroids when neurological signs
– Possible correlation between eye involvement and CNS
• Early eye exam
2 weeks
Atypically pustular ( sterile) Blashkoid
Genetics of IP
• X linked Dominant, NEMO Gene
• Transmitted by mother
• Male usually lethal
( some male cases described , maybe mosaics)
In this case
• 5monthsF/U
• Crust stage
• Frontal Bossing , microstomia
• Seems to have normal development
• Eye exam so far normal
• Genetic testing positive
• Mother test pending
• Should we test Dad?
• Cutis. 2007 May;79(5):355‐62.
• Incontinentia pigmenti (Bloch‐Sulzberger syndrome): a systemic disorder.
• Ehrenreich M, Tarlow MM, Godlewska‐Janusz E, Schwartz RA.
• Source
• Dermatology and Pediatrics, UMDNJ‐New Jersey Medical School, Newark 07103‐2714, USA.
• Abstract
• Incontinentia pigmenti (IP) is a rare inherited multisystem disorder characterized by a distinctive swirling pattern of the skin; defects of teeth, hair, and nails; and ophthalmic, central nervous system, and musculoskeletal abnormalities. It progresses through several well‐defined stages. IP is transmitted as a dominant X‐linked trait with variable expressivity, but many‐‐if not most‐‐cases are sporadic. IP has been shown to result from mutations in the NEMO gene that completely abolish expression of NF‐kappaB essential modulator. The diagnosis of IP typically is made based on characteristic clinical findings. Molecular analysis of the NEMO gene is now possible, as is analysis of skewed X‐chromosome inactivation, which can further reduce diagnostic confusion. A number of disorders, including hypomelanosis of Ito, should be considered in the differential diagnosis. The considerations vary according to the stage of IP. Careful head‐to‐toe clinical evaluation is critical in the evaluation of a child with suspected IP given the frequent multisystem involvement. A multidisciplinary approach including dermatology, ophthalmology, neurology, and dental consults is typically warranted. The skin manifestations of IP do not require specific treatment other than reassurance; spontaneous resolution of the lesions usually occurs.
02/10/2013
10
9 mo female with abnormal nailsBrittle conic nails,
why look at the knees?
Mother and grand father nail anomaly
• Mother had hip, knees and feet pain
Nail Patella syndrome
• Baby no patella palpated
• Mother obvious absence of patella!!
• Classically triangular lunula
• RISK FOR KIDNEY AND EYE ( lifelong risk)
– Send to Kidney US, bloodwork, Urinanalysis
– Eye exam , risk glaucoma
• 1 case in Sickkids, same age same nails,
• Natalie had one case too and just wrote it down
Nail patella syndrome
• Learning point
• Look for renal involvement
• Nail‐Patella Syndrome.
• Authors
• Sweeney E, Hoover‐Fong JE, McIntosh I.
• Editors
• In: Pagon RA, Bird TD, Dolan CR, Stephens K, editors.
• Source
• GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993‐.2003 May 31 [updated 2009 Jul 28].
• Excerpt
• DISEASE CHARACTERISTICS:
• Nail‐patella syndrome (NPS) involves a classic clinical tetrad of changes in the nails, knees, and elbows, and the presence of iliac horns. Nail changes are the most constant feature of NPS. Nails may be absent, hypoplastic, or dystrophic; ridged longitudinally or horizontally; pitted; discolored; separated into two halves by a longitudinal cleft or ridge of skin; and thin or (less often) thickened. The patellae may be small, irregularly shaped, or absent. Elbow abnormalities may include limitation of extension, pronation, and supination; cubitus valgus; and antecubital pterygia. Iliac horns are bilateral, conical, bony processes that project posteriorly and laterally from the central part of the iliac bones of the pelvis. Renal involvement, first manifest as proteinuria with or without hematuria, occurs in 30%‐50% of affected individuals; end‐stage renal disease (ESRD) occurs in about 5% of affected individuals. Primary open‐angle glaucoma and ocular hypertension occur at increased frequency and at a younger age than in the general population.
02/10/2013
11
Nail patella syndrome Reference
• DIAGNOSIS/TESTING:
• The diagnosis of nail‐patella syndrome is based on clinical findings. Molecular genetic testing of LMX1B, the only gene known to be associated with NPS, is available on a clinical basis.
• MANAGEMENT:
• Treatment of manifestations: Orthopedic problems may be helped by analgesics, physiotherapy, splinting, bracing, or surgery; ACE inhibitors to control blood pressure and possibly to slow progression of proteinuria; renal transplantation as needed. Surveillance: Annual monitoring for hypertension and renal disease; screening for glaucoma as soon as a child is compliant. Agents/circumstances to avoid: Chronic use of NSAIDs because of the detrimental effect on kidney function.
• GENETIC COUNSELING:
• Nail‐patella syndrome is inherited in an autosomal dominant manner. Eighty‐eight percent of individuals with NPS have an affected parent. Twelve percent of affected individuals have a de novomutation. The offspring of an affected individual are at a 50% risk of inheriting NPS. Prenatal diagnosis for pregnancies at increased risk is possible if the disease‐causing mutation in the family has been identified.
14 years old female • Shin hyperpigmented plaques
• PMHX: 2 years history of diabetes I
Necrobiosis lipoidica
• My first pediatric case?
• Do you see any?
• Bad prognosis for the kidney, eye, arteries..
10/2/2013
1
Are There New Therapies for Skin Cancer?
A New Chance for Stevens Johnson Syndrome (SJS)?
Jeanne Zeller MDDermatology
Assistant professor University of TorontoSt Joseph’s Health Center
Conflict of interest?
• None
Objectives
1) To recognize the Non Melanoma Superficial Skin Cancers, and have a therapeutic approach.2) To know the use and effect of new topical treatment for Non Melanoma Superficial Skin Cancers.
3) To consider the use of cyclosporin in SJS and TEN ( Toxic Epidermal Necrosis)
Actinic keratosis
Actinic Keratosis Differential
Seb keratosis stucco keratosis
10/2/2013
2
Differential
Psoriasis ( guttate) Discoid lupus
Actinic Keratosis (AK)
• UV light induced epidermal lesion
• May progress to invasive Squamous Cell Carcinoma ( SCC)
• Histology and molecular features , same as Squamous cell carcinoma
Prevalence of Actinic keratosis
• Fair sun exposed skin
• Northern Hemisphere 11 to 25%
• Australia 40 to 60%
• Organ transplant 40% after 5 years of transplant
Risk to transform in SCC
• 0.18 to 0.24 % per AK per year
• If you have 7 AK, you have 10 to 18% of risk of developing a SCC in 10 years.
What changes are suspiscious
High risk of SCC:
• I—Induration/Inflammation
• D—Diameter >1 cm
• B—Bleeding
• R—Rapid Enlargement
• E—Erythema
• U—Ulceration
Squamous Cell Carcinoma( SCC )
10/2/2013
3
Other non melanoma skin cancers
• Bowen disease
– Considered a SCC in situ
– Well demarcated homogenous red plaque shins, calf, genital or anal mucosa
Bowen disease
Bowen diseaseBasal Cell Carcinoma ( BCC)
NodularFirm, shiny pearl, vessels.
Superficial BCC Treatment of Actinic Keratosis ( AK)
• The European Dermatology Forum.
– Guidelines for the management of actinic keratoses.
20 October 2010.
• Classification
– A= good evidence to support use;
– B= fair evidence to support use;
– C= poor evidence to support use;
– D= fair evidence to support rejection;
– E= good evidence to support rejection
10/2/2013
4
Treatment of actinic keratosis (AK)
• Cryotherapy,
– Category A
– Liquid Nitrogen minus 195 C , 5 to 40 seconds
– Quick easy , office based
– for a few lesions
– works better associated with another topical treatment,
Photodynamic therapy
• Photodynamic therapy– Category B
– Methyl 5 amino‐levulinate cream for 3 hours , then red light 75 j/cm2
– a light activates a photosensitizer localized in disease tissues, spurring the formation of cytotoxic reactive oxygen species
– For many lesions
– Good results but needs time and complicate “machinery”, expensive.
Treatment of AK
• Curettage and electrodessication,
– Category C
– one to three procedures, local anesthesia.
– Higher risk of scarring
• Dermabrasion, Chemical peels:
– Category D
– Long time to heal,
– Risk of scarring and dys‐pigmentation
Immunomodulators
• Imiquimod:
– Category A, clearance 70%.
– Aldara 5% 3 times a week 16 weeks
– Zyclara 2.5% OD 2 weeks on, 2 weeks off, 2weeks on
– Side effect: red blister, crust burn, oozing.
– Expensive
• Ingenol mebutol
– Picato gel ( LEO) 2 or 3 days!
– Approved in US, not yet in Canada
Topical Chemotherapy
• Efudex: 5 Fluorouracil (5 FU):
– Approved category B, clearance 52%
– Blocks the DNA synthesis
– Severe inflammation
• Pruritus
• Ulceration
• scarring
Diclofenac
• How does it work?
• The sun provokes – an increase of COX2
• increase PG2proliferation, growth, angiogenesis
• increase Bcl2antiapoptosis
• Anti‐inflammatory – blocks COX 2 and blocks Prostaglandin2:
• Antiproliferative , antiangiogenesis
– blocks Bcl2• Apoptosis
10/2/2013
5
Diclofenac
• Category A
• Complete clearance rates vary from 50% to 79%
• Well tolerated
– Scales,itch
• Used BID 3 months
• Check for allergy
• No renal impairment described
My experience with Diclofenac
• “ The miracle gel “
• Cheap !
• Try 4 days on a small area of hand(R/U allergies)
• Erases most of the AK
• Selects the most suspicious ones
• Combined therapy is more efficient
• Cryotherapy or imiquimod on the resistant lesions
AK resist to diclofenac?
• Cryotherapy or Imiquimod
• If High risk of SCC: Biopsy and Refer for surgery– I—Induration/Inflammation
– D—Diameter >1 cm
– B—Bleeding
– R—Rapid Enlargement
– E—Erythema
– U—Ulceration
Conclusion
• First line therapy– One or two AK , Cryotherapy
– Many AK , Diclofenac if no allergy
• Second line therapy– Cryotherapy
– Imiquimod
– 5FU
• High Risk– BIOPSY
Are you sleeping?Here is the “ foot breaker” !
A new therapy for Stevens Johnson?
• Until now, several curative treatments have been proposed without evidence of effectiveness.
• Several case and case series reports have shown with Cyclosporin :– The arrest of disease progression
– A shorter time to re‐epithelialisation
– No death.
Cyclosporine for SJS/TEN: a case series and review of the literature. Cutis. 2011 Jan;87(1):24‐9Reese.D ,Gilson R.
10/2/2013
6
Stevens Johnston(SJS) Toxic Epidermal Necrolysis (TEN)
• SJS –TEN:
– Severe cutaneous reactions that are medication‐induced in most instances.
– Epidermal detachment
• <10% = SJS
• >30% = TEN
• 10%< =Overlap SJS‐TEN <30%
Purple macules, coalescent, dusky blisters , sloughing ( Nikolsky sign)
Affects the mucosae and the skin
• SCORTEN is a used as a prognosis factor.It will calculate the % risk of death
• Severe complications: Acute skin failure– pulmonary embolism ,– sepsis,– gastric hemorrhage ,– renal failure, – vision impairment
SCORTEN = 0 to 7• Risk Factor 0 1
• Age <40years >40years
• Malignancy Yes No
• Hear rate <120 >120
• Epidermal <30% >30%
detachment
• Blood Urea level <10mmol/l >10 mmol/l
• Serum bicarbonate <20 mmol/l >20 mmol/l
• Serum Glucose <14mmol/l >14mmol/l
Treatment
• 1‐ Remove the causal medication+++
– Antiepileptics,
– Sulphonamide antibiotics,
– Penicillins,
– Allopurinol
– Oxicam NSAIDs
Treatment
• 2 ‐Send to a burns unit ( if not ICU) for acute skin failure:
– Monitoring of the fluid – electrolyte balance,
– Provision of enteral or parenteral nutrition,
ASAP nasogastric tube.
– Wound care
– Treatment of sepsis.
10/2/2013
7
Treatment
• In addition, supportive care of affected mucosal surface
– Aggressive ocular lubrication
topical corticosteroids and topical antibiotic
– Amniotic membrane graft has improved the ocular prognosis
– Hygienic mouthwashes and topical oral anesthetics
– Monitoring for urinary retention
Treatment specific so far
• IVIg :3g/Kg total dose in 2 to 3 IV– Retrospective studies: controversial results
– Risk of liquid overload
– Very expensive
• Steroids, Bolus IV less side effects than Prednisone oral– Again controversial studies
• Plasmapheresis
• Cyclophosphamide ( side effects+++)
Cyclosporin’s mechanism of Action
• Antiapoptotic activity
• Inhibits CD8 activation
• inhibition of cytotoxic activity.
Cyclosporin for Stevens Johnson –TEN syndrome
One prospective study
• Open trial of ciclosporin treatment for Stevens‐Johnson syndrome and toxic epidermal necrolysis.
Br J Dermatol 2010 Oct;163(4):847‐53.Valeyrie‐Allanore l,
Roujeau J
29 patients included
• Cyclosporin 3mg/kg OD 10 days , then tapered for 1 month
• Side effects :
– 3 severe , stopped at day 10
• leucoencephalopathy (n = 1),
• neutropenia (n = 1)
• nosocomial pneumopathy(n = 1).
– 2 alteration renal function(n=2) tapered earlier
SCORTEN
• Was predicting 2.8 deaths (10%)
• No deaths occurred.
10/2/2013
8
Sunnybrook experience
• Cyclosporin will be first line therapy.
• Patient discharged after a few days
• Stops the progression rapidly
• Dr Shear , at each conference, will tell his experience and ask the audience to try.
• But no real study.
Two patients in St Joseph with Cyclosporin
• Favorable evolution
• One stopped evolution rapidly
• The second stayed longer but favorable evolution
• Both had steroids as well.
• Maybe should we try Cyclosporin alone to avoid the immunosuppressive effect of the steroids?
Retrospective study
• Will be possible only with other centers
• Prospective double blinded study, technically and ethically difficult
– When you think a medication will save the patient’s life,
– You don’t want to keep trying another one.
Conclusion
• The recent literature puts Cyclosporin on the same level as IVIG and Steroids.
• IVIg and Steroid use is becoming controversial
• Should we keep trying?
• Australasian Journal of Dermatology (2012) 53,165‐171.The management of toxic epidermal necrolysis. Fernando SL
10/2/2013
1
Dermatological Emergencies
Jeanne Zeller MDDermatology
Assistant Professor University of TorontoSt Joseph’s Health Center
Why calling a dermatologist ?
• Wound care
• Cellulitis
• Rash
• Tumor
Why calling a dermatologist ?
• Wound care:– Until Leslie Heath, the wound care nurse arrives!
– Now we see some wounds together but…
– She takes all the decisions!
• Cellulitis:– Identify and treat the cause
– Differential diagnosis, risk of necrotizing fasciitis.
• Rash: Cause and management
• Tumor: Type and management
Wound care
• St Joseph HC has two specialists of Wound care– Leslie Heath
• First approach as a nurse, diagnostic, causes,
• First line treatment, dressings, VAC
• Referral if needed to Foot clinic, Plastic surgery
– Franklynne Vincent• Follow up in Wound Care Clinic ( 1/week high load…)
– My role in Wounds• Biopsy if suspicious ( SCC, Pyoderma gangrenosum, vasculitis)
• Replace Leslie if away.
Cellulitis #1 referral
• Identify the bacteria
– Swab and blood culture• Streptococcus #1 (57% ref 1)
• Staphylococcus #2 ( 14%)
• BG negative#3 (28%)
• Choice of antibiotic
– Cephalexin #1 po
– Cefazolin IV
When to worry for a cellulitis?Necrotizing fasciitis
Plastic surgery emergency
• Pain>>>>local signs
• Rapid extension
• Sensitivity loss
• Turns dark,Necrosis
10/2/2013
2
Cause of Cellulitis
• Venous stasis dermatitis Easy to recognize
• Edema
• Hemosiderin deposits
• Varicosities
• Inverse Champagne bottle: lipodermatosclerosis
• Ulcers
• Rarely isolated!
Cause of Cellulitis
• Associated Peripheral Arterial Disease
• To look for ++
• ARTERIAL DOPPLER, specially in diabetes patient
• Absent pulses
• Claudication
• Distal necrosis
• Small suspended ulcers
• Neuropathic ulcer• Diabetes, paraplegia
Cause of cellulitis
• Lymphoedema
• Either primary ( lack of development of lymphatic vessels)
• Or secondary to – Venous stasis
– Surgery
– Radiotherapy
– Trauma
– Reduced mobility
Cause of Cellulitis
• Toes intertrigo: – Dermatophyte– Bacterial suprainfection
• Nail fungus :– A white or yellow opaque
streak on side of the nail– Subungual hyperkeratosis.
Scaling under the nail– Distal onycholysis. The end
of the nail lifts up. The free edge crumbles.
Cause of Cellulitis• Contact dermatitis:
– Topical antibiotic• Bacitracin, polysporin
– Topical anti‐ inflammatory
• Diclofenac
Case #1
• Female in her sixties
• Sent from emergencies to clinic for
– Leg rash
– Blisters
– Cellulitis?
– Bullous pemphigoid ?
10/2/2013
3
Case #1
• Female in her sixties
• Sent from emergencies to clinic for– Leg rash
– Blisters
– Cellulitis?
– Bullous pemphigoid ?
• Key question:
ITCHY OR PAINFUL?
• Walked in a field:
POISON IVY
Case #2
• Female age 12
• Diagnosis of admission:
Bilateral feet cellulitis
• Admitted , on IV antibiotics
• Question: ITCHY OR PAINFUL?
• Was coming back from Camp
• Poison Ivy
Case#3
• Male 92 yold
• Admitted for
• Bilateral arm “cellulitis”
• On IV antibiotics
Case#3
• Male 92 y old
• Admitted for
• Bilateral arm “cellulitis”
• On IV antibiotics
• Question: ITCHY?
• Look for blister
• Biopsy :
Bullous pemphigoid
Bullous Pemphigoid
• The most frequent auto immune bullous disease
• Incidence 4/100,000
• 10 cases seen in St Joseph in 2 years
Bullous Pemphigoid
• All elderly patients
• Very itchy rash, edematous, looks like fix urticaria or cellulitis
• Until strong blisters appear
10/2/2013
4
Bullous pemphigoid
Management:
• Feed, proteins
• Wound care :Empty the blisters keep the roof
• Steroids:– Topical 20‐40g clobetasol per
day
– If does not respondOral steroid ½ to 1mg/kg
• Sparing agent– Azathioprine,
– Methotrexate
Patient with non healing woundsHas not been eating for long
Questions?
Rash
• When to worry for a rash?– Sloughing more than blistering on erythematousor purple baseRisk:
• Stevens Johnsons Syndrome ( SJS) /
Toxic Epidermal Necrolysis (TEN)
• Staphylococcal Scaled Skin Syndrome (SSSS)
– Fever, chills, generalized pruritic rash, lymph nodes, scaling.
• Erythroderma
Case #4
• Female 45, Afro –American skin‐type
• Bad burn on forearm
supra‐infected, given Septra
• 8th day of Septra,
• Purple flat small coalescing papules with sloughing
Case #4
• Diagnosis: SJS
• Face lips sloughing with crusts
• Muccal buccosa spared
• Eyelids affected but conjonctiva and cornea spared
• Feet , blisters of both soles, longer time to heal.
• Evolution favorable with: Stop Septra,shortcourse Prednisone and Cyclosporin: Stopped progression of the rash.
Case #5
• Female late 70 , treated with many antibiotics for Infected Knee Prosthesis
• Most recent antibiotic
Vancomycin, second course.
• Generalized rash , sloughing of folds, face, oral mucosa and pharynx
10/2/2013
5
Case #5
• Diagnosis : SJS/ TEN (sloughing progressed to cover nearly the whole body)
• Treatment :STOP Vanco.Wound care , Electrolyte balance,Feeding, Prednisone and Cyclosporin.
• Evolution: favorable– The rash kept progressing , conjonctiva spared
– vitals stable , stayed in ICU and HDU
– sloughing healing very quickly
SJS Treatment
• 1‐ Remove the causal medication+++
( easier to say than to do !!!)
– Antiepileptics,
– Sulphonamide antibiotics,
– Penicillins,
– Allopurinol
– Oxicam NSAIDs
SJS Treatment
• 2 ‐Send to a burns unit ( if not ICU) for acute skin failure:
– Monitoring of the fluid – electrolyte balance,
– Provision of enteral or parenteral nutrition,
ASAP nasogastric tube.
– Wound care
– Treatment of sepsis.
SJS Treatment
• 3 In addition, supportive care of affected mucosal surface
– Aggressive ocular lubrication
topical corticosteroids and topical antibiotic
– Amniotic membrane graft has improved the ocular prognosis
– Hygienic mouthwashes and topical oral anesthetics
– Monitoring for urinary retention
SJS Treatment specific so far
• IVIg :3g/Kg total dose in 2 to 3 IV– Retrospective studies: controversial results– Risk of liquid overload– Very expensive
• Steroids, Bolus IV less side effects than Prednisone oral– Again controversial studies
• Cyclosporin 3mg/kg PO• Plasmapheresis• Cyclophosphamide ( side effects+++)
Questions?
10/2/2013
6
Case # 6
• Male in forties
– Depressed , • Weak
• Febrile, chills
– Worsening of his previous skin disease:
Full body• Erythema
• Scales
• Small lymph nodes
Case # 6
• Diagnosis Erythroderma
• Dermatological emergency:
– Looses water and proteins through the skin,
– Infectious risk
• Treatment: Same for any erythroderma
– Water ( drinks,baths) , electrolytes,high protein intake
– Blood‐cultures and swabs on regular basis
– Moisturizing ( to keep water in)
– Topical steroid ( to treat the skin condition)
Case #6: Psoriasis
• Rapidly blanched by topical treatments but relapsed
• Given Acitretin
Case #7
• 75 Y old male
• Consulted one year ago for a generalized rash
• 3 weeks after Ramipril* started for high blood pressure
• Blanched twice by Prednisone given by emergencies and by Family Physician
Case#7
• Diagnosis : Erythroderma– Variable fever– Looses weight– Thirsty
• Plan:– Feed, moisturize, baths, topical steroids, infectious control
• First proposed diagnosis: Pityriasis rubra Pilaris• Treatment added :
– Methotrexate– Keeps under control for a few months
Case #7
• Relapses and worsens
• Many biopsies not specific: Lymphocytes and eosinophils
• Affects also hair and nails
• Mildly itchy
10/2/2013
7
Case #7
• Patient stops Methotrexate
• Tumors appear
• Third biopsies: CutaneousT cell Lymphoma CTCL.
• Sezary cells in blood:
• Tumoral SezarySyndrome
• Patient deceased after many therapeutic trials
Erythroderma
• Etiologies:– Previous dermatosis:65%
• Psoriasis 40%
• Dermatitis 20%
• Pitiriasis rubra pilaris 1%
– Drug reaction: 14%• Carbamazepine
• Penicillin, cephalosporin
• Allopurinol
– Idiopathic:16%
– CTCL, malignancies, GVHD,4%
• Prognosis:– Life threatening: Acute skin
failure.
– Risk • Sepsis
• Heart failure
• Loss of temperature control
• Hypoalbuminemia
• Renal failure
• DVT
• Pulmonary embolism
• Stroke by hypovolemia etc..
Erythroderma
• Management– Electrolytes – Proteins PO– Baths– Moisturizers: ointments to retain the water– Repeated swabs and blood culture, urine culture– Prevention DVT– Topical steroids, oral steroids needed mostly for drug induced ( DRESS)
– Never oral steroids for Psoriasis: bad relapses– Specific treatment of the cause.
Questions?
Drug Reaction with Eosinophilia and Systemic Symptoms
DRESS• Fever first
• Rash morbilliform
• Lymph nodes
• Affects multiple organs
– Liver ( hepatitis)
– Heart ( pericarditis)
– Kidney ( interstitial nephritis )
– Lung ( pneumonitis)
DRESS Rash
• Generalized morbilliform
• Often facial edema
• Sometimes vesicles, pustules ,purpura
• Can lead to erythroderma or exfoliative dermatitis
10/2/2013
8
Drug more often causal
• Allopurinol
with renal impairment and with Thiazide diuretic
• Antiepilepsy drugs
– Carbamazepin
– Phenitoin
– Phenobarbital
• Sulfonamide antibiotics
DRESS Diagnosis
• Triad – Fever
– Extensive drug rash
– Organ involvement
• Supported by– Eosinophilia
– Abnormal liver tests
• Medication can have been taken up to 8 weeks before the rash!
DRESS Criteria: at least 3/7
• Hospitalisation
• Reaction suspected to be drug related
• Acute skin rash
• Fever about 38 Celsius
• Enlarged lymph nodes at two sites
• Involvement of at least one internal organ
• Blood count abnormalities such as low platelets, raised eosinophils or abnormal lymphocyte count.
Dress Alarms
1. Beware of cross allergies:
between anticonvulsivant therapies
– Phenitoin
– Phenobarbital
– Carbamazepine
–Avoid the 3 types
2. Beware of genetic factor:
First degree relatives should avoid the same drug
Main reported drugs
• Abacavir• Allopurinol• Atenolol• Azathioprine• Captopril• Carbamazepine• Clomipramine• Dapsone• Diltiazem• Gold salts• Isoniazid
• Lamotrigine• Mexiletine• Minocycline• Nevirapine• Oxicam• NSAIDs*• Phenobarbitone• Phenytoin• Sulfasalazine• Sulphonamides• Trimethoprim(*NSAIDs: Nonsteroidal anti‐inflammatory drugs)
Questions?
10/2/2013
9
“Crack” vasculitis
• 3 severe cases in 1 year
• Many minor cases
• Crack is a cocaine to smoke
• Levamisole is in 69% of North American cocaine
• Levamisole provokes a vasculitis similar to Cryoglobulin
Rapid onset of necrotising vasculitis
Affect typically nose, ears
Limbs,
Rapidly extensive, painful+++
Levamisole vasculitis
Typical sequella Saddle nose
Levamisole vasculitis
• Often “lupus like” Blood work:
• Low complement
• Neutropenia
• Lupus anticoagulant
• ANCA
• Treatment very deceiving
– Stop the cocaine!!
– Bolus steroid
– No consensus for treatment yet
– Debridement , amputation often necessary
Levamisole Vasculitisto suspect if acral necrotic blisters
Minor cases heal quickly If offending drug is removed
Tumor emergency?Melanoma:
Identify by excisional biopsyInvasive Squamous cell carcinoma
10/2/2013
10
Avoid late diagnosis
More than 6 months of evolution Large lymph nodes, Palliative RX
Working in a “Skin‐team”
• Wound care nurse
• Wound care dermatologist
• Plastic surgeons
• Foot surgeon
• Chiropodist
• Oncologist
Thanks for including me!
• And all the
– Medical students,
– Electives,
– Geriatric Fellows,
– Family medicine residents
– Internal medicine residents
• Who have courageously approached Dermatology in St Joseph Hospital!
Hope it helps!
References
1)Gunderson CG, Martinello RA .A systematic review of bacteremias in cellulitis and erysipelas.J Infect 2012 .Feb;64(2):148-55.
2) Li J, Zheng HY. Erythroderma: a clinical and prognostic study.
Dermatology 2012;225(2):154-62. 3) Castelain F, Humbert P
Toxic Epidermal Necrolysis.Curr Drug Saf. 2013 Feb 1 ( ahead of print)
4) Chung C, Tumeh PC, Birnbaum R, Tan BH, Sharp L, McCoy E, Mercurio MG, Craft N.Characteristic purpura of the ears, vasculitis, and neutropenia,a potential public health epidemic associated with levamisole-adulterated cocaine.
J Am Acad Dermatol. 2011 Oct;65(4):722-5. 5) Cacoub P, Musette P, Descamps V , Meyer O, Speirs C, Roujeau JCThe DRESS syndrome: a literature review. Am J Med. 2011 Jul;124(7):588-97.