lorraine l. rosamilia, md, faad€¦ · bilateral), can be fistulous buboes, groove sign,...

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Lorraine L. Rosamilia, MD, FAAD Geisinger Health System, Department of Dermatology – Scenery Park, State College, PA

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Page 1: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Lorraine L. Rosamilia, MD, FAAD

Geisinger Health System, Department of Dermatology –

Scenery Park, State College, PA

Page 2: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Disclosure

I have no conflicts of interest relating to

the content of this presentation

Page 3: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Objectives

Upon completion of this presentation,

participants should be able to:

Identify common clinical presentations of oral

and genital diseases

Clarify first line treatment regimens for these

processes

Determine systemic associations of these

diseases

Page 4: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant
Page 5: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Molluscum

STD in adults

MCV-1 usually

(MCV-2 in HIV),

poxvirus

Treatments vary,

generally anecdotal

Page 6: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Chancroid

Painful ulcer

(„kissing‟), LA/draining,

usually unilateral

male>female

GNR „school of fish‟,

Haemophilus ducreyi

azithromycin 1g x 1

Page 7: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Granuloma inguinale

(donovanosis)

Usually painless, no LA, slow course

‘beefy red’, serpiginous ulcers

GNR, Klebsiella granulomatis

Less communicable than other STDs

Parasitized histiocytes, Donovan bodies (ovoid, „safety-pin‟)

Doxy or TMP-SMX

Page 8: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Lymphogranuloma venereum

Painless ulcer, suppurative inguinal adenitis (chain/matted, can be bilateral), can be fistulous buboes, Groove sign, constitutional symptoms

GN Chlamydia trachomatis (L1-3)

Doxy or erythromycin

Page 9: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Syphilis

Page 10: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Syphilis testing

Which is nonreactive in latent and treated disease? RPR, VDRL (CSF mostly) – very sensitive (nontreponemal

antibody tests), active disease confirmatory, titer given

Duration of titer depends on severity of disease

Fade at 3yrs

Treponemal antibody tests? FTA-ABS (very sensitive for primary), MHA-TP, TP-PA,

other immunoassays/ELISA (very specific, early positivity for congenital and primary)

Present for life (usually)

Other? Darkfield, PCR – (direct bacterial detection), early disease

Page 11: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Herpes

Testing? PCR, DFA, Tzanck,

bx, culture

HSV-specific IgM not available (IgG can merely indicate seroconversion)

By adulthood, seroprevalence HSV-2? 20-80%

HSV-1? 50-80%

Page 12: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

ABCs of STDs

ABC (Chlamydia) Trachomas (chronic conjunctivitis, Africa/Asia)

D-K Genital tract infection

L1-3 LGV

Bubo “usually tender, enlarged, inflamed lymph node characteristic of several infectious

entities”

Gonorrhea, chlamydia/LGV, chancroid, syphilis, TB, bubonic plague (Yersinia, rat flea)

Chancre “usually painless, indurated, nonexudative ulcer that can be the presenting sign of

several infectious entities”

Syphilis, TB, trypanosomiasis (tsetse fly bite site), sporotrichosis

„Chancroid‟ (soft chancre), chancres more indurated?

Esthiomine “chronic ulcerations and scarring of the vulva, can have elephantiasis features”

LGV, granuloma inguinale, TB

Page 13: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Genital exposures

What are common allergens in baby wipes? Fragrance

Methylchloroisothiazolinone and methylisothiazolinone (MCI/MI, Kathon CG)

Iodopropynyl butylcarbamate

Quaternium-15

Benzalkonium chloride

If the scrotum is involved, is tinea likely or unlikely? Unlikely, candida/psoriasis/LSC/EM Pagets more likely

Itch but no rash? Neurologic, GI, MSK, psych

What is the usual predisposing factor for red scrotum syndrome? Long-term topical steroid use

Page 14: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

STDs: general (genital) points…

Know painful vs. painless, ulcerated vs.

non-ulcerated, acute vs. chronic,

communicable genital lesions/STDs

http://www.cdc.gov/std/tg2015/default.htm

Page 15: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Perianal streptococcal infection

Page 16: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Perianal strep

Group A beta-hemolytic strep

Blood-streaked stool in 1/3 patients, constipation

Testing Rapid strep or routine culture

3-6 wks after infection – UA, post-strep GN (10-25% of skin cases)

Guttate psoriasis association (strep 2-3 wks prior)

Trt amoxicillin vs. PCN, or erythromycin x 2-3 weeks

(clindamycin or mupirocin topicals)

Page 17: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Infantile pyramidal protrusion

Midline perineum

Typically follows

episode of diarrhea

Resolves

spontaneously

Page 18: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Erythrasma

Organism

Corynebacterium

minutissimum

Woods lamp

Coral pink

coproporphyrin-III

Antiinfective topicals

Erythromycin po x 1-

2 wks if extensive

Page 19: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

DDx

Inverse psoriasis Tinea

cruris

Candidal

intertrigo

Page 20: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

DDx Acrodermatitis

enteropathica

Irritant contact

dermatitis

Langerhans cell

histiocytosis

Page 21: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

DDx Pagets

Hailey-Hailey

Pemphigus

vegetans

Page 22: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Perianal pseudoverrucous papules and

nodules

Cause

Form of irritant

contact dermatitis

Prolonged waste

contact exposure

Likely in spectrum

with Jacquets and

GGI

Page 23: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Idiopathic scrotal calcinosis

Page 24: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Pearly penile papules

•Usually asymptomatic

•Idiopathic

•Uncircumcised>circumcised

males

Page 25: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Zoon (plasma cell) balanitis

•Red moist plaque

•Uncircumcised males

•Plasma cell infiltrate on path

•Circumcision curative

Page 26: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Lichen sclerosus

•Clinical figure of 8

•Risk scarring and

SCC

•Potent topical

steroid trt

Page 27: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Angiokeratomas

Page 28: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant
Page 29: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Diagnosis?

Page 30: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

MEN2B

Other names Multiple mucosal neuroma, Wageman-Froboese,

Williams

First symptom Mucosal neuromas – lips, tongue, conjunctiva

Difference from 2A (Sipple) Mucosal, marfanoid, and megacolon features more

prominent in 2B; 2A can have amyloid skin changes, and parathyroid neoplasms

Difference from Cowdens (mucosal) Oral papillomas (whole mouth), high arched palate

Page 31: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Misc lesions

Oral hairy

leukoplakia

-EBV, HIV pts,

lateral tongue

Leukoplakia

-premalignant/

malignant

Erythroplakia

-

>>premalignant/

malignant

Page 32: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Misc lesions Geographic tongue

Mucocele

Smokeless

tobacco

leukoplakia

Torus palatinus

Page 33: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Orofacial granulomatosis

Noncaseating

granulomas,

upper>lower lip

Associations

Crohns

Sarcoidosis

Allergic

Infectious

Melkersson-Rosenthal

○ Facial nerve palsy,

facial swelling, fissured

tongue

Page 34: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Tumors

What is the most common site in the oral cavity for melanoma? Hard palate

SCC? Lateral and ventral

tongue, floor of mouth

What are the new guidelines for HPV vaccination? https://www.fda.gov/New

sEvents/Newsroom/PressAnnouncements/ucm622715.htm

Page 35: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant
Page 36: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Perioral

HSV

HPV

Molluscum

Perioral dermatitis

Contact dermatitis

Cheilitis

Perleche

Perianal

HSV

HPV

Molluscum

Scabies

IBD

Hidradenitis

Contact dermatitis

Page 37: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Fixed drug eruption

Recurrent eruption,

same location

Sooner with each

exposure

Laxatives, Abx,

NSAIDs

Page 38: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

What is this?

Page 39: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Lichen planus

Autoimmune inflammatory eruption of

skin and mucous membranes

1% incidence US population

Often self-limited

Most cutaneous cases resolve by 18 mo

(85%)

Mucous membrane disease more

chronic/recalcitrant (5 yrs avg)

Page 40: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Clinical Features

Pruritic purple polygonal (planar) papules/plaques

Wickham striae – gray/white streaks or puncta crossing the lesions

Flexors, oral/genital, nails classic, but most surfaces possible

Pruritus most prominent on skin, pain and burning possible on mucous membranes

Page 41: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Clinical variants

Mucosal

Classic white

reticulated plaques

on buccal mucosa,

but can involve any

mouth surface

Erosive disease more

symptomatic

Genital skin can have

similar lesions

Page 42: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Clinical variants

Nail

Hypertrophic

Annular

Atrophic

Bullous

Follicular

Drug-induced

Others…

Page 43: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Diagnostics, workup

Clinical appearance, symptoms, drug history, skin biopsy

Hepatitis C screening

R/O drug or contactant triggers

Assess steroid/immunosuppressant risks

Biopsy of outlier lesions to R/O SCC

Page 44: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Management

Topical steroids and calcineurin inhibitors

Intralesional steroid

Systemic steroids, other

immunosuppressants

Light therapy

Oral retinoids

Pruritus control?

Page 45: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Cheat sheets

LP is pruritic but often rubbed, not scratched like scabies

LP can Koebnerize

SCC and hepatitis C risk higher in mucosal disease

Medication triggers Antimalarials, NSAIDs, PPIs, sildenafil,

any antihypertensive, gold, allopurinol (many others)

Slow to resolve after D/C offending drug

Contactant triggers Oral metals, flavorings (menthol, mint,

cinnamon, lemon oil), film developers, hair dye, tattoo ink

Page 46: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Cheat sheets

LP is most common cause of trachyonychia (20-nail dystrophy) and dorsal pterygium (v-shaped skin lifting and scarring between proximal nail fold and matrix)

Oral lesions typically appear on tongue and buccal mucosa; genital lesions on glans, labia, anus

Oral and genital exams are difficult – ensure specialty care is up-to-date

Page 47: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Lichen planus take-homes

LP has a myriad of presentations, triggers, and clinical courses

Erosive oral disease is particularly chronic and symptomatic

Co-association with hepatitis C should be considered

SCC development is possible, particularly on mucosal surfaces

Specialty care is paramount for more symptomatic, extensive, and chronic cases

Page 48: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Behcet disease

Oral aphthous ulcers

Genital ulcers

Uveitis

Dx?

Trt?

Page 49: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Other ulcers?

Herpes

Mono

Baboon

Hand/

foot/

mouth

Aphthous

stomatitis

Page 50: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Dangerous things?

Pemphigus, pemphigoid

Stevens-Johnson/TEN

Tumors

Not dangerous?

Hand-foot-mouth

Page 51: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Pemphigus

Vulgaris

Paraneoplastic

Page 52: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

Pemphigoid

Bullous

Cicatricial (MMP)

Page 53: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

TEN/SJS HSV

Mycoplasma

Drug

All: High risk superinfection Hospitalization

Fluid resuscitation

“JAMA

Dermatology

patient page”

Page 54: Lorraine L. Rosamilia, MD, FAAD€¦ · bilateral), can be fistulous buboes, Groove sign, constitutional symptoms ... Torus palatinus . Orofacial granulomatosis ... R/O drug or contactant

References Nico MM, et al. Oral mucosal manifestations in some genodermatoses:

correlation with cutaneous lesions. Eur J Dermatol. 2013;23(5):581-91.

Pickert A. Concise review of lichen planus and lichenoid dermatoses. Cutis.

2012;90(3):E1-3.

Ramos-e-Silva M, et al. Oral involvement in autoimmune bullous diseases.

Clin Dermatol. 2011;29(4):443-54.

RS Arvind Babu, et al. A Study on Oral Mucosal Lesions in 3500 Patients

with Dermatological Diseases in South India. Ann Med Health Sci Res.

2014; 4(Suppl 2): S84–S93.

Stamm AW, et al. Urologic dermatology: a review. Curr Urol Rep.

2017;18(8):62

Thete SG, et al. Oral Manifestation in Patients diagnosed with

Dermatological Diseases. J Contemp Dent Pract. 2017;18(12):1153-1158.

Yura E and S Flury. Cutaneous lesions of the external genitalia. Med Clin

North Am. 2018;102(2):279-300.