Dermatologic Disease Flashcards
What group of inherited conditions has two or more ectodermal derived anatomic structures fail, typically including Skin, hair, nails, teeth, sweat glands?
Ectodermal Dysplasia
Which best known ectodermal dysplasia is x-linked with Male predominance, and is characterized by heat intolerance, fine, sparse hair, periocular wrinkling with hyper pigmentation and fewer, conical shapped teeth?
Hypohidrotic Ectodermal Dysplasia
A genetically determined skin disorder is called a?
Genodermatosis
What AD lesion in keratin appears at birth or early childhood as a symmetrical thick, white, digress plaque on the bilateral buccal mucosa?
White sponge Nevus
*bening condition with no treatment
What rare condition is characterized by Freckle-like lesions of the hands, perioral skin, and oral mucosa (lips especially), Intestinal polyposis
and a predisposition for affected patients to develop cancer?
Peutz-Jeghers Syndrome
*intestinal polyps are not premalignant
Peutz-Jeghers syndrom patients are how many times more likely to have malignancy in their lifetime?
18x
*GI adenocarcinoma or other tumors such as breast cancer
What condition is characterized by CNS manifestations, Angiofibromas of the skin, Ungual (or perungual) fibromas (fingernails), and Characteristic skin lesions?
Tuburous Sclerosis
What are the 3 CNS manifestations of Tuburous Sclerosis?
1-Mental retardation
2-Seizure disorders
3-Potato-like growths (“tubers”)
What are the two characteristic skin lesions of Tuburous Sclerosis?
1-Shagreen Patches (connective tissue hamartomas)
2-Ash-leaf spots (ovoid areas of hypo pigmentation)
What condition has oral lesions that are the first to show but last to go?
Pemphigus Vulgaris
The intraepithelial split that takes place in Pemphigus vulgaris is due to antibodies directed at what?
Desmosomes
A bulla being induced with lateral pressure on normal appearing skin is called what?
Positive Nikolsky sign
*characteristic of pemphigus vulgaris
Localized pemphigus in a small 1 cm or less usually on the palate is called?
Hailey Hailey Disease
AKA Cicatricial pemphigoid, what condition is characterized by an intraoral blood blister and is twice as common as pemphigus?
Mucous Membrane Pemphigoid
What are the two stages of occular involvement in mucous membrane pemphigoid?
1-Symblepharons (Scarring adhesions resulting from inflamed conjunctiva)
2-Entropion (eyelid turns inward)
A subepithelial split is observed in what condition where antibodies attack the basement membrane?
Mucous Membrane Pemphigoid
Which blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis is usually due to a preceding infection or exposure to medication?
Erythema Multiforme
*acute onset with a spectrum of clinical disease. Prodromal symptoms.
When erythema multiforme is caused by infection it is usually _________ And when it is caused by medication it is usually _______
Herpes simplex
Antibiotics
What characteristic erythematous lesions develop in Erythema Multiforme?
Target lesions
*also crusting of the vermilion zone of the lips
Steven Johnson syndrome is usually triggered by a drug, involves skin, oral mucosa and either ocular or genital mucosa and is known as what?
Erythema Multiforme Major
What is the most severe form of Erythema Multiforme?
Toxic Epidermal Necrolysis
AKA geographic tongue or Benign Migratory glossitis, what condition has a serpentine border and can change from day to day?
Erythema Migrans
What condition is characterized by a lace like network of white lines known as wickhams striae?
Lichen Planus
If a lichen planus looking lesion is due to medication or amalgam it is known as?
Lichenoid mucositis
What are the 4 Ps of Skin lesion in lichen planus?
1-Purple
2-Pruritus
3-Polygonal
4-Papules
What are the two forms of lichen planus lesions?
1-Reticular (more common)
2-Erosive (ulcerated)
LIchen planus lesions have saw toothed rate ridges and can be induced by what 4 things?
1-Stress
2-Trauma
3-Advil (any NSAID except acetaminophen)
4-Yeast
What condition occurs in recipients of allogeneic bone marrow transplantations?
Graft-Versus-Host Disease
Of those with acute GVHD, __% have oral involvement,and _____% of chronic do
50, 80
*Most have fine reticular network of striae that look like lichen planus
What classic example of an immunologically mediated condition is the most common collagen vascular or connective tissue disease in the US?
Lupus Erythematous
With a 10:1 female predilection, what are 5 common findings of Systemic Lupus Erythematous?
1-Fever 2-Weight loss 3-Arthritis 4-Fatigue 5-Malar rash (butterfly rash) – 50%
What is the most significant aspect of Systemic Lupus Erythematous?
Kidney complications or failure
Pericarditis is the most common cardiac complication of Systemic Lupus Erythematous, what are the warty vegetations called that affect the heart valves in this condition?
Libman-Sacks Endocarditis
What condition is a disease in which dense collagen is deposited in the tissues of the body in extraordinary amounts?
Systemic Sclerosis
A vasoconstrictive event triggered by emotional distress or exposure to cold is called?
Raynaud’s Phenomenon
How does the diffuse, hard texture and smooth surface of Systemic Sclerosis patient’s skin affect their oral cavity?
Results in microstomia making the lips hard to open and appearing to be puckered
A mild variant of Systemic Sclerosis called localized scleroderma has what lesion?
Scar named en coup de saber (“strike of the sword”)
What 5 things does CREST syndrome have?
C – Calcinosis cutis R – Raynaud’s phenomenon E – Esophageal dysfunction S – Sclerodactyly T – Telangiectasia
*mostly women in their 60s
The multiple, movable, nontender, subcutaneous, nodular calcifications in Crest disease are called?
Calcinosis Cutis
The abnormal deposition of esophageal submucosa results in what?
Esophageal Dysfunction
Stiff fingers that undergo permanent flexure resembling a claw are called?
Sclerodactyly
Numerous red papillose that are 1-2 mm in size, most frequently on the vermillion zone that blanch are called?
Telangiectasias
What condition is characterized by the development of a velvety, brownish alteration of the skin which can be a sign of internal cancer?
Acanthosis Nigricans
*most are benign
What are 5 possible causes of Benign Acanthosis Nigricans?
1-Obesity (termed pseudoacanthosis nigricans)
2-Inheritance
3-Various endocrinopathies (like diabetes mellitus)
4-Various syndromes (like Crouzon)
5-Various drugs (like oral contraceptives)
