B ULLOUS DISEASES. Vesicles and bullae are accumulations of fluid within or under the epidermis. Subepidermal blisters Occur between the dermis and the.

BULLOUS DISEASES

Vesicles and bullae are accumulations of fluid within or under the epidermis.

Subepidermal blisters Occur between the dermis and the epidermis. Their roofs are relatively thick and so they tend to be

tense and intact. They may contain blood.

Intra-epidermal blisters appear within the spinosum cell layer of the

epidermis So have thin roofs and rupture easily Leave an oozing denuded surface.

Subcorneal blisters Form just beneath the stratum corneum at the

outermost edge of the epidermis Have even thinner roofs Tendency to break is more marked

CLASSIFICATION*** Bullous Pemphigoid

Bullous Pemphigoid Chronic Bullous Disease of Childhood Cicatricial Pemphigoid Epidermolysis Bullosa Acquisita Linear IgA Dermatosis Pemphigoid

Pemphigus Pemphigus Pemphigus Foliaceus Pemphigus Vegetans Pemphigus Vulgaris

Age:middle aged Clinical features:

monomorphic Blisters:

flaccid,ruptures easily

Content of blisters: fluid filled.

Oral lesion :are common

Nikolsky’s sign:Positive

Tzank smear:acantholytic cells are seen

Elderly Polymorphic

Tense ,firm

Mostly hemmorhagic

o Less commono Negative

o No acantolytic cells.

pemphigus pemphigoid

BULLOUS PEMPHIGOID

It is an autoimmune disorder, meaning it is caused when the body's immune system malfunctions.

The immune system is meant to defend the body against bacteria, viruses, and disease, but instead produces antibodies against healthy tissue, cells and organs.

Some patients with BP have other diseases such diabetes and rheumatoid arthritis.

Other factors triggering BP include drugs (Furosemide, penicillin), mechanical trauma, and physical traumas (burns from radiation, sun or heat)

BULLOUS PEMPHIGOID*** Epidemiology

Age of onset- elderly Men and women affected equally

Pathophysiology Autoimmune Subepidermal Blistering

Symptoms Pruritus Lesions may be tender at borders

Signs Initial Urticarial eruption Develops into large tense bullae after weeks to months

Contain clear fluid or may at times be hemorrhagic Diffuse cutaneous involvement

Often involves lower legs, forearms, thighs, groin Also involves abdomen Mucosa much less affected than in pemphigous vulgaris

No scar formation Milia formation as lesion heals

Bullous Pemphigoid

Labs Histology

Supepidermal Blister Superficial dermal inflammation

Immunofluorescence Ig G and C3 deposition along basement membrane zone

Management First-Line

Prednisone Methotrexate may be used for prednisone intolerance Topical corticosteroids in localized mild cases

Adjunctive agents Azathioprine Mycophenolate mofeti Tetracycline

Course Self-limited condition Remits with treatment by 6 years in 50 % of cases

CHRONIC BULLOUS DISEASE OF CHILDHOOD Epidemiology

Age of Onset: Under age 5 years Pathophysiology

Autoimmune bullous disorder Variant of Linear IgA Dermatosis (seen in adults)

Symptoms Pruritus and burning sensation

Signs Tense inflamed Blisters over red base

May appear as collarettes of Blisters Distribution

Most commonly found on genitalia Also may involves face and perioral skin Oral Mucosa involved in 50% of cases

Course Resolves within 2 years of onset in most cases

Histology Subepidermal Blisters Inflammatory infiltrates at basement membrane zone

Immunofluorescence IgA deposition along basement membrane

Management First Line: Dapsone or Sulfapyridine

(requires G6PD and monitoring of CBC) Adjunct: Low dose prednisone initially

CICATRICIAL PEMPHIGOID ***Epidemiology

Incidence (rare): 1 in 12,000 to 20,000 Ages affected: 60 to 80 years Gender predominance: Women by male ratio of 2:1

Pathophysiology Autoimmune bullous condition Associated conditions

Stevens-Johnson Syndrome Medications

Topical Glaucoma medications Practolol Clonidine

Signs Blistering of mucus membranes

Blister ruptures within hours of formation Painful Erosion

Most Erosions heal without scarring

Most common sites of involvement Oral Mucosa (most often buccal and palatal mucosa)

Results in erosive Gingivitis Conjunctiva (usually bilateral)

Results in chronic Conjunctivitis and scarring Other sites of involvement

Skin (usually non-scarring) Scalp (Cicatricial Alopecia) Pharynx and larynx External genitalia Nasal mucosa Anus Esophagus

Labs: Histology Subepidermal bulla Perivascular inflammatory cell infiltrates Fibrosis distinguishes from Bullous Pemphigoid Immunofluorescence with IgG, C3, IgA deposits

Forms linear band at Basement Membrane Zone

Differential Diagnosis Bullous Pemphigoid Epidermolysis Bullosa Acquisita(EBA) Linear IgA Bullous Dermatosis(LABD) Systemic Lupus Erythematosus Pseudopemphigoid Pemphigus

MANAGEMENT: Early aggressive treatment

First-Line: Prednisone 0.5 to 0.75 mg/kg/day x6 months Ocular Involvement: Azathioprine or Cyclophosphamide Skin Involvement: Topical Corticosteroids Oral Involvement

Dapsone Corticosteroid gel Dexamethasone mouthwash

Complications Ocular Lesions may result in blindness Laryngeal stricture Esophageal Stricture Supraglottic Stenosis

EPIDERMOLYSIS BULLOSA ACQUISITA**

Epidemiology Mean age of onset: 50 years Few isolated cases of children seen

Pathophysiology Autoimmune Subepidermal Blistering condition

Signs Trauma prone areas more commonly affected Tense Blisters and Erosions over extensor surfaces

Knuckles Dorsal hands Elbows Knees Ankles

Mucosal involvement Oral, nasal, and esophageal mucosa Conjunctival mucosa

CLASSIFICATION1. Simple Epidermolysis bullosa2. Junctional Epidermolysis bullosa3. Dystrophic Epidermolysis

bullosa4. Acquired Epidermolysis bullosa

Junctional Epidermolysis bullosa

Dystrophic Epidermolysisbullosa

LINEAR IGA DERMATOSIS Epidemiology

Onset over age 30 years Pathophysiology IgA mediated dermatosis mediated by IgA

and causes "sausage-like" lobulated blisters arranged at the outer edges of flattened erythematous patches. Autoimmune bullous disorder Variant of Chronic Bullous Disease of Childhood

Symptoms Pruritus

Signs Annular Lesions

Papules (may be excoriated and crusted) Vesicles Bullae

Distribution Symmetric involvement on extensor surfaces Elbows, knees, and buttocks affected Mucosa involvement may occur (Conjunctiva, oral)

Labs Histology

Subepidermal Blister with inflammatory infiltrate Immunofluorescence

IgG deposits at dermal-epidermal junction

Complications Scarring skin lesions (with associated Milia)

Differential Diagnosis Porphyria cutanea tarda Hereditary Epidermolysis bullosa Bullous Pemphigoid Course Chronic waxing and waning course

Linear IgA Dermatosis