Chpt 16- dermatology Flashcards
group of inherited conditions in which two or more ectodermally derived anatomic structures fail to develop
ectodermal dysplasia
Ectodermal dysplasia can affect what
skin Hair Nails Teeth Sweat glands
Skin and hair problems associated with Ectodermal dysplasia
sparse hair, eyebrows (blond)
Periocular hyperpigmentation (dark circles around eyes)
Dystrophic nails
Why would an ectodermal dysplasia patient have xerostomia
decreased salivary gland development
Tooth problems with ectodermal dysplasia
hypodontia
Cone shaped teeth
3 treatments for Ectodermal dysplasia
genetic counseling (w/ Dr. Elsea)
Prosthetic teeth
Do a pedigree and a microarray
Autosomal dominant inherited skin disorder manifesting as thick white buccal mucosa bilaterally, may be corrugated or velvety
White sponge nevus
Is White Sponge nevus malignant and is there treatment
benign, no treatment
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
Histology Dr Siversky said to know for Hereditary Benign Intraepithelial Dyskeratosis
Epithelial clefting
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)
What is the oral manifestations of Keratosis Follicularis/ Darier’s Disease
multiple white papules
How treat Keratosis Follicularis/ Darier’s Disease
Keratolytic agents or emollients
Histopathologically identical to Darier’s Disease, but manifests orally as a single white papule
Warty dyskeratosis
Where does the single white papule of Warty dsykeratoma appear
on hard palate or alveolar ridge
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
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
Treatment for Peutz-Jeghers
Genetic counseling
Monitor for intussusceptions(one part of intestine invaginates into another section, like a telescope [Wikipedia] or malignant transformation
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
With any bleeding disorder, even Hereditary Hemoorhagic Telangiectasia, what should always be considered as another problem that might follow
Iron deficiency anemia
Genetic abnormalities that lead to abnormal collagen manifesting as hypermobile joints, elasticity of skin (carnival person)
Ehlers-Danlos
Oral concerns with Ehlers-Danlos
subluxation of TMJ
Don’t respond well to surgery due to defective collagen Bruise and bleed easily
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
Demographics for Lichen planus
45-60 y.o (rare in children) Women 2x more than men
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
NO
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
No
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
