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Dermatology 3B - Lecture 9 and 10
N/A
60
Science
Undergraduate 4
05/31/2015

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Cards

Term
What structures keep the basal layer of keratinocytes glued to the basement membrane?
Definition
hemi-desomosomes
Term
What interlocking structure glues keratinocytes together?
Definition
Desmosomes
Term
What causes blisters?
Definition
1.Damage to structures holding together the keratinocytes at any level in the epidermis
2.Damage to structures holding the basal cells to the basement membrane

 

3.Damage to structures holding the epidermis and dermis together
Term
What results if the roof of a blister is thin?
Definition
it can come off easily and become an erosion, whereas thick blisters remain intact (esp if they are smal)
Term
What does blood stained fluid in a blister suggest?
Definition
That it is deeper
Term
What is pemphigus?
Definition
- rare group of autoimmune blistering diseases, desmoglein is attacked causing acantholysis
Term
4 types of pemphigus?
Definition

- pemphigus vulgaris

-pemphigus foliaceous

-pemphigus vegetans

-paraneoplastic pemphigus

Term
Where does pemphigus affect and on who?
Definition

- mouth, genitals, eyes, nose, oral and respiratory pathways

- 40-60 is common

may be nail disease

Term
Main differences between each type of pemphigus?
Definition

- P foliaceous and vegetans have more superficial blisters and present as crusted lesions. They do not usually involve the mucosa

-foliaceous can mimic eczematous eruption

-vulgaris has deeper blisters and presents as erosions or flaccid blisters

- neoplastic form is rare and severe, associated with underlying malignancy

Term
Diagnosis of pemphigus?
Definition

- skin biopsy for HandE and DIF

-Blood tests for circulating antibodies

Term
Tx of pemphigus?
Definition

- high dose steroids

-immunosuppressive agents: azathioprine, cyclophosphamide, methotrexate, rituximab

Term
Which is more common; pemphigoid or pemphigus?
Definition
pemphigoid
Term
What causes bullous pemphigoid?
Definition
immune reaction under the skin
Term
Bullous pemphigoid appearance?
Definition

- crops of tense, fluid-filled, itchy bullae- may be filled with clear, cloudy or blood-stained fluid.

- nonspecific red, itchy rash may be present for several weeks/months before blisters. These may resolve temporarily or persist, with or w/o tx. 

- may be localized to one area, or widespread on the trunk and proximal limbs. Frequently it affects skin around skin folds. 

- When blisters heal, they leave brown marks and/or tiny cysts called milia but these usually disappear within a few months.

Term
Pemphigoid affects mainly which age group?
Definition
60 yrs or older
Term
Diagnosis of bullous pemphigoid?
Definition

- biopsy of skin histolgy and DIF

- serology IIF ab in blood or blister fluid

Term
Tx of bullous pemphigoid?
Definition

- potent topical steroids

-oral steroids

immunosuppressive agents: methotrexate, azathioprine, mycophenolate

Term
Appearance of dermatitis herpetiformis?
Definition
- grouped vesicles/papules with itching that arise on normal or reddened skin, scratching from itching leads to erosions and crusts. Pigmentation may result on heaing. They may initially present as digital petechiae.
Term
Onset age and distribution of dermatitis herpetiformis?
Definition

- early adult life

- symmetrical, elbows, knees, buttocks, palms and soles

Term
Associations with dermatitis herpitiformis?
Definition

gluten sensitive enteropathy and other autoimmune diseases like

thyroid disease, diabetes

Term
Diagnosis of dermatitis herpetiformis?
Definition
Skin Biopsy for Histology ( H&E) and DIF
• IIF- serology not reliable
• Testing for Gluten sensitive enteropathy

 

• Testing for other auto immune associations
Term
Tx of dermatitis herpetiformis?
Definition
Dietary changes ( Gluten free diet) – mainstay, otherwise risk of gut Lymphoma

 

•Anti- neutrophilic medications e.g Dapsone, Sulphonamides, Colchicine
Term
What is epidermolysis bullosa?
Definition

inherited connective tissue disease caused by mutation in keratin or collagen gene

protein anchors fail between dermis and epidermis creating friction leading to blisters and painful sores

Term
Types of epidermolysis bullosa and their features?
Definition

Simplex - Can be hands and feet, or all over, good prognosis 

Dystrophic - contraction of joints and mouth membranes, narrowing esophagus, fusion of fingers and toes, possibility of skin cancers

Junctional - extensive blistering inside and outside body, many die by 2 due to malnutrition because of blistering to eosophagus and trachea  

Term

Can epidermolysis affect nails?

Which joints does it usually affect on the feet?

Definition

Yes

IPJ joints

Term
Tx of epidermolysis bullosa?
Definition
  • Maintain cool environment and avoid overheating
  • Use foam padding or sheepskins to help reduce friction on furniture such as beds, chairs and infant car seats
  • Wear clothing made of soft non-irritating fabrics
  • Pierce, drain and dress blisters to promote healing 
  • Try to avoid using nappies in infants with severe EB, instead place child on a clean pad
Term
What is epidermolysis bullosa acquisita?
Definition
acquired form of mechanobullous disorder, usually affects adults. Tend to be on areas that are prone to injury like fingers, knees, elbows
Term
Does pemphigus have flaccid or tense blisters?
Definition
flaccid
Term
Main investigations for immune bullous disorders?
Definition
Biopsy  4mm Punch Biopsy x 2

Lesional for histopathology

-Perilesional / normal skin – Direct Immuno Fluorescence(DIF)
Indirect Immuno Fluorescence (IIF) - blood /urine/ blister

 

Swabs  Microscopy/Culture/Sensitivity  bact., viral, fungal
Term
General treatments for immune bullous disorders?
Definition
GENERAL
Examine , evaluate, educate, explain (complications, prognosis, A/E)
Identify and remove / Rx triggers

  (drugs, gluten sensitivity, IgA disease)

Admit if required*
Care of mucous membranes

 

Pain relief
Term
What are specific treatments for immune bullous disorders?
Definition
Corticosteroids
Antiseptics
Antibiotics
Dressings
- need to inhibit disease activity, maintain doses until complete clearance and then maintain meds at lowest dose

 


Term
Onset and clinical features of epidermolytic PPK (vorner disease)?
Definition

genetic, presents age 1-2 yrs

diffuse HK over palms and soles, red border

aberrant keratotic lesions on dorsum area

nails be thickened

blistering in areas of trauma, improves with age

Term
Main 3 causes of mechanical blisters?
Definition

- friction

-thermal injury

-chemicals

Term
Other types of blisters besides mechanical and bullous disorders?
Definition

- diabetic acral bulla

-coma

-renal failure, porphyrias

-acute dermatitis

-impetigo

Term
What is the spread sign?
Definition

lateral pressure spreads bulla into surrounding healthy skin

This sign is positive in all varieties of pemphigus and many cases of subepidermal blisters, including bullous pemphigoid, dermatitis herpetiformis, epidermolysis bullosa acquisita, cicatricial pemphigoid, dystrophic epidermolysis bullosa,

Term
What is nikolskys sign?
Definition

- lateral pressure over bony prominence, shearing force dislodges upper epidermis layers.

- can be done near lesion (marginal) or distal (direct)

- positive direct means severe activity of pemphigus vulgaris 

Nikolskiy sign may be elicited over the normal looking skin of a pemphigus patient either close to existing lesions (marginal Nikolskiy sign) or over normal looking skin at a distant site (direct Nikolskiy sign) a positive distant sign indicates active disease in pemphigus

Term

[image]

Cause of this blistering?

Definition
diabetic acral bulla
Term

[image]

Cause of this blistering?

Definition
acute dermatitis
Term

[image]

Cause of this blistering?

Definition
Bullous pemphigoid
Term
[image]
Definition
Epidermolysis bullosa
Term

[image]

What is causing this blisterning at the IPJ joints?

Definition
epidermolysis bullosa
Term
[image]
Definition
Epidermolysis bullosa acquisita
Term

[image]

 

Definition
Epidermolytic palmoplantar keratosis (vorner disease)
Term
[image]
Definition
Pemphigus vegetans
Term
[image]
Definition
Pemphigus vulgaris
Term
[image]
Definition
Pemphigus vulgaris
Term
[image]
Definition
Pemphigus vulgaris
Term
[image]
Definition
Thermal blister
Term
[image]
Definition
Pemphigus vulgaris
Term
Differences between pemphigus vulgaris and bullous pemphigoid
Definition
[image]
Term
What is porokeratosis? and appearance?
Definition

- disorder of keratinisation

- one or more atrophic patches surrounded by Hk ridgelike border called cornoid lamella

Term
5 variations of porokeratosis; sites, onset, characteristics?
Definition
[image]
Term
What is the main risk of porokeratosis?
Definition
malignant transformation
Term
is treatment successful for porokeratosis?
Definition
not usually
Term
which type of porokeratosis requires sun protection?
Definition
Disseminated superficial actinic
Term
What is required to confirm porokeratosis?
Definition
biopsy
Term
Granuloma annulare; appearance, distribution, prevelance
Definition

smooth discoloured, annular lesions that are asymptomatic

limbs and trunk

females mainly, all ages

Term
3 main types of granuloma annulare?
Definition
localised, generalised, perforating
Term
Tx of granuloma annulare?
Definition

self resolving

topical - steroids, TCNI

Intralesional steroids

phototherapy

systemci retinoids, steroids, hydroxychloroquine

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