Vesiculobullous and Ulcerative Lesions Part 2

Question 1

what is the etiology of benign mucous membrane pemphigoid

Answer 1

• autoimmune; trigger unknown
• autoantibodies directed against basement membrane zone antigens causes ulceration

Question 2

what is the clinical presentation for BMMP

Answer 2

• vesicles and bullae followed by ulceration
• multiple intraoral sites (occasionally gingiva only)
• usually in older adults
• 2:1 female predilection
• ocular lesions noted in one third of cases
• scarring tendency in ocular, laryngeal, nasopharyngeal and oropharyngeal tissues (cicatricial pemphigoid

Question 3

what is the nikolsky sign

Answer 3

• epithelial splitting
• application of firm lateral shearing force on uninvolved skin or mucosa can produce a surface slough or induce vesicle formation

Question 4

what is the dx for BMMP

Answer 4

• biopsy
• direct immunofluorescent examination

Question 5

what is the DDX for BMMP

Answer 5

• pemphigus vulgaris
• erythema multiforme
• erosive lichen planus
• lupus erythematosus
• epidermolysis bullosa acquisita

Question 6

what are the microscopic findings for BMMP

Answer 6

• subepithelial cleft formation
• linear pattern IgG and complement 3 along basement membrane zone; less commonly IgA
• direct immunofluorescence examination positive in 80% of cases

Question 7

what is the treatment for mucous membrane pemphigoid

Answer 7

• topical corticosteroids
• systemic prednisone, azathrioprine or cyclophosphamide
• tetracycline/niacinamide
  -dapsone

Question 8

what is the prognosis for MMP

Answer 8

• morbidity related to mucosal scarring (oropharyngeal, nasopharyngeal, laryngeal, ocular, genital)
• managment often difficult due to variable response to corticosteroids
• management often requires multiple specialists working in concert

Question 9

what is the etiology for pemphigus vulgaris

Answer 9

• an autoimmune disease where antibodies are directed towards the desmosome related proteins of the epithelial intercellular bridges - desmoglein 3 or desmoglein 1
• a drug induced form exists with less specificity in terms of immunologic features, clinical presentation and histopathology

Question 10

what is the clinical presentation for pemphigus vulgaris

Answer 10

• over 50% of cases develop oral lesions as the initial manifestation
• oral lesions develop in 70% of cases

Question 11

what is the clinical presenation of pemphigus vulgaris

Answer 11

• painful shallow irregular ulcers with friable adjacent mucosa
• nonkeratinized sites (buccal, floor, ventral tongue) often are initial sites affected
• positive nikolsky sign

Question 12

in pemphigus vulgaris there are autoantibodies against:

Answer 12

intracellular bridges

Question 13

what are the microscopic findings in pemphigus vulgaris

Answer 13

• separation or clefting or suprabasal from basal layer of epithelium
• intact basal layer of surface epithelium
• vesicle forms at site of epithelial split
• direct immunofluorescence examination positive in all cases
• igG localization to intercellular spaces of epithelium
• C3 localization to intercellular spaces in 80% of cases
• IgA localization to intercellular spaces in 30% of cases
• general correlation with severity of clinical disease

Question 14

what is the diagnosis for pemphigus vulgaris

Answer 14

• clinical appearance
• mucosal manifestations
• direct/indirect immunofluorescent studies

Question 15

what is the differential diagnosis for pemphigus vulgaris

Answer 15

• mucous membrane pemphigoid
• erythema multiforme
• erosive lichen planus
• drug reaction
• paraneoplastic pemphigus

Question 16

what is the treatment for pemphigus vulgaris

Answer 16

• systemic immunosuppression
• prednisone, azathioprine, mycophenolate mofetil, cyclophosphamide
• plasmapheresis plus immunosuppresson
• IV Ig for some recalcitrant cases

Question 17

what is the oral management for pemphigus vulgaris

Answer 17

• periodontal disease aggravates the condition
• forming a relationship for maintenance and observation with a periodontist is prudent management
• restorative treatment aggravates the condition

Question 18

what is the prognosis for pemphigus vulgaris

Answer 18

• guarded
• approximately a 5% mortality rate secondary to long term systemic corticosteroid related complications

Question 19

what is the etiology for lupus

Answer 19

• an autoimmune/immunologically mediated condition
• antibodies demonstrable against an array of cytoplasmic and nuclear antigens
• most often occurs in women

Question 20

what are the 3 forms of lupus

Answer 20

• chronic cutaneous (CCLE) or discoid (DLE)
• subacute cutaneous (SCLE)
• systemic (SLE)

Question 21

what is the clinical presentation for lupus

Answer 21

• black females have highest incidence
• predominates in women 40 years and older
• 80% of patients have concurrent cutaneous findings
• 30-40% of SLE patients have oral mucosal findings
• oral mucsoal lesions may appear lichenoid, keratotic, and erosive
• labial vermillion with crusted, exfoliative, erythematous, and keratotic appearance
• oral findings are most common in CCLE or DLE

Question 22

what are the topical treatments for lupus erythematosus

Answer 22

• flocinide gel/cream 0.05% 60g; apply after meals and at bedtime
• tacrolimus (protopic) ointment 0.1% 30g; apply after meals 3 times daily, do not ear or drink for 30 minutes
• intralesional therapy: triamcinolone acetonide 5-10mg/mL; inject 1-3mL per session with sessions at 3-4 week intervals

Question 23

what is the prognosis for lupus

Answer 23

• good prognosis with CCLE or DLE form
• variable prognosis with SLE
• SCLE has an intermediate prognosis between that of SLE and CCLE or DLE forms

Question 24

what is the etiology of erythema multiforme

Answer 24

• immunologically mediated reactive process, possibly related to circulating immune complexes
• many cases preceded by infection with herpes simplex; less often with mycoplasma pneumoniae or other organisms
• may be related to drug consumption, including sulfonamides, other antibiotics, analgesics, phenolphthalein containing laxatives, barbituates
• another trigger may be radiation therapy

Question 25

what is the clinical presentation for erythema multiforme

Answer 25

- acute onset of multiple, painful shallwo ulcers and erosions with irregular margins - early mucosal lesions are macular, erythematous and occasionally bullous - may affect oral mucosa and skin synchronously or metachronously - lips most commonly affected with eroded, crusted, and hemorrhagic lesions (serosanguinous exudate) known as stevens johnson syndrome when severe - predilection for young adults; 20-40 years

Question 26

what do the lesions look like in erythema multiforme

Answer 26

- target or iris skin lesions may be noted over extremities - genital and ocular lesions may occur - usually self limiting; 2-4 week course - recurrence is common

Question 27

what is the dx for erythema multiforme

Answer 27

- appearance - lip crusting - rapid onset - multiple site involvement in one half of cases - biopsy results often helpful but not always diagnostic

Question 28

what is the ddx for erythema multiforme

Answer 28

- viral infection in particular acute herpetic gingivostomatitis - erythema multiforme rarely affects the gingiva - pemphigus vulgaris - major aphthous ulcers - erosive lichen planus - mucous membrane pemphigoid

Question 29

what is the treatment for erythema multiforme

Answer 29

- mild (minor) form: symptomatic/supprotive treatment with adequate hydration, liquid diet, analgesics, topical corticosteroid agents - severe (major) form: systemic corticosteroids, parenteral fluid replacement;antipyretics - if evidence of an antecedent viral infection or trigger exists, systemic antiviral drugs during the disease or as a prophylactic measure may help

Question 30

what is the prognosis for erythema multiforme

Answer 30

- generally excellent - recurrences common

Question 31

what is the etiology for stevens johnson syndrome

Answer 31

- a complex mucocutaneous disease affecting two or more mucosal sites simultaneously - most common trigger: antecedent recurrent herpes simplex infection - infection with mycoplasma also may serve as a trigger - medications may serve as initiators in some cases - sometimes referred to as erythema multiforme major

Question 32

what is one type of clinical presentation of stevens johnson syndrome

Answer 32

- labial vermillion and anteiror portion of oral cavity usually affected initially - macular lesions erode, then slough and ulcerate - later appear crusted and hemorrhagic - pseudomembrane; foul smelling presentation as bacterial colonization supervenes

Question 33

what is the second type of clinical presentation for SJS

Answer 33

- posterior oral cavity and oropharyngeal involvement leads to odynophagia, sialorrhea, drooling - eye involvement may occur - genital involvement may occur - cutaneous involvement may become bullous - iris or target lesions are characteristic on skin

Question 34

what is the dx for SJS

Answer 34

- usually made on clinical grounds - histopathology is not diagnostic

Question 35

what is the DDX for SJS

Answer 35

- pemphigus vulgaris - paraneoplastic pemphigus - mucous membrane pemphigoid - bullous pemphigoid - acute herpetic gingivostomatitis - stomatitis medicamentosa

Question 36

what is the treatment for SJS

Answer 36

- hydration and local symptomatic measures - topical compounded oral rinses - systemic corticosteroid use controversial - recurrent, virally associated cases may be reduced in frequency with use of daily, low- dose antiviral prophylactic therapy 0 acyclovir, famiciclovir - may require admission to hospital burn unit

Question 37

what is the prognosis for SJS

Answer 37

- good, self limiting usually - recurrences not uncommon