Mucocutaneous Disorders

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Mucocutaneous Disorders

• Dentistry 645

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Mucocutaneous Disorders

• I. Immunologically-mediated
• II. Genetic

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Mucocutaneous DisordersImmunologically-mediated

• Pemphigus vulgaris
• Pemphigoid
• Lichen planus
• Lupus erythematosus
• Systemic sclerosis (scleroderma)
• Psoriasis
• Behcets syndrome

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Mucocutaneous DisordersGenetic

• Keratosis follicularis
• (Dariers disease)
• Epidermolysis bullosa

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Pemphigus vulgaris

• Etio autoAbs to desmosomal complex
• Clin adults, 30s-50s (ave 50 yrs)
• skin- vesicles, bullae, ulcers
• oral- same, may precede skin lesions palate,
  labial/buccal mucosa, ventral tongue
• both share Nikolsky sign

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Pemphigus vulgaris

• Histo intraepi/suprabasilar clefting,
  acantholysis, Tzanck cells
• IF IgG/C3 (direct and indirect)
• Tx syst. corticosteroids /- MTX, azathioprine,
  cyclophosphamide
• Prog 5-10 mortality, tx side effects

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Cicatricial pemphigoid(benign mucous membrane
pemphigoid)

• Etio autoAbs to basement membrane
• Clin older adults, 50s-70s (ave 60), females gt
  males (21), any mucosal surface (oral, eye,
  vaginal)
• oral- 90 of pts. bullae, ulcers desquamative
  gingivitis, palate, buccal mucosa

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Cicatricial pemphigoid(benign mucous membrane
pemphigoid)

• Histo clean subepithelial split
• IF linear BMZ IgG/C3 (direct only)
• Tx steroids (top/syst), tetracylines, ?OH
  measures
• Prog good but eye involvement (symblepharon)can
  lead to blindness

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Bullous pemphigoid

• Similar etiology to cicatricial pemphigoid, but
• no gender predilection
• oral lesions uncommon (20)
• circulating autoAbs present (indirect IF)

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Lichen planus

• Etio autoimmune ?, cytotoxic T cells
• Clin adults, female predom. (32), relatively
  common (1-2)
• skin- purple polygonal pruritic pap.
• flexor surfaces arms, legs (1-1.5 yrs)
• oral- reticular (common) or erosive, buccal
  muc., tongue, ging. (yrs-decs)

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Lichen planus

• Histo keratosis, saw-tooth rete, band-like
  lymphocytic infiltrate, basilar vacuolopathy,
  Civatte bodies
• Tx topical steroids, ? OH, CHX, tx associated
  candidiasis if necessary
• Prog good, close follow-up recommended,
  malignant trans.???

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Lupus erythematosus

• Classified as a type of chronic connective tissue
  disorder
• 2 forms
• Systemic- multiorgan involvement
• Chronic cutaneous- skin or mucosa primary sites
  of involvement

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Systemic lupus erythematosus(SLE)

• Etio autoimmune disorder, T and B cell
  dysfunction with autoAbs
• Clin females (81), 20s-40s FUO, arthritis,
  skin (butterfly rash), wt. loss, anemia, kidney,
  cardiac dis.
• oral- 5-25, erythema/ulcers/kerat. palate,
  buccal mucosa, gingiva

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Systemic lupus erythematosus(SLE)

• Dx 4/11 criteria from ARA specific autoAbs
  against dsDNA, Sm protein
• Histo lichenoid mucos/dermatitis and vasculitis,
  PAS material at BMZ, IF shaggy BMZ IgG, M /-
  C3, seen even areas of normal skin/mucosa
• ( lupus band test)

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Chronic cutaneous L.E.(discoid L.E.)

• Clin females (21), 4th decade
• skin scaly, erythematous patches face/scalp,
  scarring (UV sensitive)
• oral erosive/keratotic lesions, mimic erosive
  LP
• Lab ANAs in 1/3rd, rare dsDNA Abs

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Chronic cutaneous L.E.(discoid L.E.)

• Histo same as SLE
• Tx avoid sun exposure, topical steroids,
  anti-malarial meds
• Prog good, conversion to SLE in 5

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Erythema multiforme (EM)

• Etio Unknown in 50, 25 HSV, 25 drugs
• Clin young adult males, 20s-30s, widely variable
  presentations -
• mild EM minor
• mod EM major (Stevens-Johnson)
• severe toxic epidermal necrolysis

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Erythema multiforme (EM)

• Clin
• oral hemmorhagic erosions/ulcers, lips, buccal
  mucosa, tongue
• hemmorhagic crusting of lower lip
• Oral ocular and genital lesions EM major
  (Stevens-Johnson synd.)

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Erythema multiforme (EM)

• Clin
• skin many forms (multiforme), annular,
  elevated, central purpura target lesions
• Histo not Dx, subepithelial vesicles, necrotic
  basilar keratinocytes. IF not helpful.

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Erythema multiforme (EM)

• Tx mild - supportive care
• severe - systemic corticosteroids, ulcer/fluid
  management
• - avoid contact with inciting agent(s) -
  anti-HSV tx, if appropriate
• Prog mild - good, EM major - 2-10 mortality,
  TEN - gt 30 mortality

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Systemic sclerois (scleroderma)

• Clin females (31), 30s-50s
• claw-like deformities (sclerodactyly), internal
  organs (lungs, heart, kidneys, GI tract)
• oral microstomia, dysphagia, sym. widening of
  PDL, resorption of distal mandible

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Systemic sclerois (scleroderma)

• Histo diffuse, dense collagen
• Lab ANAs, esp. anti-Scl-70 Abs
• Tx often futile penicillamine, azathioprine
  Photochemotx for skin
• Prog poor, exc. for limited skin cases

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Systemic sclerois (scleroderma)

• CREST syndrome mild form of scleroderma
• Calcinosis cutis
• Raynauds phenomenon
• Esophageal dysfunction
• Sclerodactyly
• Telangiectasia

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Psoriasis

• Etio cytokine dysregulation
• Clin common (1-2), teens-20s
• erythematous plaques with keratotic scale, may
  follow local trauma (Koebner phenomenon)
  hairline, scalp, elbows, knees
• oral rare, white/red palatal plaques

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Psoriasis

• Tx UV exposure, coal tar derivs., topical
  steroids (mild-mod), PUVA or cyclosporine
  (severe)
• Prog good, persistent cases can be difficult to
  manage

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Behcets syndrome

• Clin males gt females, 20s-30s
• mouth, eyes and genital lesions
• oral multiple, aphthous-like ulcers
• ocular uveitis
• genital aphthous-like ulcers
• other - skin, CNS, joints, GI tract
• Lab pathergy test

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Behcets syndrome

• Tx topical or systemic corticosteroids /-
  immunosuppressives, colchicine
• Prog good, unless CNS involved

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Keratosis follicularis(Dariers disease)

• Etio abnormal desmosomal complex
• Genetics autosomal dominant (AD)
• Clinical childhood, adolescence
• multiple erythematous papules which become
  confluent, collect surface keratin ?
  foul-smelling crust
• oral small, white papules, cobblestone

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Keratosis follicularis(Dariers disease)

• Histo keratin plug, intraepithelial cleft
  (acantholysis), dyskeratotic cells (grains, corps
  ronds)
• Tx keratolytic agents, retinoids for skin,
  genetic counseling
• Prog good

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Epidermolysis bullosa

• Group of heritable blistering, mucocutaneous
  disorders
• Etio abnormal formation of structures
  responsible for epithelial cell adherence to each
  other, the basement membrane and CT

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Epidermolysis bullosa (AD)

• Genetics autosomal dominant (AD)
• Clin trauma exposed extremities knuckles,
  elbows, knees. heal with scarring. loss of
  nails.
• oral mild ging. recession, erythema
• Histo Subepithelial clefting
• Prog good

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Epidermolysis bullosa (AR)

• Genetics autosomal recessive (AR)
• Clin severe blistering/scarring with minimal
  trauma (mitten deformities)
• oral erosions, ulcers, microstomia
• Histo Subepithelial clefting
• Tx soft foods, ? OH ? blisters, infection and
  scarring as possible
• Prog poor

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Immunofluorescence Studies
INDIRECT IF
DIRECT IF
Patient serum (with autoAb)

Fluorescein labeled anti-human IgG


Control tissue
Patient biopsy (autoAb in tissue)
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Immunofluorescence StudiesDirect
Fluorescein labeled anti-human IgG

UV
microscopy
Patient biopsy (autoAb in tissue)
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Immunofluorescence StudiesIndirect
Patient serum (with autoAb)

Control tissue (monkey esophagus)

UV
microscopy
Fluorescein labeled anti-human IgG