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Goals and Objectives: Dermatomyositis

• At the end of this lecture, the learner will be able to: • Skin Signs: • Work Up: 1. Identify benign growths of the • Heliotrope • Myopathy (may be amyopathic) • Cuticular dilated capillary loops • (ovarian, breast, lung) 2. Identify manifestations of collagen vascular disease on the face • Others: • Interstitial lung disease • Anti‐Jo1, Anti‐MDA5 3. Appreciate the difficulty in identifying maligna • Gottron’s • Mechanics • Tx: 4. Create a differential diagnosis of perioral, peri‐ocular, labial, and • Poikiloderma atrophicans vasculare • Prednisone malar lesions and • Shawl sign • Hydroxychloroquine • V‐sign • Methotrexate 5. Implement basic treatment paradigms of common conditions, • cutis including , , and eczema • scaling • Mycophenolate mofetil • IVIg

Heliotrope Rash Dermatomyositis ‐ heliotrope eyelids

Heliotrope, the flower

She is not wearing eyeshadow. Dermatomyositis Seborrheic Dermatitis Gottron’s papules & cuticular dilated capillary loops • Same etiology as …BUT ALSO…. • Caused by Pityrosporum fungus • Signs: scaling and of: • Brow • Paranasal gutters • Posterior auricular (behind ) • Conchae of ears • Scalp (a.k.a. dandruff) • Chest • Worse in HIV • Treatment: ketoconazole 2%, pimecrolimus, hydrocortisone 1%

melanoma Seborrheic Dermatitis Seborrheic Dermatitis in HIV from Topical Steroids • Looks like a mix of acne and eczema • Acquired • DDx: allergic contact • no zinc in baby formula dermatitis (e.g., to • alcoholics toothpaste) • Genetic • Treat: • acrodermatitis enteropathica • tacrolimus • autosomal recessive • pimecrolimus • zinc malabsorption • d/c topical steroid

Photo from: http://healthh.com/wp‐ content/uploads/2014/05/perioral‐ dermatitis‐pictures‐3.jpg

Scleroderma

• Pinched nose • Immune‐mediated • Tightened orifice displaying teeth depletion of melanocytes • Few wrinkles • Tx: tacrolimus & excimer laser • Telangiectases

• Therapy: • Mycophenolate mofetil • Methotrexate • Prednisone pernio (sarcoidosis of face) Discoid Lupus

• Aka chronic cutaneous • Cutaneous sarcoidosis • Hyperpigmented border, hypopigmented/pink center • Nose, scalp with scarring alopecia • Face, ears, scalp (with scarring alopecia) • More indurated than DLE • • Therapy: Therapy: hydroxychloroquine; thalidomide • infliximab • intralesional triamcinolone 5‐10mg/cc

Discoid Lupus Acute systemic lupus

• “Butterfly” malar (cheek) rash • Photosensitivity • Spares nasolabial fold Lupus vulgaris : note annular, scaly border (cutaneous tuberculosis) • + PPD • Search for TB elsewhere Treatment: • Slowly progressive terbinafine 250mg po qd x 14 days

Psoriasis Seborrheic dermatitis

• Can look like seb derm Therapy: • but more well‐demarcated • Ketoconazole 2% cream bid than seb derm • • Treat face with: Topical calcineurin inhibitors • Tacrolimus • Tacrolimus • Pimecrolimus • Pimecrolimus • Topical steroid • mid‐potent steroid • for flare, not for maintenance Eczema Psoriasis Dry, scaly, red plaques Well‐demarcated Red Often impetiginized Plaque(s) • swab culture Silver scale • mupirocin New therapy: Therapies: Anti‐IL 23 agent: guselkumab • 90% of folks get 75% clear • Tacrolimus 0.1% ointment • 40% of folks get 100% clear • Pimecrolimus 1% cream Very safe! • Crisaborole 2% ointment Primary care can do it! • Topical steroids

Pityriasis alba Allergic • Associated with eczema • Treatment: Diagnosis: • Tacrolimus • Patch testing • Pimecrolimus • Hydrocortisone • Crisaborole Therapy: NEW! • Allergen avoidance Dupilumab • Topical steroids • IL 4/13 receptor antagonist • Tacrolimus • moderate to severe • Pimecrolimus eczema • safer than prednisone • may cause conjunctivitis Intradermal – scalp, flesh colored Intradermal nevus – face

• Benign rest of non‐functional eccrine (sweat gland) cells • Cluster of 1 to 3 mm dermal papules • Eyelids or upper cheeks Compound nevus • Onset: puberty or adult Junctional nevus - • Tx: electrofulguration but recur with risk of post‐inflammatory brown macule

Intradermal nevus: Junctional nevus: nests in only nests in dermo- epidermal junction only

• Small, mamillated yellow papules with telangiectasia and umbilication • depressed center, like “inny” belly button • Confused with basal cell carcinoma • Harmless Compound nevus: • nests both in dermis Seen in folks with rosacea and in D-E junction Seborrheic

• Warty, stuck‐on papule • Greasy • Tan to black • Elderly –“barnacles of life”  • VERY COMMON LESION • BENIGN…but if something looks funky to you, always involve the dermatologist

Milium Dermatosis Papulosa Nigra (pl. milia) • Many little SKs • Small epidermoid • Treat via I&D Incision Extraction with comedone extractor Traction behind blade = GOOD (or long finger nails :O)

Epidermoid Cyst: Schematic

• Misnomer = gets occluded  Skin still sloughing below occlusion  collects and causes and scarring around it  Capsule/cyst wall formation • Periodically, keratin discharges (smells!!!) accounting for “growing and shrinking” course Epidermoid Cyst

Central punctum

Central punctum

Nevus areneus (aka spider telangiectasia) Nevus araneus

• Associations: cirrhosis • Therapy: • Electrodesiccation of central feeder vessel • Pulse dye laser abaltion

Spider photo from: http://zerotermitepest.com.au/spider‐control‐sunshine‐coast/ Hidrocystoma

• Translucent, fluid‐filled cyst on eyelid • Lined by sweat gland • Treatment: • Incision and drainage • Excision • Electrofulguration

Xanthelasma Hordeolum (stye)

• Yellow plaques on • acute focal infection eyelids • staphylococcal • Sometimes associated • with • glands of Zeis hypertriglyceridemia, • external hordeola –lash but often, not found • meibomian glands • Tx: 100% TCA; • internal hordeola –tarsal plate electrodesiccation; ablative laser https://en.wikipedia.org/wiki/Stye BONUS FINDING: SYRINGOMA!!!! Chalazion Acrochordon • Chronic inflammation of Zeis or meibomian glands • Aka Therapy: • Pedunculated • warm compress • Snip excision • ILTAC 2.5‐5mg/cc • Cauterize base

By Michal Klajban (Hikingisgood.com) ‐ Own work, CC BY‐SA 3.0, https://commons.wikimedia.org/w/index.php?curid=11037123

Lentigo Maligna vs. Solar Lentigo Lentigo Maligna Melanoma vs. Solar Lentigo?

• Lentigo maligna = melanoma in situ, usu. face • Solar lentigo = hyperpigmented macule from excess sun exposure

To distinguish: • Time/progression • Color variegation • Size • Similar lesions • Old melanoma excision Lentigo Maligna Melanoma Lentigo Maligna Melanoma vs. Solar Lentigo? vs. Solar Lentigo?

Lentigo Maligna Melanoma vs. Solar Lentigo? Solar lentigo or melanoma?

Photo from: https://edrugsearch.com/age‐spot‐removal/ Solar lentigo –dirt comes off with alcohol!!! Basal Cell Cancer

• Rolled border • Telangiectasia • +/‐ Ulceration

• Does not usually metastasize BUT locally destructive

Basal Cell Carcinoma Squamous Cell Cancer (SCC)

• Morphology: • Hyperkeratotic • Red base

• Sun damaged skin; Organ transplant patients

• Metastatic risk • Mucosa (e.g., Lip) • • Genitalia • > 2cm • Recurrent SCC in situ SCC (Bowen’s disease)

SCC

Note background of photodamaged skin and actinic SCC keratoses SCC

• Rough, thin, scaly papule • sometimes felt, not seen • Precursor to SCC • Sun exposed areas: • bald scalp • face • forearms • dorsal hands

Actinic Keratoses Actinic Keratosis

forehead Solitary Hyperkeratotic Papule

Cutaneous Horn Verruca Vulgaris Seborrheic () keratosis Can be: red base, • Wart filiform pigmented, hyperkeratotic • SCC stuck on, • AK waxy

Cryotherapy –10 sec freeze After 5‐FU Osler‐Weber‐Rendu Disease Venous Lake

• Aka Hereditary hemorrhagic telangiectasia •  AV malformations GI bleed • Venous ectasia of lip • Epistaxis • Benign • Lip telangiectases • Tx: • Gene: • pulse dye laser • endoglin (ENG) • • activin receptorlike kinase type I (ALK‐1) electrodesiccation • Cf: Venous Lake

Peutz‐Jagher Syndrome Solitary Labial Lentigo –very common! • Brown macules of lips • Benign hamartomas polyps in GI tract  intussusception • Cancer predisposition • breast • pancreas • others • Gene: STK11/LKB1

• Cf: solitary labial lentigo –very common • Cf: Carney complex – cardiac myxoma, lentigines Figure from: https://openi.nlm.nih.gov/detailedresult.php?img= PMC3505710_medoral‐17‐e919‐g003&req=4 Carney Complex Angioedema (LAMB/NAME syndrome) • Labial lentigines • Allergic vs. hereditary • Cardiac myxomas • Angioedema + = allergic • Cancers • Therapy: • Endocrine abnormalities • Epinephrine • Prednisone • Gene: PRKAR1A • Antihistamines • Stop offending drug • E.g., ACE inhibitor

Figure from: https://www.researchgate.net/publication/234124406_The_complex_of_myxomas _spotty_skin_pigmentation_and_endocrine_overactivity_Carney_complex_Imagin g_findings_with_clinical_and_pathological_correlation/figures

Herpes Associated Herpes pearls… • Targetoid • Topical don’t work • Ulcerative enanthem • First outbreak (1o HSV1) • + HSV 1 or 2 serology worse than recurrent • Therapy: • 1st outbreak can be intraoral • Famciclovir • Subsequent usually just • Valacyclovir lip/nose • Apremilast • Dosing options for recurrent • Prednisone • Valacyclovir: 2g bid x 1d • Thalidomide • I use VZV dosing: 1g tid x 7d • Cyclosporine Angular Cheilitis Retinoid Cheilitis

• Maceration from drooling • Lip inflammation from isotretinoin • Candida • Tx: • Vitamin deficiency • decrease dose • Low potency topical steroid • Therapy: • Mid‐potent steroid • Clotrimazole • MVI

Figure from: http://www.webmd.com/oral‐health/angular‐ cheilitis#1

Actinic cheilitis My bunnies….. 

• Actinic keratosis of the lower lip • Due to chronic sun exposure • Precursor to SCC • Tx: • Imiquimod • 5‐fluorouracil

• CO2 laser • Photodynamic therapy More lectures at: http://bit.ly/dermedu Bonus Material!!!

Rosacea

• Pustules • Telangiectasia • Flushing • Sebaceous Sebaceous hyperplasia Hyperplasia • • Ocular rosacea (dry eyes) Rhinophyma Rosacea Therapy • Pustules: • Ivermectin cream 1% (kills Demodex folliculorum) • Metrogel is out • Azelaic acid –does ok • Doxycycline 40mg daily • Low dose isotretinoin (e.g., 10mg/d) • Erythema: • Brimonidine gel 0.33% daily –beware rebound erythema • Oxymetazoline cream 1% daily • Ocular: • Isotretinoin 1mg/kg/d x 5 months • Doxycycline 100mg po bid • Phymas/Sebaceous Hyperplasia: • Isotretinoin (1mg/kg/d) • CO2 laser ablation • Electrodesiccation

Lesions of Acne: Etiology Open Comedones (black heads)

• Papule: inflammation (vasodilation, edema), no pus • Pustule: pus, inflammation • Comedone: keratin plug in ostium of hair follicle Acne • Open comedone (aka blackhead): wide ostium, oxidized sebum, appears Topical retinoid black • Closed comedone (aka whitehead): narrow ostium, appears white (sebum remains protected from open air) • Cyst: deep encapsulated pustule Closed comedones (white heads) Comedone extraction

Acne surgery Topical retinoid

Acne Papules and Pustules Topical: Acne (here, too) • Clinda/BPO • Topical retinoid • Dapsone Endocrine w/u: • Sulfur • Prolactin • LH Oral: • FSH • Doxycycline • DHEAS • free and total For red : • Pulse dye laser Menstrual history for scars Doxy or Isotretinoin or ILTAC Acne Cysts Scarring from Untreated Acne

Isotretinoin  1‐1.5mg/kg/d x 5 months

Acne Acne – Pathophysiology Treatment post inflammatory hyperpigmentation Follicular occlusion Acne surgery (I&D) • Keratin viewed as foreign  inflammation • for comedones Retinoids (topical and oral) Intralesional triamcinolone (ILTAC) 2.5mg/cc Tx: • for cysts • hydroquinone Propionibacterium acnes Doxycycline hyclate 100mg po bid 4% bid Clindamycin 1%/benzoyl peroxide 5% gel bid • Chemical • clinda monotx  resistance peels Sebaceous hyperactivity Retinoids (topical and oral) • by androgens Oral contraceptives • food for P. acnes • ↑sex steroid binding protein  less available free testosterone Spironolactone 25‐100mg bid • androgen antagonist, esp. for PCOS Therapy Melasma Retinoid: sloughs pigmented stratum corneum (qd) Steroid: hypopigmentation (bid) Hydroquinone 4%: inhibits tyrosinase (bid)

Combo cream is qd due to retinoid, but retinoid is weak, so bid is ok

How Hydroquinone works: Pseudofolliculitis barbae • Pathophysiology: • Shave  hairs grow curving inward  skin views hair as “foreign”  inflammation  pustules and scarring • Treatment: • prevent close shave  no sharply angled hair tips • hydrate beard  thick shaving gel  bump‐fighter razor • hydrocortisone after shaving • Inhibit tyrosinase in basal layer  no new pigment forms • Shave less often • Old pigment sloughs • Laser hair removal • Dermal pigment, if present, remains Impetigo • Bullous and non‐bullous forms • Causes: Staphylococcus aureus, streptococci (often colonize nose) • Superinfects any defect in skin (eczema, arthropod bite, etc.) • Appearance: honey‐colored crust • Treatment: mupirocin = best 3x/day!!!; may require oral