Chapter 16: Dermatologic Flashcards by Chris King

Autosomal Dominant
Thick white buccal mucosa bilaterally
Benign hyperparakeratotic
No treatment

White sponge nevus

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Autosomal dominant
NC Lumbi Indians
Thick white plaques on buccal mucosa
Dyskeratosis
Benign, no Tx

Hereditary Benign Intrepithelial Dyskeratosis

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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

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Identical histology to Darier’s disease

Orally: Single white papule on hard palate or alveolar ridge

Tx: excision

Warty Dykeratoma

Isolated Darier’s Disesase

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Autosomal dominant
Freckle like lesions in and around oral cavity and hands

Oral oncogenes

Intestinal polyposis that can change to adenocarcinoma

Peutz-jeghers syndrome

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Autosomal dominant
Vascular hamartomas 
Frequent epistaxis
Telangiectasias: intraoral, hands, feet, GI, GU, eye
Iron deficiency including anemia

Hereditary Hemorrhagic Telangiectasia

Osler-Weber-Rendu Syndrome

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Abnormal collagen production due to genetic abnormalities

Hypermobility of joints and elasticity of skin

Ehlers-danlos syndrome

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Number of closely related lichenoid reactions that reflect immunologic recognition of a variety of stimuli

Lichen planus

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Purple
Polygonal
Papular
Pruritic

Skin Lichen Planus

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Educate patients
Photograph
Erosive form see four times a year
Biopsy
Reassure

How to monitor Lichen Planus in patients

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First used in 1895 to diagnose pemphigus
Dislodgement of skin by lateral pressure
Multiple Diseases

Nikolsky sign

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Average age 60
Women 2:1
Oral Lesions: Conjunctival, nasal, esophageal, laryngeal, vaginal
Clinical vesicles and bullae

Benign mucus membrane pemphigoid

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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

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Dapsone (CI: G6PD deficiency)
Steroids
Tetracycline & Niacinamide
Immunosuppresive agents

BMMP Tx

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Mucocutaneous AI disease characterized by acantholysis due to immune complex deposition at cellular attachment bridges
Intraepithelial clefting

Pemphigus Vulgaris

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Adults age 50
Rare in kids
Males = Females
Almost all cases have oral involvement

Clinical signs of Pemphigus Vulgaris

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Intra-epithelial separation

Immunofluorescence testing shows autoantibodies to the spinous layer of skin

Pemphigus Vulgaris

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Jewish predilection
Ragged erosions and ulcers of ANY oral mucosa
Bullae rupture early and are rarely seen by DDS
(+) Nikolsky sign

Pemphigus vulgaris

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Oral lesions precede skin lesions
Large, ragged ulcerations
Marginal gingiva erosions early
1-5 Million /year

Pemphigus vulgaris

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Oral lesions may be the first to present in the potentially fatal disease

Blistering due to autoimmune attack of desmosomes

Pemphigus vulgaris

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The oral lesions are the first to show and the last to go!

Pemphigus vulgaris

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Incisional biopsy ASAP
Steroids from dentist and dermatologis
60-80% die w/o steroids
5-10% dies with steroids

Pemphigus vulgaris

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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

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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

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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