Skin and Mucosal Disease 2 Flashcards
What are the 2 stains/analyzing tools used for biopsies to distinguish between pemphigus, pemphigoid, and lichen planus?
- Half formalin-fixed for H&E
* Half frozen for direct immunofluorescence (DIF) ->Michel’s solution
________ immunofluorescence is a biopsy
• Performed on the patient’s tissue (biopsy specimen) using an anti-human antibody panel
• Direct immunofluorescence
______ immunofluorescence is a serological test
• Performed on animal tissue (esophagus) using the patient’s serum antibodies
• Indirect immunofluorescence
Histopathology of \_\_\_\_\_\_\_ •Hyperkeratosis •Saw tooth rete ridges -> elongated •Band- like infiltrate of lymphocytes •Liquefaction degeneration of basal cell layer
Lichen Planus
Does pemphigus destroy desmosomes or hemidesmosomes?
Desmosomes
Does pemphigoid destroy desmosomes or hemidesmosomes?
hemi desmosomes
Which disease shows a fish net pattern on DIF?
Pemphigus
Which disease shows an intraepithelial split?
Pemphigus
Which disease shows a sub epithetlial split?
Pemphigoid
Which disease shows a dark green band at basement zone on DIF?
Pemphigoid
An immunologically mediated self limited
mucocutaneous disease
Young adults
Sudden onset of widespread painful superficial
mucosal ulcers and target lesions of skin
Crusted ulcers on lips
May be recurrent
Erythema multiforme
Which disease shows bullseye/target/iris lesions?
erythema multiforme
Which type of hypersensitivity occurs in erythema multiforme?
Type 3
•Antibody response to an exogenous antigen produces circulating
immune complexes which are deposited in vessels
Type III Hypersensitivity Response seen in EM
The following are associated with \_\_\_\_\_\_: Drugs • Sulfonamides • Penicillin • Diphenylhydantoin • Tetracycline • Codeine • Ibuprofen • ~ 30 others Bugs • Herpes simplex • Mycoplasma pneumoniae • Epstein Barr Virus • Hepatitis B • Streptococci Other • Tumors (Leukemia, Lymphoma) • Immunization (DPT) • Inflammatory bowel disease • Sarcoidosis • Sunlight
EM
• Prodromal symptoms • Sudden onset of mucocutaneous lesions • Self-limiting disease • Lasts 2-6 weeks • Avoid dehydration • Recurrent episodes • Lesions may be limited to oral mucosa
Erythema Multiforme
•Stevens-Johnson Syndrome, a severe form of erythema multiforme, usually triggered by a drug, rather than an infection
Erythema Multiforme Major
- Most severe form of erythema multiforme, usually seen in older individuals, almost always triggered by drug exposure
- Diffuse sloughing of epidermis leading to a scalded appearance, fluid loss and infection
- Treated as severe burn victims
Toxic Epidermal Necrolysis (Lyell Disease)
- A multisustem autoimmune disease most common in adult women in child-bearing years
- Autoantibodies - anti-nuclear antibodies (ANA)
- Immune complexes are deposited throughout the body, especially kidney and blood vessels
Lupus Erythematosus
____ lupus erythematous
• Skin
• No systemic involvement
Discoid LE
____ lupus erythematous
• Skin
• Limited systemic involvement
Subacute cutaneous LE
____ lupus erythematous
• Systemic involvement
• Periodic skin rashes
Systemic LE
- Chronic, limited to skin and mucosa
- Sun-exposed skin
- Scaly, erythematous patches
- Cosmetic problems
- Cutaneous atrophy
- Scarring
- Pigmentation
Discoid Lupus Erythematosus
Oral Mucosal Lesions in \_\_\_\_\_\_\_\_\_ •May be seen in any form of disease •Red and white lesions that may be clinically identical to erosive lichen planus
Lupus Erythematosus
- Most common form of lupus
- Highest morbidity
- Kidney involvement –glomerulonephritis - renal failure
- Butterfly rash 50% - malar area and nose, aggravated by sunlight
- Libman-Sacks endocarditis
- Arthritis, arthralgia, heart and lung involvement, anemia, bone marrow depression, vasculitis, skin rashes
Systemic Lupus Erythematosus SLE
Tx of ________:
• Systemic corticosteroids
• Antimalarial drugs - Plaquenil
• Avoid excessive exposure to sunlight
• Prognosis depends on organs affected and disease activity
• Renal failure most frequent cause of death
Systemic lupus ery
- An autoimmune disease of adults, predominately females, characterized by excessive fibrosis
- May be limited to the skin or be widespread affecting various organ systems
- May be associated with other autoimmune diseases
Systemic Sclerosis
- Slow continuous replacement of loose fibrovascular connective tissue with dense collagen
- Fibrosis causes loss of mobility and altered function of organs - skin, esophagus, salivary glands, kidneys, lungs, heart, muscle
Systemic sclerosis
Oral manifestations of \_\_\_\_\_\_\_ •Microstomia •Xerostomia •Generalized widening of PDL space •Mandibular resorption
Systemic scleorsis
Mask-like face
•Telangiectasia
•Raynaud phenomenon
Systemic sclerosis
• Arterial spasm in response to cold or emotional stress • Pallor, cyanosis and then erythema • Numbness, tingling or pain on recovery.
Raynaud Phenomenon
Hands and Fingers •Fibrosis, stiffness, deformity •Ischemia, atrophy and ulceration
Systemic sclerosis
What makes up CREST syndrome seen in systemic sclerosis?
• Calcinosis • Raynaud’s Phenomenon • Esophageal dysfunction • Sclerodactly • Telangiectasia
• A group of mechanobullous diseases involving structural
defects in the attachment apparatus
• Many hereditary forms involving different defects in the
attachment apparatus
• Recessive dystrophic form is most severe, involving defective
type VII collagen anchoring fibrils
• Not an autoimmune disease. Defective structural proteins
Hereditary
Epidermolysis Bullosa
• Primarily a severe and debilitating skin
disease in which blisters form at sites of
minor trauma and may heal with scarring
(Nikolsky + )
Hereditary Epidermolysis Bullosa
Oral Sequalae
of _________
•Normal diet produces bullae that heal with scarring leading to obliteration of vestibule, ankyloglossia, microstomia and esophageal stricture
-Can have enamel hypoplasia in junctional type
Epidermolysis bullosa
What condition does this person have?
• My hands have been like this as long as I can remember
• I lost all my fingernails by age 2 and they fused into this fist shape
• I have never had hand surgery because I have chosen not to
• I have a lot of thumb space still free, meaning my thumbs have not fused, so I can do things such as write, type etc. with no problem
Epidermolysis Bullosa –Recessive Dystrophic
- A common, chronic, genetically-determined, inflammatory and hyperproliferative skin disease
- Exacerbations and remissions
- Association with certain HLA types
- Immunoregulatory disorder –T cells trigger inflammation
- Defect in control of keratinocyte proliferation -turnover rate 8x normal
Psoriasis
- Well-demarcated, red plaques covered by silvery scales
- Auspitz Sign - removal of scale leaves pinpoint bleeding area
- Koebner Phenomenon - lesions develop following trauma to normal-appearing skin
Psoriasis
•Removal of scale leaves small
pinpoint bleeding
-associated with psoriasis
Auspitz Sign
Development of lesions
following trauma to normal-
appearing skin
-associated with psoriasis
Koebner Phenomenon
Histopathology of ______
Intra-epithelial microabscesses
Psoriasis
What condition presents as the same lesion intra-orally as psoriasis?
Erythema migrans
Treatment of \_\_\_\_\_\_\_ •Topical medications - corticosteroids •Phototherapy - UVB •Photochemotherapy –PUVA (psoralen and UVA)
Psoriasis
•Hereditary, autosomal
dominant mutation of
keratin genes that
requires no treatment
White Sponge
Nevus
• Asymptomatic, bilateral, white lesions with a thick, folded, consistency
“spongy” appear before puberty
• Primarily involves buccal mucosa, but may affect other mucosal
surfaces –anogenital, esophageal
White sponge nevus
Histopath of ______:
Perinuclear keratin condensation
White Sponge Nevus
