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Habif 16: Vesicular and bullous disease > Blisters > Flashcards

Blisters Flashcards

1
Q

What are the major bullous diseases

A

Pemphigus foliaceous: all ages, red/crusting, few fragile blisters, can be associated with SLE (pemphigus erythematosus), can be drug induced

Pemphigus: elderly, oral lesions, esophageal erosions, flaccid blisters, non-itchy, + Nikolsky’s, paraneoplastic variant

Cicatricial pemphigoid: elderly, itch, oral and eye involvement, few tense blisters on face and neck, heals with scar

Pemphigoid: elderly, itchy, uritcarial plaques, tense blisters, palms and soles

Dermatitis herpetiformis: middle age, very itchy, gluten-sensitive enteropathy, small bowel lymphoma

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2
Q

What diseases target the granular layer

A

Bullous ichthyosiform erythroderma

pemphigus foliaceus and erythematosus

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3
Q

What diseases target the upper and mid spinous layer

A 
Dermatophyte infxn
Dyshidrosis
eczematous blister
friction blister
insect bite and scabies
miliaria rubra
viral blisters
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4
Q

What diseases target the basal layer

A 
Erythema multiforme (epidermal type)
Epidermolysis bullosa simplex
fixed drug eruption
kerosene necrosis
lichen planus
TEN
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5
Q

What diseases target the lamina lucida layer

A 
BP
Cicatricial pemphigoid
DH
Epidermolysis bullosa acquisita/letalis
herpes gestationis
suction blister
thermal lesions
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6
Q

What diseases target the basal lamina and sublaminar connective tissue layer

A 
Bullous dermatosis of hemodialysis
bullous eruption of SLE
Epidermolysis bullosa dystrophica
Erythema multiforme (dermal type)
Ischemic bullae (drug overdose)
Lichen sclerosus et atrophicus
porphyria cutanea tarda
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7
Q

What diseases target the subcorneal layer

A 
Candida
impetigo
miliaria crystallina 
SSSS
subcorneal pustular dermatosis
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8
Q

What diseases target the lower spinous layer

A

Benign familial chronic pemphigus
keratosis follicularis
pemphigus vulgaris
transient acantholytic dermatosis

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9
Q

Target molecule in Pemphigus vulgaris

A

Desmoglein 3 and 1

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10
Q

Target molecule in Pemphigus foliaceous

A

Desmoglein 1

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11
Q

Target molecule in Paraneoplastic pemphigus

A

Desmoglein 3, desmoplakin 1 and 2, BP230, envoplakin, periplakin, and others

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12
Q

Target molecule in IgA Pemphigus

A

Desmocollin 1

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13
Q

Target molecule in BP

A

BP180, 230 (hemidesmosome and lamina lucida)

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14
Q

Target molecule in Herpes gestationis

A

BP180, 230 (hemidesmosome and lamina lucida)

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15
Q

Target molecule in Cicatricial pemphigoid

A

BP180, laminin V (hemidesmosome and lamina lucida)

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16
Q

Target molecule in Epidermolysis bullosa acquisita

A

Type VII collagen (anchoring fibrils)

17
Q

Target molecule in Linear IgA

A

LAD antigen (BP180) (hemidesmosome and lamina lucida)

18
Q

Target molecule in Bullous SLE

A

Type VII collagen (anchoring fibrils)

19
Q

Target molecule in dermatitis herpetiformis

A

unknown

20
Q

Biopsy work up for blisitering disorders

A

Take one from the edge of a fresh lesion and one from normal, non-edematous skin

21
Q

What disease process would you want to take a biopsy of the actual lesion (not edge) to perform DIF on?

A

HSP and vasculitis lesions

22
Q

Dermatitis herpetiformis clinical presentation

A

usually in 2nd-5th decade
usually in caucasian patients
intensely burning urticarial papules, vesicles symmetrically distributed across extensor surfaces, scalp, and buttocks

23
Q

DDx of DH

A

bullous erythema multiforme and bullous pemphigoid

24
Q

Associated diseases in DH

A

Celiac disease

thyroid dysfunction

25
Q

Linear IgA bullous dermatosis clinical presentation

A

May look like DH, BP, or cicatricial pemhigoid

26
Q

Etiology of Linear IgA bullous dermatosis

A

Medications such as vancomycin (within 1-15 days after first dose)

27
Q

Histology of DH

A

IgA deposits in upper dermis

- also see antibodies against epidermal transglutaminase 1 on Indirect immunoflorescence/ELISA

28
Q

Histology for Linear IgA

A

IgA linear across basement membrane zone

29
Q

How many DH patients have the gluten sensitive enteropathy

A

majority (there is a direct correlation between IgA anti-endomysial antibodies and the severity of gluten induced jejunal damage)

30
Q

Does dapsone fix the enteropathy

A

No, just the dermatitis herpetiformis. People still need to be on gluten free diet to avoid MALT lymphoma formation

31
Q

Diagnosis of DH

A
  • Subepidermal cleft with neutrophils and few eos at tips of dermal papillae
  • IgA on DIF in granular deposits on tips of dermal papillae and along BMZ (not altered if pt on dapsone but gone if gluten free)
  • anti-endomysial IgA or anti-transglutaminase IgA testing on serum
  • also screen with CBC, TSH, and blood glucose
32
Q

Diagnosis of linear IgA

A

just have the IgA at BMZ not dermal papillae tips

33
Q

Treatment of DH

A

Dapsone and sulfapyradine/sulfasalazine (2nd line)

- reported treatment with tetracycline and nicotinamide combined

34
Q

Monitoring while on Dapsone

A
  • first check G6PD level

- then weekly CBC x 1 mo, monthly x 6mo, then two times per year

35
Q

Adverse reactions to Dapsone

A

Agranulocytosis, aplastic anemia, peripheral motor neuropathy (resolves over months to years once discontinued)

36
Q

Dapsone hypersensitivity syndrome

A
  • occurs 1 mo or more after starting, Mono like illness + exanthematous rash +/- hepatitis and hypothyroidism
  • treat with atleast 1 mo of prednisone while monitoring organ dysfunction via labs

Habif 16: Vesicular and bullous disease (8 decks)

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