Chapter 16 Flashcards
What is the inherited condition in which two or more ectodermal derived structures fail to develop?
Ectodermal Dysplasia.
What is the best known form of ectodermal dysplasia and how does it present?
Hypohidrotic: X-linked male predominance, heat intolerance, fine sparse hair, periocular wrinkling with hyperpigmentation, brittle nails, absent salivary glands, missing teeth.
What is the likely dx?
White sponge nevus.
What is the defect seen in white sponge nevus?
Defect in keratins 4 and 13.
What is the likely Dx?
Warty dyskeratoma.
What is the likely Dx?
Peutz-Jeghers Syndrome.
What is the likely Dx?
Peutz-Jeghers syndrome.
What is a system complex characterized by mental retardation, seizure disorders, potato-like growths called tubers, and angiofibromas of the skin?
Tuberous sclerosis.
What are the characteristic skin lesions seen in tuberous sclerosis?
Shagreen patches—> Connective tissue hamartoma.
Ash-leaf spots—> Reverse pigment of cafe au let.
Facial Angiofibromas—> Multiple, smooth-surfaced papules occuring primarily on the nasolabial flod area.
What is the likely Dx?
Tuberous sclerosis.
What attachement disfunction is seen in pemphigus vs pemphigoid?
Pemphigus destroys desmosomes while pemphigoid destroys hemidesmosomes.
What are the 4 diseases of pemphigus?
Vulglaris, vegetans, erythematosus, and foliaceus.
What is the first sign of a pemphigus disease?
Oral lesions. “First to show and last to go.”
What affect does pemphigus have on the epithelium?
There is an intraepithelial split due to the loss of desmosomes.
What is a positive Nikolsky sign and what disease does it present in?
It is when a bulla can be induced on normal-appearing skin when firm lateral pressure is placed on the skin and pressure is exerted.
It is seen in pemphigus.
What term does acantholysis refer to and what disease is it seen in?
When cells of the epithelium “fall apart.”
Seen in pemphigus.
What disease will present with Tzank cells?
Pemphigus and herpes.
What is the purpose of direct and indirect immunoflourescence in pemphigus?
Direct will be positive for C3 and IgG within the epithelium while Indirect correlates with disease activity.
What is the likely Dx?
Pemphigus.
What is the likely Dx?
Pemphigus.
What is pathognomonic of mucous membrane pemphigoid?
An intraoral blood blister.
What is the most significant complication of mucous membrane pemphigoid?
Ocular involvement.
What are the adhesions that form in mucous membrane pemphigoid and what are their significance?
Symblepharons which can cause scarring of the eyelid leading to entropion (inward turning).
What is the likely Dx?
Symblepharon of mucous membrane pemphigoid.
What is the term for a blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis?
Erythema multiforme.
What are the likely infections to cause erythema multiforme?
Herpes simplex and Mycoplasma pneumoniae.
What is another name for erythema mulitforme major?
What are the characteristics of it?
Steven-Johnson syndrome.
Usually triggered by a drug, for this Dx there must be skin, oral mucosa, and either ocular or genital mucosa involvement.
What is the likely Dx?
Erythema Multiforme.
What is the term for a common, chronic dermatologic disease that can affect the oral mucosa?
Lichen Planus.
How are the skin lesions of lichen planus described?
purple, pruritus, polygonal, and papules.
What are the names of the thin, lacelike network of white lines seen in lichen planus>
Wickham’s striae
What are the two forms of oral lesions in lichen planus and describe them.
Reticular: Much more common and asymptomatic.
Erosive: symptomatic
What are some of the things that can induce Lichen Planus?
Stress
Trauma
Advil
Yeast
What is the likely Dx?
Lichen Planus.
What is the likely Dx?
Lichen Planus.
What is the most common collagen, vascular, or connective tissue disease in the US?
Lupus erythematous.
What are some of the common findings with systemic lupus erythmetous?
Fever, weight loss, arthritis, fatigue, malar rash.
What is the characteristic rash of systemic lupus erythematous?
Malar rash: butterfly rash that develops in the malar area.
What is the most signifcant factor of systemic lupus erythematous?
The complication may lead to kidney failure.
What is the disease in which dense collagen is deposited in the tissues of the body in extraordinary amounts?
systemic sclerosis.
What is the first sign of systemic sclerosis?
Raynaud’s phenomenon: vasoconstrictive event triggered by emotional stress or exposure to cold.
What is the mild variant of systemic sclerosis and describe it.
Termed localized scleroderma.
The lesions look like scars described as en coup de sabre.
What is the likely Dx?
En coup de sabre from localized scleroderma.
What are the characteristics of CREST syndrome?
Calcinosis cutis, Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly, Telandiectasia.
What is raynaud’s phenomenon?
What disease is it seen in?
When the extremeties are exposed to cold temps leading to blanching due to vasospasm and then turning blue due to venous stasis.
CREST syndrome and systemic sclerosis.
What is telangiectasia and what disease is it associated with?
It is the numerous red papules from blood being trapped in capillaries close to the mucosal surface. It is seen in CREST syndrome.
What is the likely Dx?
Telangiectasia of CREST syndrome
