Chapter 16: Dermatologic Flashcards
Autosomal Dominant
Thick white buccal mucosa bilaterally
Benign hyperparakeratotic
No treatment
White sponge nevus
Autosomal dominant NC Lumbi Indians Thick white plaques on buccal mucosa Dyskeratosis Benign, no Tx
Hereditary Benign Intrepithelial Dyskeratosis
Autosomal dominant
Red, itchy papules with foul ododr
Trunk and scalp
Ridges and split nails
Oral white papules
Keratolytic agents
Darier’s Disease
Keratosis Follicularis
Identical histology to Darier’s disease
Orally: Single white papule on hard palate or alveolar ridge
Tx: excision
Warty Dykeratoma
Isolated Darier’s Disesase
Autosomal dominant
Freckle like lesions in and around oral cavity and hands
Oral oncogenes
Intestinal polyposis that can change to adenocarcinoma
Peutz-jeghers syndrome
Autosomal dominant Vascular hamartomas Frequent epistaxis Telangiectasias: intraoral, hands, feet, GI, GU, eye Iron deficiency including anemia
Hereditary Hemorrhagic Telangiectasia
Osler-Weber-Rendu Syndrome
Abnormal collagen production due to genetic abnormalities
Hypermobility of joints and elasticity of skin
Ehlers-danlos syndrome
Number of closely related lichenoid reactions that reflect immunologic recognition of a variety of stimuli
Lichen planus
Purple
Polygonal
Papular
Pruritic
Skin Lichen Planus
Educate patients Photograph Erosive form see four times a year Biopsy Reassure
How to monitor Lichen Planus in patients
First used in 1895 to diagnose pemphigus
Dislodgement of skin by lateral pressure
Multiple Diseases
Nikolsky sign
Average age 60
Women 2:1
Oral Lesions: Conjunctival, nasal, esophageal, laryngeal, vaginal
Clinical vesicles and bullae
Benign mucus membrane pemphigoid
Vesicles, bullae, ulcers occur anywhere in oral cavity
Blood filled characteristics
Complaints of oral bleeding, sore gums, difficulty swallowing
Clinical signs of Benign Mucus Membrane Pemphigoid
Dapsone (CI: G6PD deficiency)
Steroids
Tetracycline & Niacinamide
Immunosuppresive agents
BMMP Tx
Mucocutaneous AI disease characterized by acantholysis due to immune complex deposition at cellular attachment bridges
Intraepithelial clefting
Pemphigus Vulgaris
Adults age 50
Rare in kids
Males = Females
Almost all cases have oral involvement
Clinical signs of Pemphigus Vulgaris
Intra-epithelial separation
Immunofluorescence testing shows autoantibodies to the spinous layer of skin
Pemphigus Vulgaris
Jewish predilection
Ragged erosions and ulcers of ANY oral mucosa
Bullae rupture early and are rarely seen by DDS
(+) Nikolsky sign
Pemphigus vulgaris
Oral lesions precede skin lesions
Large, ragged ulcerations
Marginal gingiva erosions early
1-5 Million /year
Pemphigus vulgaris
Oral lesions may be the first to present in the potentially fatal disease
Blistering due to autoimmune attack of desmosomes
Pemphigus vulgaris
The oral lesions are the first to show and the last to go!
Pemphigus vulgaris
Incisional biopsy ASAP
Steroids from dentist and dermatologis
60-80% die w/o steroids
5-10% dies with steroids
Pemphigus vulgaris
Intraepithelial separation just above the basal cell layer leaving “row of tombstones”
Acantholsysis with floating Tzanck cells
Positive indirect IF for intercullular IgG, IgM, C3
Pemphigus Vulgaris Histology
Cross-reacting antibodies in lymphoma or leukemia attack desmosomal complex
May precede the discovery of the underlying malignancy
Often fatal.
Paraneoplastic pemphigus
Neoplasia-Induced Pemphigus
Prioons to steroids, 60-80% cases fatal from infections and electrolyte imbalance
Today 5-10% mortality from complications of medications to manage
PV Treatment
Occurs in recipients of allogenic bone marrow transplants
Graft vs. Host Disease (GVHD)
HLA matched donor
Hematopoietic stem cells from either bone marrow, peripheral blood or umbilical cord blood
Engrafted cells recognize body as foreign
Graft versus host disease
Conical teeth Hypohydrosis Hypodontia Missing sweat glands Missing hair
Ectodermal dysplasia
Milder disease seen:
Patients with better histocompatibility match
Younger patients
Cord blood
Females
Graft versus host diesease
Occurs a few weeks after transplant
Affects 50% of patients
Mild rash to TEN
Diarrhea, nausea, vomiting, abdominal pain and liver dysfunction
Acute Graft versus Host Disease
Continuation of acute disease
100 days to years and develops in 33-64%
Mimics autoimmune disease
Skin lesions resemble LP or systemic sclerosis
Chronic GVHD
33-75% of AGVHD and 80% of CGVHD
Resembles lichen planus
Some complain of burning sensation (R/O candidiasis)
Oral Graft vs. Host Disease
Ulcerations that are related to chemotherapeutic conditioning and neutropenic state develop first two weeks after BMT
Ulcers that last longer then two weeks AGVHD
Oral GVHD
Xerostomia is a common complaint
Immunologic response destroying salivary glands
Mucoceles of soft palate
Oral GVHD
Oral lesion highly predictive index of this disease
Goal of therapy is to prevent occurrence
Careful tissue histocompatibility matching is key
Graft versus host disease
Prophylactic therapy with immunomodulatory and immunosuppressive agents such as cyclosporine & prednisone
Methotrexate has been added and reduced disease further
Treatment of Graft vs Host Disease
Thalidomine has shown some promise
Topical CS oral lesions
Psoralen and Ultraviolet A (PUVA) improve lichenoid form
Treat xerostomia
Treatment of Graft v. Host Disease
Increased proliferative activity of cutaneous keratinocytes
Immunologic with activated T-lymphocytes
Psoriasis
Well demarcated silvery plaque with silvery scale of surface
Non-symptomatic to itching
Psoriatic arthritis
Signs of Psoriasis
2nd-3rd decade, persists for years
Improves during the summer/UV light
Symmetrical/scalp, elbows, knees
Oral uncommon?
Psoriasis
Mild disease has no treatment
Moderate: coal tar, keratolytic agents & UV light, Vit. D analogs, retinoids, trazarotine and calcipotriene
Severe cases: PUVA, methotrexate and cyclosporine
Treatment of psoriasis
Parakeratosis with elongated rete ridges
CT papillae dilated capillaries close to epithelial surface
Perivacular chronic inflammation
Munroe abscesses
Histology of Psoriasis
Immunologically mediated condition
Most common of the collagen vascular disorders
1.5 million affected
Lupus Erythematous
Increased activity of the humoral limb of the immune system (B lymphocytes)
Abnormal function of T lymphocytes
Genetic factors (identical twins 32%/fraternal twins 6%)
Systemic lupus erythematous
Woman 8x
Average age at diagnosis is 31
Weight loss, arthritis, fatigue, malaise
40-50% butterfly rash
Sunlight makes lesions worse
Systemic lupus erythematous
40-50% kidney problems, may lead to renal failure (most significant aspect of disease)
Cardiac: Pericarditis, 50% at autopsy have warty vegitations of heart valves (Libman-Sacks endocarditis), sterile overgrowth of fibrinoid material and CT celles (may lead to BE)
Systemic lupus erythematous
5-40% of cases
P, BM, and G, appears lichenoid
Lupus cheilitis
Varying degrees of ulceration, pain, erythema and hyperkeratosis
Systemic lupus erythematous Oral
Ulcerated or atrophic with erythematous central zone surrounded by white radiating striae and may show fine stippling of white dots
Lichenoid and may be painful
Oral lesions of Lupues erythematous
Scaly erythematous patches of sun exposed skin, head and neck common, sun makes it worse
Heal in one area only to appear in another
Oral lesions not without skin
Lichenoid appearance
Chronic Cutaneous Lupus Erythematous
Patch deposits of PAS and material in BM zone
Subepithelial edema
Direct IF show deposition of one or more, IgM, IgG or C3 in a shaggy granular band at BM zone
Normal skin shows and lupus band test
Histology of Lupus erythematous
95% of patients + ANA
Antibodies against double stranded DNA in 70% patients with SLE more specific for the disease
Lupus erythematous
With treatment 5 year survival = 95%, 15 year = 75%
Depends of organs affected and remissions, renal failure most common cause of death
Worse for men
Prognosis of systemic lupus erythematous
Avoid sun exposure
NSAID with antimalarial drugs (hydroxycloroquine) for mild disease
Severe disease: heart, renal, thrombocytopenia, arthritis (CS + other immunosuppressive agents)
Treatment of Lupus erythematous
Known as scleroderma and immunologically mediated
Dense collagen deposited in tissues
Adults: Women 3x
First sign is Raynaud’s phenomenon (vasoconstrictive event triggered by emotional distress or exposure to cold)`
Systemic sclerosis
Organ involvement is subtle at first
Fibrosis of lung, heart, kidney, and GI tract
Pulmonary fibrosis significant leading to pulmonary hypertension and heart failure are primary cause of death
Systemic sclerosis
Acro-osteolysis: resorption of terminal phalanges and flexure contractures produce shortened club-like fingers
Vascular events and abnormal collagen deposition produces ulceration of the fingertips
Systemic sclerosis
Collagen deposition results in smooth, taut, mask-like face
Nasal alae atrophied in a pinched nose (mouse facies)
Systemic sclerosis
Microstomia 70%, pulse string furrows radiating from mouth
Loss of attached gingiva
Dysphasia: deposition of collagen results in hypomobile tongue and inelastic esophagus hindering swallowing
Oral systemic sclerosis
Widened PDL space
Resorption of posterior ramus, coronoid process and condyle in 10-15% of patients (resorbed due to increased pressure associated with abnormal collagen production)
Oral systemic sclerosis
D-penicillamine inhibits collagen production
Esophageal dilation
Calcium channel blockers help peripheral blood flow
Angiotension-converting enzyme for HTN if kidney is involved
Treatment of systemic sclerosis
Hard to wear dentures with microstomia and inelasticity of mouth
80% survival = 2 years
30-50% = 8 years
15-3-% = 15 years
Systemic Sclerosis
Calcinosis cutis
Raynaud’s phenomenon
Esophageal dysfunction
Sclerodactly
Telangiectasias
CREST Syndrome
Movable, non-tender subcutaneous nodules
.5 cm to 2 cm in size
Deposition of calcium salts
Calcinosis Cutis
Dramatic blanching of digits (dead white) when exposed to cold, turns bluish few minutes later (venous stasis)
After warming dusky-red hue (return of hyperemic blood flow)
May have throbbing pain
Raynaud’s Phenomenon
Cause is abnormal collagen deposition in esophageal submucosa
not noticeable early in CREST but may cause difficulty in swallowing later
Barium swallow x-ray studies to diagnose
Esophageal Dysfunction
A result from fibrosis and atrophy of smooth muscle in the GI tract
Decreased function may cause: hyper-mobility, dysphasia, reflex esophagitis and fibrotid strictures
Symptoms are not progressive, but are not reversible
Esophageal dyfunction
Fingers become stiff and skin takes a smooth, shiny appearance
May undergo permanent flexure and “claw” deformity
Abnormal deposition of collagen in the dermis is the cause
Sclerodactyly
Similar to those seen in HHT
Bleeding form fuperficial dilated capillaries may occur
Facial skin and vermillion zone of lips commonly affected
Telangiectasias
Anticentromere antibodies
HHT in differential if other signs of CREST not there
Histopathologic findings similar to scleroderma but milder
Prognosis better then scleroderma with 80T
CREST Syndrome
