1. Oral Manifestations of Dermatologic Diseases

t/f: Desquamative Gingivitis is a diagnosis.

true

what is a clinical term to describe a manifestation of what is usually lichen planus, mucous membrane pemphigoid, or pemphigus vulgaris?

Desquamative Gingivitis

The following are all _______ diseases.

• Idiopathic lichen planus

• Lichenoid drug eruptions

• Lupus erythematosus

• Graft versus host disease

Lichenoid

The following are all _______ diseases.

• Pemphigus vulgaris

• Mucous membrane pemphigoid

autoimmune

The following are all _______ diseases.

• Erythema multiforme

• Stevens-Johnson syndrome/

• Toxic epidermal necrolysis

other immune-mediated

Desquamative Gingivitis

Desquamative Gingivitis have a predilection for what gender?

females

Desquamative Gingivitis may display sensitivity to…?

spicy foods

what condition?

• Diffuse, markedly erythematous gingiva

• Generally involves attached tissues

Desquamative Gingivitis

What condition?

• Purple, polygonal, pruritic papules

• Develops on flexor surfaces of extremities

Lichen Planus (cutaneous)

what are the 3 oral variants of lichen planus?

• Reticular

• Erosive/erythematous

• Ulcerative

lichen planus affects % of the population

1%

what is the most common oral form of lichen planus?

reticular

what condition?

• Often asymptomatic

• Posterior buccal mucosa

• Lacy white striations (Wickham striae)

• almost always bilateral

lichen planus reticular form

what condition?

• Often symptomatic

• Striae at periphery of erythema

lichen planus Erythematous form

Erythematous and ulcerative lichen planus may cause …?

desquamative gingivitis (Must be distinguished from mucous membrane pemphigoid or pemphigus)

what is the least common form of lichen planus?

ulcerative

what condition?

• Least common form

• Usually symptomatic

• Ulcers with striae and erythema

ulcerative lichen planus

lichen planus is associated with meds including…?

• NSAIDs

• anti-hypertensive agents

• anti-hyperglycemic agents

• cholesterol lowering agents

• anti-hypothyroidism agents

• anti-gout medications

• amalgam restorations

• cinnamon flavoring agents

• herbal remedies

what are some histopathologic features of lichen planus?

• Orthokeratosis or parakeratosis

• Degeneration of basal cell layer

• Colloid bodies

• Saw tooth rete ridges

• Band-like infiltrate of lymphocytes

what tx is indicated for lichen planus?

• Observation if asymptomatic

• Topical corticosteroids

  • Fluocinonide or clobetasol gel 0.05%

  • Dexamethasone mouthrinse 0.5mg/5ml

patients on topical corticosteroids for lichen planus should be monitored for…?

candidiasis

how should you instruct patients to use Fluocinonide or clobetasol gel 0.05%?

• Dispense: one 60g tube

• Dry and apply to affected area QID

how should you instruct patients to use Dexamethasone mouthrinse 0.5mg/5ml?

• Dispense: 600cc

• Swish 5 cc for 5 minutes and expectorate QID

lichen planus displays a % increased risk of developing SCCa. Reported cases are associated with which form?

0.9%

erosive

t/f: Life-long follow-up recommended for licehn planus patients

true

Systemic Lupus Erythematosus has a predilection for which gender?

8-10:1 female predilection

what is the average age at diagnosis of Systemic Lupus Erythematosus?

31 years

what are some early stage non-specific clinical features of Systemic Lupus Erythematosus?

• Fever, weight loss, arthritis, fatigue, malaise

• butterfly rash

• kidney and cardiac involvement

40-50% of butterfly rash in SLE occurs where?

malar area and nose

kidney involvement occurs in SLE in % of the time

40-50

what % of SLE patients have oral lesions?

5-40%

what are some features of oral lesions that occur in SLE patients?

• Lichenoid lesions of palate, buccal mucosa, or gingiva

• May involve vermilion (lupus cheilitis)

• Ulceration, pain, erythema, and hyperkeratosis seen in varying degrees

Chronic Cutaneous Lupus Erythematosus is also known as…?

discoid lupus

Chronic Cutaneous Lupus Erythematosus is exacerbated by exposure to ____

sun

in Chronic Cutaneous Lupus Erythematosus patients, healing results in…?

atrophy with scarring

Skin and oral lesions, but few or no systemic signs or symptoms is characteristic of what condition?

Chronic Cutaneous Lupus Erythematosus

what condition?

• Scaly red patches on sun-exposed skin

• Oral lesions identical to lichen planus

• Rarely without skin lesions

Chronic Cutaneous Lupus Erythematosus

Chronic Cutaneous Lupus Erythematosus

oral lesions in Lupus Erythematosus resemble…?

lichen planus

DIF of Lupus Erythematosus shows what antibodies in the granular band at basement membrane?

IgM, IgG, and C3

whgat tx is indicated for Lupus Erythematosus?

• Avoid sun exposure

• Mild disease managed with NSAIDs and anti-malarial medications

what is the 5-year survival of SLE?

95%

what is the 15-year survival of SLE?

75%

what is the #1 cause of death in SLE?

renal failure

SLE has worse prognosis when seen in men/women?

men

prognosis of SLE depends on…?

which organs involved and frequency of reactivation

what tx is indicated for chronic Cutaneous Lupus Erythematosus?

• Avoid sun exposure

• Topical corticosteroids

• Thalidomide or anti-malarial medications for resistant cases

% of chronic Cutaneous Lupus Erythematosus transforms to SLE

5%

% of chronic Cutaneous Lupus Erythematosus resolves after several years

50%

most chronic Cutaneous Lupus Erythematosus remain confined to ____

skin

what condition is seen in allogeneic hematopoietic stem cell transplant recipients?

Graft vs. Host Disease

how many allogeneic hematopoietic stem cell transplant recipients are there in the USA annually?

Graft vs. Host Disease

what is the MOA of graft vs host disease?

Engrafted cells recognize they are not in their own environment and attack host as perceived foreign body

systemic signs of Graft vs. Host Disease depends on…?

organ(s) involved and whether disease is acute or chronic

milder versions of Graft vs. Host Disease can be seen in patients with…?

• Better histocompatibility match

• Younger age

• Cord blood received

• Female gender

acute form of Graft vs. Host Disease is seen within ___ days of transplant

100

% of Graft vs. Host Disease patients are affected by the acute form

50%

how does acute form of Graft vs. Host Disease present?

• mild rash to diffuse sloughing (resemble TEN)

• Diarrhea, nausea, vomiting, liver dysfunction, abdominal pain

how long does it take for chronic form of Graft vs. Host Disease to develop?

continuation of acute form or develops >100 days after transplant

% of patients are affected by chronic form of Graft vs. Host Disease

33-64%

oral lesions in Graft vs. Host Disease resemble…?

lichen planus (both clinically and microscopically)

% of acute Graft vs. Host Disease patients have oral lesions. what % of chronic form?

33-75% of acute patients, 80% of chronic patients

what tx is indicated for Graft vs. Host Disease?

• Prevention

• Topical or intra-lesional steroids for oral ulcers

• Topical fluoride and/or sialogogues for patients with hyposalivation

patients with Graft vs. Host Disease display an increased risk of …?

oral SCCA

what test identifies auto-antibodies bound to patient’s tissue?

Direct Immunofluorescence

with direct immunofluorescence, Antibodies directed against human immunoglobulins are tagged with …?

fluorescein

with direct immunofluorescence, Patient’s tissue incubated with …?

anti-human antibodies

what test?

Section viewed with ultraviolet light source to determine area of antibody deposition

direct Immunofluorescence

what test identifies auto-antibodies circulating in patient’s blood?

Indirect Immunofluorescence

what test?

Frozen section of tissue similar to human oral mucosa incubated with patient’s serum

Indirect Immunofluorescence

what test?

Autoantibodies in serum bind to homologous structure in tissue

Indirect Immunofluorescence

When considering vesiculobullous disease… always include ______

intact epithelium

When considering vesiculobullous disease… Submit separate biopsies in:

• 10% formalin for H&E

• Michel medium for DIF

t/f: When considering vesiculobullous disease…Defer empiric treatment, if possible, until after biopsy is complete

true

which form of pemphigus is most common?

Pemphigus vulgaris

which form of pemphigus is rare, sometimes oral lesions?

Pemphigus vegetans

which form of pemphigus has skin lesions only?

Pemphigus erythematosus AND Pemphigus foliaceus

what condition?

Potentially life-threatening skin and mucous membrane disease where patients have antibodies that target desmosomes?

Pemphigus Vulgaris (PV)

what is the incidence of Pemphigus Vulgaris (PV)?

1-5 per million

what demographics are more affected by Pemphigus Vulgaris (PV)?

• Usually seen over 50 years of age

• No gender predilection

• More common in people of Ashkenazi Jewish descent

what are some clinical features of Pemphigus Vulgaris (PV)?

• Painful chronic oral sores, dysphagia, weight loss

• Oral lesions → 50% show oral lesions first, almost all show oral lesions eventually

• Positive Nikolsky sign

Histopathologic Features of what condition?

• Perilesional tissue required for diagnosis

• Intraepithelial separation above basal layer (SUPRA-basilar cleavage)

• Acantholysis of spinous layer

• Rounded Tzanck cells

• Generally positive with direct or indirect immunofluorescence

Pemphigus Vulgaris

what tx is indicated for Pemphigus Vulgaris?

• Corticosteroids

• Azathioprine

• Immunoglobulin therapy

Untreated Pemphigus Vulgaris has a % mortality

80%

what condition was Formerly known as “benign mucous membrane pemphoid” or “cicatricial pemphigoid”?

Mucous Membrane Pemphigoid

what is an Autoimmune disorder with autoantibodies to hemidesmosomes?

Mucous Membrane Pemphigoid

Mucous Membrane Pemphigoid is ___ times as common as pemphigus vulgaris

twice

what demographics are more affected by Mucous Membrane Pemphigoid?

• Average age= 50-60

• Female predilection= 2:1

what are some sub-types of Mucous Membrane Pemphigoid?

• Only ocular involvement

• Only oral cavity involvement

• Mucosal & cutaneous lesions

• Multiple mucosal surfaces, no significant skin disease

Mucous Membrane Pemphigoid is often restricted to …?

gingiva

% of Mucous Membrane Pemphigoid patients with oral lesions develop eye lesions

25%

what are some clincial features of Mucous Membrane Pemphigoid?

• Positive Nikolsky sign

• Symblepherons (adhesions)

• Entropions (inward turning eyelids)

• Trichiasis (irritation secondary to eyelids rubbing against globe)

Histopathologic Features of what condition?

• Sub-epithelial separation below basal layer (SUB-basilar cleavage)

• Mimics linear IgA disease, epidermolysis bullosa acquisita, and angina bullosa hemorrhagica

Mucous Membrane Pemphigoid

what tx is indicated for Mucous Membrane Pemphigoid?

• Topical steroids for oral lesions

• REFER TO OPHTHALMOLOGIST

most cases of Erythema Multiforme is secondary to …?

infection (HSV or mycoplasma pneumoniae)

what demographics are more affected by Erythema Multiforme?

Male predilection; mostly age 20-40

Erythema Multiforme has a characteristic _____ onset

rapid

what are some clinical features of Erythema Multiforme?

• Erythematous mucosal patches

• Shallow ulcers

• Entire perimeter of tongue may be affected

• Hemorrhagic crusts of lips

• Targetoid cutaneous lesions