Terms Definitions
What is the most common autoimmune blistering disorder?
Bullous Pemphigoid
What is the split for Pemphigoid d/o vs Pemphigus?
Pemphigoid - impaired epidermis to BM connection
Pemphigus - impaired cell to cell adhesion
Diseases that affect the Spinous Layer (upper and mid epidermis)
Dermatophyte fungus infection
Eczematous blister
Friction blister
Insect bites and scabies
Miliaria rubra
Viral blisters (HSV, VZV)
Diseases that affect the Granular cell layer (aka subcorneal)
Bullous ichthyosiform erythroderma
Pemphigus foliaceus
Pemphigus erythematosus
Diseases that affect the Subcorneal
Candida albicans infection
Miliaria crystallina
Staph Scalded Skin Syndrome
Subcorneal pustular dermatosis
Diseases that affect the Basal Cell Area
Erythema multiforme (epidermal type)
Epidermolysis bullosa simplex
Fixed drug eruption
Kerosene necrosis
Lichen Planus
Diseases that affect the Spinous layer (lower epidermis and suprabasal area)
Benign Familial Chronic Pemphigus
Keratosis follicularis
Pemphigus vulgaris
Transient acantholytic dermatosis
Diseases that affect the Lamina Lucida
Bullous Pemphigoid
Cicatrical Pemphigoid
Dermatitis herpetiformis
Epidermolysis bullosa acquista
Epidermolysis bullosa letalis
Herpes gestationis
Suction blister
Thermal lesions (LN2, burns)
Diseases that affect the Basal Lamina and sublaminar connective tissue
Bullous dermatosis of hemodialysis
Bullous eruption of SLE
Epidermolysis bullosa dystrophica
Erythema multiforme (dermal type)
Ischemic bullae (drug overdoses)
Lichen sclerosus et atrophicus
Porphyria cutanea tarda
What is the role of the hemidesmisome in the epidermal basement membrane?
Binds the keratin intermediate filaments to the anchoring filaments that attach the epidermal cells to the lamina densa (basement membrane).
Target of autoantigens in Pemphigus Foliaceus?
Desmoglein 1
Target of Ab in Pemphigus vulgaris
Desmoglein 3 - in mucosal dominant PV
Desmoglein 1 & 3 in mucocutaneous PV
Target of Ab in Paraneoplastic pemphigus
1) Desmoglein 1
2) Desmoglein 3
3) desmoplakin 2 (desmosomal plaque protein)
4) BP230
What type of collage forms the anchoring fibrils in the lamina lucida ?
Collagen type VII
In what diseases is the target BP 180?
herpes gestationis
cictricial pemphigoid
linear IgA disease
What dz targets the laminin 5?
cictricial pemphigoid
What dz targets Type VII collagen?
epidermolysis bullosa acquista
What protein is targeted in IgA pemphigus?
Subcorneal Pustular Dermatosis variant: Desmocollin 1
Intra-Epidermal Neutrophilic (IEN) variant: Desmoglein 1 or 3
Target of Bullous pemphigoid
BP 180, BP 230 (hemidesmosome and lamina lucida)
Target of Herpes gestationis
BP 180, BP 230 (hemidesmosome and lamina lucida)
Target of cicatricial pemphigoid
BP 180, laminin V (hemidesmosome and lamina lucida)
Target of Epidermolysis bullosa acquisita
Type VII collagen (anchoring fibrils)
Target of Bullous SLE
Type VII collagen (anchoring fibrils)
Target of of Linear IgA disease
Type I (childhood variant) = LAD1 (97k) =
Cleaved ectodomain of BP2 - 180kD = also called ladinin
Type 2
Target of Dermatitis herpetiformis
Tissue Transglutaminase
Prevalence of dermatitis herpetiformis
1.2 to 39.2 per 100,000;
avg age 41.8 y/o, rare in children
What type of enteropathy is assoc with Dermatitis Herpetiformis?
subclinical gluten-sensitive enteropathy (rice and corn are okay. Avoid Wheat and Barley)
HLA links for dermatitis herpetiformis?
*** HLA-DQw2 (100%) ***
HLA-B8 (60%)
HLA class II antigens HLA-DR3 (95%)
Difference b/tw IgA Bullous dermatosis and dermatitis herpetiformis
Linear IgA has no assoc bowel dz and is different histologically
Common areas for dermatitis herpetiformis
elbows, knees, scalp and nuchal area, shoulders and buttocks;
63% c/o of oral symptoms
Rarely see an intact bullous
Describe teeth defects in dermatitis herpetiformis
Celiac-type permanent tooth enamel defects (in 53% of pts)
What drugs can cause linear IgA bullous dermatosis (LABD)?
In general, may develop 24 hours to 15 days after first dose
S = Somatostatin/Sulfa
A = Amiodarone
D = Diclofenac
C = Captopril
L = Lithium/Lasix
I = IFgamma, IL2
P = Phenytoin, PCN, PUVA, Piroxicam
What drugs can cause linear IgA bullous dermatosis (LABD)?
How do you confirm Linear IgA Bullous dermatosis?
DIF shows linear deposition of IgA at the epidermal BMZ.
Some pts have both IgA and IgG at the BMZ.
No gluten-sensitive enteropathy.
What antibody can be found in patients with dermatitis herpetiformis that can correlate with jejenal damage?
IgA antiendomysial antibodies
What type of cancer has been linked to dermatitis herpetiformis?
Small bowel lymphoma and nonintestinal lymphoma
All cases in patients who did not adhere to a Gluten Free Diet!
*Hypothyroidism (hashimoto's) is another assoc d/o*
In LABD, what deposits are seen in DIF?
IgA in the BMZ
In DH, what are the DIF deposits?
IgA deposits in the dermal papillae
What type of circulating antibodies have been seen in patients with gluten sensitive enteropathy?
Abs to Endomysium, tissue tranglutaminase, reticulin, and gliadin
Treatment for dermatitis herpetiformis
Dapsone & Sulfapyridine
Other treatments: Gluten-Free diet, elemental diet
Tetracylcine + Nicotinamide
What medication interaction with Dapsone is important to know?
Probenicid blocks renal excretion
Rifampin incrases rate of plasma clearance
Side Effects of Dapsone
Elevated Liver Enzymes
Peripheral Neuropathy
Agranulocytosis/Aplastic Anemia
Side Effects of Dapsone
Describe Bullosis diabeticorum
may be huge, tense on non-erythematous base; Painless ulcer.
No immunopathologic feature found.
Tx: wound care
Common drug assoc with drug-induced pemphigus
HLA assoc with pemphigus?
HLA-DR4, DQ8 haplotypes in Jewish pts, HLA DR6, DQ5 in non-jewish
Name for endemic form of pemphigus foliaceus in rural S. America?
Fogo Selvagem
Where are Dsg1 and Dsg3 restricted to?
Stratified squamous epithelia
What is the most common form of Pemphigus?
Pemphigus vulgaris
Describe Pemphigus vulgaris lesions
mucosal involvement
thin blisters -- scalp, face, axilla, and oral cavities
What is Pemphigus vegetans?
Variant form of pemphigus with large verrucous confluent plaques and pustules localized to flexural areas in the axilla and groin
Pemphigus foliaceus rash
gradually on the face in a butterfly distribution -- seb derm distribution;
mucous membrane is rarey involved (desmoglein compensation theory*);
tends to be localized, better prognosis than PV
DDX for Pemphigus foliaceus?
Pemphigus foliaceus
IgA pemphigus -- has IgA ab and circulating IgA anti-cell surface Ab
Pemphigus erythematosus
Pemphigus erythematosus aka?
Senear-Usher syndrome
Combo of pemphigus foliaceus and SLE
Ways to Dx of Pemphigus
Bx for light microscopy
Indirect IF (to DSG1, 2, 3)
Treatment of PV
High dose systemic glucocorticoids
Topical Steroids
Adjuvants: Cyclophosphamide, Azathiaprine. MTX not used due to high incidence of infections reported.
Others: Plasmapheresis, extracorporeal photophoresis, or IVIG
Case reports: hydroxychloroquine
Combo of nicotinamide and TCN or MCN
Sfx of Cyclophosphamide
bone marrow suppression
hemorrhagic cystitis
bladder fibrosis
reversible alopecia
incrased risk of bladder carcinoma and lymphoma
Sfx of Azathioprine
bone marrow suppression
hepatotoxicity, increased risk of malignancy
Remission criteria
Can stop treatment when negative DIF or lower ab levels
What other diseases have been reportedly assoc with pemphigus?
Myasthenia gravis, thymoma, malignancy of the lymphoid or reticuloendothelial system
Drug induced vs drug triggered pemphigus?
Drugs with sulfhydryl radical (thiol drugs or SH drugs) induce PV (50% recovery when drug is discontinued)
Other drugs trigger the dz in predisposed people (only 15% recovery seen)
Criteria for the Dx of Neoplasia-Induced Pemphigus
All three major, or two major + two minor criteria.
Major Criteria:
Polymorphous mucocutaneous eruption
Concurrent internal neoplasia
Characteristic serum immunoprecipitation findings
Minor Criteria:
Postitive cytoplasmic staining of rat bladder epithelium by indirect immunofluorescence
Intercellular and BMZ immunoreactants on DIF of perilesional tissue
Acantholysis in biopsy specimen from at least one anatomic site of involvement
Clinical appearance of Neoplasia induced pemphigus
mucosal ulcerations
conjunctival reactions
polymorphous skin lesions on the trunk and extremities
DIF findings in Neoplasia induced pemphigus
- surface deposits of IgG and C3
- granular basement membrane deposits of C3
How can you differentiate PNP from PV?
Indirecti IF with rat bladder for PNP
(monkey for PV)
List the subepidermal blistering diseases (vs. intraepidermal)
Bullous Pemphigoid
Herpes gestationis
Cicatricial pemphigoid
Demographics of BP?
benign, autoimmune, subepidermal bullous dz of the elderly
Target of BP autoantibodies?
BP1 (230kD) & BP2 (180kD, aka type XVII collagen)
What is a bad predictive factor in BP?
generalized disease
Clinical picture of BP
Oral blisters (24%)
starts like an urticarial rash, then becomes dark and/or cyanotic, then becomes blistered
Areas: abdomen, groin, genital regions, flexor surfaces of arms and legs
palms and soles are affected
What lab tests correlate with disease remission in BP?
blood eosinophilia (in 50% of patients)
Histo of BP
subepidermal bullae w/ eos
DIF findings in BP
linear band at the BMZ (IGG, C3, IGA, IGM, AND FIBRIN CAN BE SEEN)
What diseases can linear band of IgG at BMZ be seen?
Epidermolysis bullosa acquisita
Cicatricial pemphigoid
Herpes gestationis
Bullous eruption of SLE
Which Dz can you monitor activity using IIF?
IIF in Pemphigus (monkey esophagus)
You can not use IIF to monitor BP
Treatment of BP
Atarax for itching
Systemic steroids combined with immunosuppressor (azathioprine, cyclophosphamide, MTX, or chlorambucil)
Antibiotics, dapsone and topical steroids are an option
cyclophosphamide aka?
What enzyme do you need to check prior to starting azathioprine?
Thiopurine methyltransferase (TPMT) activity
Azathioprine aka?
List variants of localized pemphigoid
Cicatricial pemphigoid (aka Mucous membrane pemphigoid - MMP)
Localized childhood vulvar pemphigoid
Pretibial Pemphigoid (nonscarring bullous lesions on legs of women)
Chronic pemphigoid of Brunsting-Perry (crops of grouped blisters on the head and neck that heal with atrophic scars)
dyshidrosiform pemphigoid (vesiculobullous hemorrhagic lesions of the palms and soles)
Pemphigoid vegetans (erosive and vegetating plaques)
Cicatricial pemphigoid
aka MMP
Pts older than 40;
2:1 women.
Oral disease 85% of cases -- spares the vermilion border in contrast to pemphigus
Eye Dz 65% -- relapse is common, fibrosis of the conjunctival sac leads to scarring and blindness in 20% of cases
Cutaneous lesions - 25%
Remission uncommon.
Target of antigens in Mucous Membrane Pemphigoid?
BMZ antigens: BP2 (180kD), Epiligrin (subunit of laminin 5), and B4-integrin.

All in lamina lucida and involved in keratinocyte adhesion to extracellular matrix
Brunsting-Perry type of cicatricial pemphigoid
cutaneous MMP involvement of the head and neck, but NOT the mucous membrane
Treatment of MMP
Topicals, IM injections
Dapsone & Corticosteroids first
Immunosuppressives -- cyclophosphamide is better than Azathioprine
Benign Chronic Bullous dermatosis of childhood aka?
Linear IgA type 1
Herpes gestationis
aka Pemphigoid gestationis
appears in pregnancy, usu 2nd or 3rd trimester, and recurs with subsequent pregnancy;
mucous membrane involvement is RARE
HLA assoc with H. gestationis?
Class II angigens HLA DR3 &
Class III antigen (C4)
AutoAb target in H. Gestationis?
IgG1 specific for BP2 (180kD), the NC16A domain
Histology of H. gestationis?
bandlike deposit of C3 and IGG at BMZ
circulating IgG (this is what can cause lesions in newborns - 10% incidence)
Treatment of H. Gestationis?
topical corticosteroids or oral
*Dz has risk of morbidity for child, unlike PUPPP*
Target of Ab in Epidermolysis Bullosa Acquisita (EBA)
Type VII collagen (anchors fibrils) -- same target as bullous SLE
Salt Skin - Ab on Floor
Classic EBA presentation
skin fragility, heals with scar and milia formation;
tense blisters on a noninflammatory base in trauma prone areas;
may have alopecia or nail dystrophy
BP-like EBA presentation
50% of pts w/EBA
Tense blisters on an inflammatory base on trunk and flexural surfaces
pruritis, minimal skin fragility, healing of some of the lesions w/o scarring and milia
Differentiating BP from EBA
Look the same on DIF
On salt-split -- IgG is on dermal side in EBA (vs epidermal side in BP)
Treatment of EBA
topical and/or systemic steroids
colchicine, cyclosporine, IVIG
Benign Familial Chronic pemphigus aka?
Hailey-Hailey Dz
Genetics of Hailey-Hailey
ATP2C1 (Ca2+ pump ATPase)
Describe clinical picture of Hailey-Hailey
grouped (in an annular pattern) pruritic vesicles from a red or noninflmaed base.
usu appears in adolescents, usu in the summer.
areas exposed to UV light or friction/heat/sweating
Longitudinal white bands in fingernails seen
Treatment for Hailey-Hailey blisters
Oral Abx can help along with topical steroids
For groin/axilla: add anti-yeast.
Other Rx: excision, topical cyclosporine, CO2 laser, grafting
Name the three major groups of Epidermolysis Bullosa
Epidermolysis Bullosa Simplex (92%) - epidermal, nonscarring
Dystrophic Epidermolysis Bullosa (5%) - dermal, atrophy and scarring
Junctional Epidermolysis bullosa (1%) -- junctional, atrophy
Where is the split in Epidermolysis Bullosa groups?
Epidermolysis Bullosa Simplex - epidermal basal cells
Dystrophic Epidermolysis Bullosa (5%) - split through upper dermis
Junctional Epidermolysis bullosa -- split through basement membrane area
Epidermolysis Bullosa Simplex
Target: K5 & 14 defect, AD.
May be sporadic; seen in childhood;
blisters seen especially on the palms and soles;
Biggest complication is infection
Junctional epidermolysis bullosa
AR: Laminin 5 or BP2 (Type XVII collagen)
"exuberant granulation"
Dystrophic epidermolysis bullosa
Collagen VII defect (same as EBA target) - "mitten deformities"
Hallopeau-Siemens (AD)
Non-Hallopeu (AR):
1. Cockayne-Touraine: acral, hypertrophic scars
2. Pasini: "albopapuloid" lesions on trunk
Dx of Epidermolysis Bullosas
Electron Microscope
Treatment of Epidermolysis bullosa?
avoid trauma
Dilantin - a collagenase inhibitor
Genetic counseling
What are the Main causes of benign transient neonatal pustulosis?
Erythema toxicum neonatorum
Transient neonatal pustular melanosis
Neonatal acne
First step for dx newborns with blisters?
Tzank smear
then, gram stain & KOH
Describe rash in Erythema Toxicum Neonatorum
Begins on face, trunk, proximal extremities and buttocks;
palms and soles not affected;
20-50% of term infants
What stain shows eos in ETN?
Wright's stain
Describe Transient neonatal pustular melanosis
LESIONS PRESENT AT BIRTH, but may be overlooked;
in 2-5% of blacks, 0.6% of whites;
Rash: forehead, behind hears, under the chin, on the neck & back, and on hands and feet;
palms and soles are affected;
Treatment for TNPM?
none -- resolves with hyperpigmented macules
What does Wright's stain show in TNPM?
PMNs & Eos
Difference b/tw miliaria crystallina and miliaria rubra
Occlusion levels -- crystallina is at the skin surface, so sweat is under the stratum corneum
rubra is in the intraepidermal section of the sweat duct which causes a diffuse erythema from inflammation
Causes of Drug-Induced Pemphigus?
I - Indocin
C - Captopril
R - Rifampin
A - Ampicillin
P - Penicillamine
Causes of Drug-Induced Pemphigus?
IIF for Pemphigus Foliaceus with what?
Guinea Pig Esophagus
Asboe-Hansen and Nikolsky signs in PV? vs. BP?
Positive in PV
Negative in BP
Ab in BP are what part of the salt split?
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