Skin lesions

Pemphigoid Vulgaris Flaccid bullae on Normal skin, erosions, crust, (+) nickolsky sign

Mucous Membra ne
Always involved

Distribution
Localized / generalized

Blister location/ histopathology

Suprabasal acatholysis

Immunopatho logy
IgG intracellular

serum
IgA intracellular epidermis Desmoglein 3 >> desmoglein 1

Treatment / Management
Prednisone: 23 mg/kg BW until
cessation of new blister formation & disappearance of Nikolsky sign

Immunosuppresive therapy given for glucocorticoidsparing effecr

Azathioprine : 2-3 mg/kg BW

until clearing 1mg/kg tapering dose

Methotrexate: 25 35 mg/week PO or IM Continued even

after cessation of glucocorticoid tx (months)

Plasmapharesi s Gold therapy (milder case)Initial dose

To reduce antibody titers

10mg IM 25 50mg gold sodium thiomalate IM every week to max. cumulative dose to 1g

Mycophenolate mofetil
1g BID (clinical studies still on going)

High dose intravenous immunoglobuli n (HIVIg)

2g/kg BW every 3 4 weeks Have glucocorticoid sparing effect

Bullous Pemphigoid

Tense bullae

mouth

Localized / generalized

Subepidermal blister

IgG & C3 linear at

IgG antibodies

Prednisone

50

(most common)

on Normal and/or erythematous skin, Urticarial plaques and papules Bullae rupture less than PV

basement membrane

to basement membrane. IIF directed to BPAG1e & BPAG2

100mg/day until clear. Used alone or in combination

Azathioprine
150mg/day for remission induction 50 -100mg for maintenance

Sulfones (dapsone)

for milder cases 100 150mg/day

Topical glucocorticoids
very mild cases

Tetracycline + nicotinamide
effective in some cases Patients often go to permanent remission, local recurrence controlled with glucocorticoids

Epidermolysis Bullosa

Tense / Flaccid, erosions, inflammatory

Severe, esophagus, oral, vagina

Traumatized regions

Subepidermal

Linear IgG at basement membrane

IgG antibodies

a)

EB Simplex Generalized (koebner) Localized (weber-cockayne) a) a) Epidermis or uppermost layer of skin cells (keratinocytes ) Lamina lucida within the basement membrane zone (layer lying between the epidermis and dermis) Lamina densa and upper dermis (deeper

No therapy for EB Management tailored to severity, extent of skin involvement

Supportive Systemic treatment for complications Wound management Nutrition Infection control Cool environment Soft well ventilated shoes Saline compress, topical antibiotics and steriods Surgical intervention on

b)

Junctional EB (Herlitz)

b)

c)

Dystropic EB

c)

layers of skin cells)

cutaneous infection Release of contractures IgG intracellular IgG antibodies desmoglein 1 only Antiendomysi al antibodies

Pemphigous Folliaceous Dermatits Herpetiformis (Gluten Sensitive)

Pemphigoid Gestationis

Crusted erosion / flaccid vesicles Grouped papules, vesicles, urticarial plaques, crust Tense bullae, urticarial plaques, papules, vesicles, erythematous , edematous

Rare

Exposed seborrheic regions or generalized Elbows, knees, gluteal, sacral and scapular areas

Acantholysis in granular layer Papillary, miscroabsces, subepidermal vesicles

None

Granular IgA on tips of papilla

Dapsone, sulfapyridine, gluten free diet Antihistamine, topical steroids, Prednisone 20

40mg/d tapered gradually during post partum

none

Pregnant & postpartum >> abdomen

Subepidermal

C3 in basement membrane IgG

IgG antibasal membrane antibodies HG factor

Cicatricial Pemphigoid

Skin lesions in only 30% of patients

Mouth, oropharynx

Ocular Unilateral or bilateral conjunctivitis & other ocular diseases

BPAG2, type VII collagen, integrin B4 M168 antigen Laminin 5

Dapsone + low dose prednisone Cyclophospha mide, azathioprine + steroids surgery