Allergies and Immunological Diseases

What is another name for Recurrent Aphthous Ulcer?

Recurrent Aphthous Stomatitis; Canker Sores

What is one of the most common oral mucosal apthoses?

Recurrent Aphthous Ulcer

In which demographic do you see Recurrent Aphthous Ulcer

Younger patients; 80% have their first ulceration before age 30

What is the cause of Recurrent Aphthous Ulcer?

No universal etiology but systemic disorders, stress, hormonal can be associated with aphthous ulcer. It is painful

Where would you find Recurrent Aphthous Ulcer?

Exclusively occur on movable mucosa (vs primary herpes)

What are the 3 types of Recurrent Aphthous Ulcer?

1. Minor

2. Major

3. Herpetiform

Minor Recurrent Aphthous Ulcer

Size: 3-10mm (small)

Healing time: 7-10 days

No scarring

1-5 lesions

Short duration

Few recurrences

Major Recurrent Aphthous Ulcer

1-3 cm (large)

Healing time: 2-6 weeks

Biopsy to R/out cancer

Causes scarring sometimes

1-10 lesions

Longest duration

More than minor; less than herpetiform

Herpetiform Recurrent Aphthous Ulcer

Size: 1-3mm (small)

7-10 days of healing time

No scarring

Up to 100 lesions

Most recurrences

Least common

What is another name for angioedema?

Quincke’s disease

What is Angioedema?

A diffuse edema (swelling) of tissue caused by permeability of deeper blood vessels

What are the two types of angioedema?

Hypersensitivity or medication (ACE inhibitors of things that end in -pril)

Where do you see angioedema?

Extremities (most common), face, trunk, neck

What are the clinical features of angioedema?

Rapid onset swelling, non-tender, single or multiple, resolves within 24-72 hours, itching, recurrent skin swelling, abdominal pain

What can angioedema be confused with?

Cervicofacial emphysema due to air in tissue

What is contact mucositis?

Lesions that result from the direct contact of an allergen with the oral mucosa and skin

What is another name for contact mucositis?

Lichenoid mucositis

What are some products that can cause reactions in contact mucositis

• Candy

• Chewing gum

• Toothpaste

• Medication

What are the clinical presentations of contact mucositis?

The presentations vary according to the delivery medium

• Toothpaste causes a diffused appearance

• Gum/candy cause a localized appearance

What does contact mucositis clinically look similar to

Lichen planus

What is the treatment for contact/lichenoid mucositis?

It will disappear within 1 week of discontinuation of product

What is lichen planus?

An immune mediated, benign, chronic dermatologic disease that often affects oral mucosa

What demographic do you mostly see lichen planus in?

F > M (3:2), and middle aged people

Where does lichen planus show up?

Buccal mucosa, gingiva, tongue, lip

What is the clinical presentation of lichen planus intraorally?

White lace-like striations (Wickham striae), maybe erythematous, bleeding

What is the clinical presentation of lichen planus on the skin?

Purple, pruritic, polygonal papules (4Ps)

What is reticular lichen planus?

More common, asymptomatic, lace-like (Wickham striae)

What is erosive lichen planus?

Not as common, painful, erythematous, bleeding

What is the treatment for lichen planus?

There is no treatment, only palliative. If patients are asymptomatic, do a follow up. If patients are symptomatic, use topical steroids. If a chronic condition, waxes and wanes. <1% may develop cancer

What is Behçet Syndrome?

A chronic recurrent autoimmune disease,20’s and 30’s, Mediterranean or Asian descent (Silk road)

What is the criteria for diagnosis of Behçet Syndrome?

Recurrent oral ulcers 3x/yr + 2 of

1. Recurrent genital ulcerations (75%)

2. Eye lesions (70%)

3. Skin lesions

4. + pathergy test (results are variable)

In which condition do you see arthritis

Behçet Syndrome

If a patient has an autoimmune condition, they are

More prone to having another autoimmune disease

What is sarcoidosis?

Multisystem granulomatous disorder of unknown cause

Which demographic does scarcoidosis affect?

F > M

African Americans >>> Caucasians

Prior to 50 yrs

At the start of sarcoidosis, what are you expected to see?

Variable symptoms

Where would sarcoidosis be located?

• Organ involvement most common

• Lungs

• Lymph nodes

• Skin

• Eyes

• Salivary glands

What would a chest x-ray look like in sarcoidosis?

90% will show abnormal chest x-ray

In almost all cases of sarcoidosis, what kind of tissue is involved?

Lymphoid

Name the most common types of sarcoidosis

• Lymphoid

• Pulmonary

• Cutaneous

• Ocular

What other things can show up in sarcoidosis?

25% of people have ocular involvement

25% of people have skin lesions

What are the intraoral manifestations of sarcoidosis?

Enlarged salivary gland, xerostomia

What is lupus pernio?

A type of sarcoidosis that is chronic, purple and has indurated lesions on head and neck

What is erythema nodosum?

A type of sarcoidosis that is scattered, nonspecific, tender, red nodules on lower legs

What is the diagnosis of sarcoidosis?

Increased serum angiotensin-converting enzyme (ACE) levels which is mainly located in the capillaries of the lung but also in the kidney

Use a chest x-ray!

What is no longer used for the diagnosis of sarcoidosis?

Kveim test

What is the treatment and prognosis of sarcoidosis?

• 60% of symptoms resolve spontaneously in 2 years

• Corticosteroids if patients have progressive disease

• 5-10% succumb to disease and have further complications

What is another name for granulomatosis with polyangiitis?

Wegener Granulomatosis

What is the cause of granulomatosis with polyangiitis?

Abnormal immune reaction, it causes vasculitis in small and medium blood vessels

What demographic does granulomatosis with polyangiitis show up in?

M = F, average age is 40

What are the clinical features of granulomatosis with polyangiitis?

• Commonly affects the respiratory system (nose, sinuses, throat, lungs)

• Nose bleeds, saddle nose deformity

• Kidney (most common cause of death)

What are general signs and symptoms of granulomatosis with polyangiitis?

• Fever, night sweats

• Fatigue, lethargy

• Loss of appetite

• Weight loss

What are the intraoral manifestations of granulomatosis with polyangiitis?

(2%) Strawberry gingiva is red, friable, granular gingiva, non-

specific ulcer

What is Erythema Multiforme (EM)

A blistering ulcerative mucocutaneous condition of uncertain pathogenesis. You see a spectrum of hypersensitivity reaction. The sisease lasts 2-6 weeks (self limiting); 20% have recurrent episodes

What is the cause of Erythema Multiforme (EM)?

• Herpes virus (HSV)

• Mycoplasma pneumoniae

• Medications

What are the clinical features of Erythema Multiforme (EM)

• You get prodromal signs a week before

• Crusting of the lips and sometimes the eyelids

• Intraoral ulcers

• Targetoid or bulls eye

What demographic does Erythema Multiforme (EM) affect?

Young M>F

What is the treatment for Erythema Multiforme (EM)?

• Steroids

• IV rehydration

• Topical anesthetic

• Discontinue drug

What are characteristics of minor Erythema Multiforme (EM)?

• Mild

• Young, Male > female

• Starts on skin of extremities, oral lesions appear

• Crusting on lips

• Usually due to herpes (HSV)

What are characteristics of major Erythema Multiforme (EM)?

• Wide spread skin lesions + 2 or more mucosal sites

• (Oral + ocular or genital)

• Ocular scarring may occur in severe cases (Symblepharon)

• Usually due to herpes (HSV)

What are characteristics of Stevens-Johnson Syndrome?

• Almost Always triggered by drugs (medication)

• <10% of body surface is affected

• Younger

• Initially on trunk

• Sever sloughing of skin

What are characteristics of Toxic Epidermal Necrolysis (Lyell disease)?

• Almost Always triggered by drugs (medication)

• ≥30% body surface is affected

• Older females

• Initially on trunk

• Sever sloughing of skin

What is another name for reactive arthritis

Reiter Syndrome

What is Reiter Syndrome?

A chronic disease that classically compromises a triad of three featuers:

• Urethritis

• Conjunctivitis

• Arthritis

What is the cause of Reiter Syndrome?

Unknown, 10% have HLA-B27

Oral lesions are 20% (nonspecific)

NSAID/AB if infection/steroids

What demographic does Reiter Syndrome show up in?

M:F (9:1)

What is Lupus Erythematosus?

An acute and chronic inflammatory autoimmune disease of unknown cause

What is the most common collagen-vascular disease?

Lupus Erythematosus

What demographic does Lupus Erythematosus show up in?

• Gender: F >>>M (8:1)

• Age: child bearing age (average age 31 yrs)

What are the 3 categories of Lupus Erythematosus?

• Systemic lupus erythematous (SLE)

• Chronic cutaneous lupus erythematous (CCLE)

• Subacute cutaneous lupus erythematous (SCLE)

How does Lupus Erythematosus show up in the kidneys?

One of the main complications that may lead to HBP, kidney failure and death

What complications do you see in Systemic Lupus Erythematosus?

• Weight loss

• Fever

• Arthritis

• Fatigue/malaise

Where does Systemic Lupus Erythematosus show up?

Skin, kidney, heart, lungs

What are the intraoral manifestations of Systemic Lupus Erythematosus?

• May appear lichenoid or non-specific

• 50% have butterfly rash over malar area of nose sparing the nasolabial fold

Lupus cheilitis in Systemic Lupus Erythematosus

Non-specific ulcer in

Systemic Lupus Erythematosus

Butterfly rash in

Systemic Lupus Erythematosus

What is Chronic Cutaneous Lupus Erythematosus (CCLE)?

Few or no systemic signs or symptoms, mild form

Skin and mucosal surfaces (25% of cases of oral lesions)

What are the clinical features of Chronic Cutaneous Lupus Erythematosus (CCLE)?

• Discoid lupus erythematosus: Scaly, erythematous patches on sun exposed areas

• Scar and hypopigmentation

What is another name for scleroderma?

Systemic sclerosis; Hide Bound Disease

What is scleroderma?

An immunologically mediated condition that is caused by an increase in collagen deposition

What are the types of scleroderma?

1. Systemic

2. Localized (Morphea-localized scar)

What demographic does scleroderma affect?

F > M (5:1)

What are the clinical features of scleroderma?

• Raynaud phenomenon (not specific for scleroderma)

• Mask-like face, loss of ala of nose

• Resorption of the terminal phalanges and flexion contractures to produce shortened, claw-like fingers

The limited symptoms of scleroderma are referred to as CREST

C-calcinosis- calcium deposits in the skin

R-Raynaud’s phenomenon-spasm of blood vessels in response to cold or stress

E-esophageal dysfunction, acid reflux and decrease in motility of esophagus

S-sclerodactyly-thickening and tightening of the skin on the fingers and hands

T-telangiectasis- dilation of capillaries causing red marks on surface of skin

What is Vesiculo-bullous Disease look like histologically?

What is Pemphigus Vulgaris?

A severe, progressive autoimmune disease that affects both the skin and mucous membranes

What is acantholysis in Pemphigus Vulgaris?

Epithelial cell separation “INTRA-EPITHELIAL”

What is Nikolsky sign in Pemphigus Vulgaris?

Gentle finger pressure with movement on clinically normal mucosa can produce acleavage in the epithelium and result in the formation of a bulla

What are Tzank cells in Pemphigus Vulgaris?

Loss of attachment between the epithelial cells results in detached cells that appear rounded, present in the area of separation

Sloughing of the epithelium Ulcers in Pemphigus Vulgaris

What is the diagnosis for Pemphigus Vulgaris?

Biopsy and Direct immunofluorescence (DIF)- INTRA-EPITHELIAL separation

What is the treatment for Pemphigus Vulgaris?

High does of steroids

What is another name for Mucous Membrane Pemphigoid?

Cicatricial pemphigoid and Benign mucous membrane pemphigoid

What is Mucous Membrane Pemphigoid?

A chronic autoimmune disease that affects the oral mucosa, conjunctiva, genital mucosa, and skin

Which is more severe, Mucous Membrane Pemphigoid or Pemphigus Vulgaris?

Pemphigus Vulgaris

How could Mucous Membrane Pemphigoid heal?

With scarring (cicatricial pemphigoid)

What demographic does Mucous Membrane Pemphigoid affect?

Gender: F > M (2:1)

Age: older adults (60’s) compared to PV

Where might you find Mucous Membrane Pemphigoid?

Skin and mucosa (mouth, genital, eye)

And intraorally: gingiva (most common)

What is a unique clinical feature of Mucous Membrane Pemphigoid?

Symblepharons (fibrous scars) along the eye