Chpt 16- dermatology Flashcards by David Lawlor

group of inherited conditions in which two or more ectodermally derived anatomic structures fail to develop

ectodermal dysplasia

How well did you know this?

Not at all

Perfectly

Ectodermal dysplasia can affect what

skin
Hair
Nails
Teeth
Sweat glands

How well did you know this?

Not at all

Perfectly

Skin and hair problems associated with Ectodermal dysplasia

sparse hair, eyebrows (blond)
Periocular hyperpigmentation (dark circles around eyes)
Dystrophic nails

How well did you know this?

Not at all

Perfectly

Why would an ectodermal dysplasia patient have xerostomia

decreased salivary gland development

How well did you know this?

Not at all

Perfectly

Tooth problems with ectodermal dysplasia

hypodontia

Cone shaped teeth

How well did you know this?

Not at all

Perfectly

3 treatments for Ectodermal dysplasia

genetic counseling (w/ Dr. Elsea)
Prosthetic teeth
Do a pedigree and a microarray

How well did you know this?

Not at all

Perfectly

Autosomal dominant inherited skin disorder manifesting as thick white buccal mucosa bilaterally, may be corrugated or velvety

White sponge nevus

How well did you know this?

Not at all

Perfectly

Is White Sponge nevus malignant and is there treatment

benign, no treatment

How well did you know this?

Not at all

Perfectly

Autosomal dominant condition seen in descendants of North Carolina Indians, with thick white plaques on the buccal mucosa(like white sponge nevus) and the conjunctiva

Hereditary Benign Intraepithelial Dyskeratosis

How well did you know this?

Not at all

Perfectly

Histology Dr Siversky said to know for Hereditary Benign Intraepithelial Dyskeratosis

Epithelial clefting

How well did you know this?

Not at all

Perfectly

Autosomal dominant skin problem with erythematous puritic papules on the trunk and scalp with foul odor, dystrophic nails (ridged and split)

Keratosis follicularis (Darier’s disease)

How well did you know this?

Not at all

Perfectly

What is the oral manifestations of Keratosis Follicularis/ Darier’s Disease

multiple white papules

How well did you know this?

Not at all

Perfectly

How treat Keratosis Follicularis/ Darier’s Disease

Keratolytic agents or emollients

How well did you know this?

Not at all

Perfectly

Histopathologically identical to Darier’s Disease, but manifests orally as a single white papule

Warty dyskeratosis

How well did you know this?

Not at all

Perfectly

Where does the single white papule of Warty dsykeratoma appear

on hard palate or alveolar ridge

How well did you know this?

Not at all

Perfectly

Autosomal dominant with freckle like lesions in and around oral cavity, on the hands, and having intestinal polyps with a predisposition to go to adenocarcinoma

Peutz-Jeghers

How well did you know this?

Not at all

Perfectly

What is the difference, as far as the intestinal polyps, between Peutz-Jeghers and Gardners Syndrome

Peutz-Jeghers has benign intestinal polyps while Garderns are going to go malignant

How well did you know this?

Not at all

Perfectly

Treatment for Peutz-Jeghers

Genetic counseling
Monitor for intussusceptions(one part of intestine invaginates into another section, like a telescope [Wikipedia] or malignant transformation

How well did you know this?

Not at all

Perfectly

Autosomal dominant conditions of multiple vascular hamartomas due to decreased blood vessel wall intregrity. Pt can have frequent epistaxis (nose bleeds) . Telangiectasias can be intraoral, hands, feet, GI tract, GU tract and eye

Hereditary Hemorrhagic telangiectasia

How well did you know this?

Not at all

Perfectly

With any bleeding disorder, even Hereditary Hemoorhagic Telangiectasia, what should always be considered as another problem that might follow

Iron deficiency anemia

How well did you know this?

Not at all

Perfectly

Genetic abnormalities that lead to abnormal collagen manifesting as hypermobile joints, elasticity of skin (carnival person)

Ehlers-Danlos

How well did you know this?

Not at all

Perfectly

Oral concerns with Ehlers-Danlos

subluxation of TMJ

Don’t respond well to surgery due to defective collagen Bruise and bleed easily

How well did you know this?

Not at all

Perfectly

Chronic inflammatory disease causing white striations, papules or plaques on buccal mucosa, tongue and gingival, thought to be autoimmune related by CD8 T cells causing oral epithelial cells apoptosis

Lichen Planus

How well did you know this?

Not at all

Perfectly

Demographics for Lichen planus

45-60 y.o (rare in children) Women 2x more than men

How well did you know this?

Not at all

Perfectly

Is Lichen planus only caused by autoimmune response

No, there can be drugs whose side effects cause a Lichenoid drug reaction, however, that goes away when drugs are stopped. True Lichen planus is chronic

If you see this phrase, you should think of Lichen Planus

Striae of Wickham

What are the skin manifestations of Lichen planus

purple puritic polygonal papules

When biopsying Lichen planus, do you biopsy the red or the white area

White area, red will just show ulceration (basement membrane with no epithelial covering)

Histology for Lichen Planus

saw tooth rete pegs and loose basal cell layer

Which phase of Lichen planus is treated: Reticular or erosive, and with what

Erosive. Corticosteroids because it is autoimmune mediated

What can superimpose on top of Lichen planus especially during corticosteroid treatment of the Erosive form

candidiasis

Dislodgement of skin by lateral pressure, can be a symptom in multiple diseases

Nikolsky sign

2 disease we have been taught that have a positive Nikolsky sign

Pemphigus | Pemphigoid

Histologically, which has a change in the basement membrane: Pemphigoid or pemphigous

Pemphigoid (goid goes down to the basement)

Histologically, which has intraepithelial separation: Pemphigoid or pemphigous

Pemphigus

Which has larger lesions Pemphigoid or pemphigous and why

pemphigoid, because they are thicker (all the way to the basement)

Pemphigoid type that affects women over the age of 60 manifesting orally as vesicles or bullae

benign mucous membrane pemphigoid

What are the clinical sizes of the benign mucous membrane pemphigoid vesicles and bullae

vesicles are less than 5mm | Bullae are greater than 5 mm

What is the histology for benign mucous membrane pemphigoid (Siversky says to be able to ID this for the final

subepithelial clefting | Immunofluorescence at basement membrane

Most commonly used antibiotics for treating benign mucous membrane pemphigoid

Tetracycline Niacinamide Nicotinamide

If going to treat someone with benign mucous membrane pemphigoid with Dapsone, what must you check first

that they are not Glucose-6-Phosphate dehydrogenase deficient. Can cause aplastic anemia

Most common of the autoimmune blistering conditions

bullous pemphigoid

mucocutaneous autoimmune disease that has intrapepithelial acantholysis resulting in intraepithelial clefting . If untreated is fatal

pemphigus vulgaris

What is usually the first sign of Pemphigus Vulgaris, but also the hardest to treat

oral lesion, “first to show, last to go”

Pemphigus Vulgaris intraepithelial clefting has what in the middle

Tzank cells (floating cells in the intraepithelial cleft)

Where does the Immunofluorescence show up in Pemphigus vulgaris versus pemphigoid

Pemphigus vulgaris at immunofluoresces at the spinous layer while pemphigoid does so at the basement membrane

What is a major distinction demographically between pemphigus and pemphigoid

pemphigous affects M=F Pemphigoid prefers males Pemphigous has Jewish predilection

Pemphigus should be managed by what medical specialty

rheumatologist

How is pemphigus diagnosed

incisional biopsy

How is pemphigus treated

jointly with dermatologist with Steroids

What is patient outcome if not treated with steroids for pemphigus

60-80% die without steroid treatment

The intraepithelial separation just above the basement membrane in Pemphigus casues what look histologically

row of tombstones (the basal layer sticking up into the intraepithelial cleft)

pemphigus type caused by cross-reacting antibodies in lymphoma or leukemia attacking desmosomal complexes that may precede the discovery of an underlying malignancy

paraneoplastic pemphigus/ neoplasia-induced pemphigus

Occurs in recipients of allogenic bone marrow transplantation. The HLA matched donor hematopoietic stem cells recognize their new host as foreign and begin to attack

Graft Versus Host Disease

only transplant that does not require immunosuppressive drugs for life

successful bone marrow transplant

What factors can make Graft versus host disease milder

young pt Good histocompatability match Cord blood Female

Graft versus host disease seen within a few weeks of transplant and can manifest as a mild rash to severe sloughing that resembles Toxic epidermal necrolysis

Acute Graft Versus Host Disease

can be seen 100 days to years after transplant. Considered a continuation of acute graft versus host disease. Will mimic an autoimmune disease

Chronic Graft Versus Host Disease

Chronic Graft Versus Host disease will resemble what clinically

Lichen planus or systemic sclerosis

Which manifests more orally, Chronic or Acute Graft versus host disease

Chronic (80%)

Oral graft versus host disease will show ulcerations within the first two weeks post bone marrow transplant why

caused by chemotherapeutic conditioning and the neutropenic state

If the ulcers last longer than 2 weeks beyond the 2 week post bone marrow transplant buffer zone have what

Acute Graft Versus Host Disease

beyond ulcers, what are 2 other oral manifestations of oral Graft Versus Host Disease

Xerostomia due to immunologica destruction of salivary glands Mucocelles on soft palate

Mucoceles, which are seen commonly on lips and buccal mucosa of young males, if seen on the soft palat, one should think what

Graft Versus Host Disease

Primary strategy for dealing with Graft versus host disease

reduce or prevent occurrence with good histocompatability match

What is the prophylaxis for Graft Versus host disease

immunomodulatory + immunosuppressive: prednisone + cyclosporine or tacrolimus. Give corticosteroids for oral lesions and treat xerostomia

Common chronic skin disease characterized by an increased proliferative activity of the cutaneous keratinocytes. Mild disease that can be treated with keratolytic agents, sunlight, Vit D analogues

Psoriasis

Classic example of an immunologically mediated condition (794)

Lupus Erythematosus

Most common of the collagen vascular and connective tissue disease in the US

Lupus Erythematosus

Most immunologic diseases, including Lupus Erythematous, affect which gender predominantly

females

Common findings of systemic lupus erythematosus (794)

``` fever Weight loss Arthritis Fatigue Malaise Characteristic butterfly pattern rash over malar area and nose that spares the nasolabial folds and is exacerbated by sun exposure ```

Most significant aspect of systemic lupus erythematosus (794)

kidney failure

Systemic lupus is caused by increased activity of what part of the immune system

humoral limb (B lymphocytes [stimulated by abnormally functioning T lymphocytes ?])

What is another systemic mortality risk in systemic lupus erythematosus

bacterial endocarditis

How can systemic lupus erythematous manifest orally

look lichenoid on palatal, buccal mucosa, and gingival. Ulcers, pain, erythema, hyperkeratosis

if Lupus manifests on the vermillion zone of the lower lip it is called

Lupus cheilitis

Lupus type with no systemic symptoms, and only lesions on the skin and mucosal surfaces, called discoid lupus erythematous and present on sun-exposed areas of skin.

Chronic cutaneous Lupus erythematosus

What is the character of the oral lesions in Chronic Cutaneous Lupus erythematosus versus erosive lichen planus, since they both look alike orally

Chronic cutaneous Lupus erythematosus oral lesions will not manifest without skin lesions

What is a lab test that is high in Lupus erythematous patients

high in ANA = autonucleic antibody

Most common cause of death in systemic lupus erythematosus

renal failure

Treatments for Lupus if mild

avoid sun NSAIDs Antimalarials (hydroxychloroquine)

Treatment for Lupus if severe

corticosteroids and other immunosuppressives

If see lichen planus, what else can be put on the differential

Lupus erythematous

immunologically mediated deposition of dense collagen in tissues. Also known as Scleroderma

Systemic sclerosis

First sign of Systemic sclerosis, but is not diagnostic for the disease

Raynaud’s phenomenon

Systemic sclerosis higher in which gender

females

What is the cause of death in systemic sclerosis

pulmonary fibrosis, leading to pulmonary hypertension and heart failure

What does pulmonary fibrosis do

reduces lung surface area for oxygen exchange

resorption of terminal phalnages and flexure contractures producing club like fingers in systemic sclerosis and some other diseases

acro-osteolysis

What is the clinical appearance of a person with systemic sclerosis

smooth, taut, mask-like face, nasal alae atrophy = mouse facies

Character of the mouth of pt with systemic sclerosis

microstomia with purse-string furrows radiating from the mouth

Character of the gingival in pt with systemic sclerosis

lose attached ginigva

Why dysphagia in systemic sclerosis

collagen deposits cause hypomobile tongue coupled with inelastic esophagus

Radiographic appearance of person with systemic sclerosis

widened PDL (isn’t that a sign in Paget’s Disease also?)

Does systemic sclerosis with its widened PDL and loss of attached gingival respond to periodontal therapy

Siversky loves this disease and brought up the fact that this disease, radiographically, shows a loss of the lamina dura

hyperparathyroidism

Systemic sclerosis that is localized usually leaving a scar called en coup de sabre, Dr, Siversky mentioned it as Morpha

localized scleraderma

What can the pressure of collagen deposition cause orally and skeletally

resorption of posterior ramus, coronoid process and condyle

What systemic medication is prescribed to try and inhibit collagen production in systemic sclerosis

D-penicillamine

Prescribing calcium channel blockers to increase peripheral blood flow or Angiotensin-converting enzyme inhibitors if the kidney is involved, all have the goal of doing what for systemic sclerosis

slow the disease process

would it be smart to fabricate dentures for a systemic sclerosis patient

No, due to microstoma and inelasticity of mouth

What does CREST stand for

``` Calcinosis cutis Raynaud’s phenomenon Esophageal dysfunction Sclerodactyly Telangiectasias ```

Part of CREST syndrome characterized by movable, non-tender, subcutaneous nodules, .5cm to 2cm in size caused by the deposition of calcium salts

Calcinosis cutis

Part of CREST as well as other syndromes characterized by dramatic blanching of digits (dead white) when exposed to cold or even stress. Digit will turn blue a few minutes later with venous stasis, then a final dusky red hue as hyperemic blood flow returns. May be accompanied by throbbing pain

Raynaud’s phenomenon

Is Raynaud’s phenomenon indicative of scleraderma or CREST

Diagnosed with a barium swallow, this part of CREST is due to abnormal collagen deposition in esophageal submucosa. It is not noticed early in CREST, but will affect swallowing later

Esophageal dysfunction

Part of CREST caused by abnormal deposition of collagen in the dermis leading to fingers becoming stiff, skin becoming shiny, and digits can undergo permanent flexture to a claw-like deformity

Sclerodactyly

Part of CREST due to fragile vessels, and is similar to Hereditary Hemorrhagic Telangiectasia

Telangiectasias

Which has better prognosis, CREST or systemic sclerosis/scleraderma

CREST