- pemphigus vulgaris

-pemphigus foliaceous

-pemphigus vegetans

-paraneoplastic pemphigus

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

- 40-60 is common

may be nail disease

- 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

- skin biopsy for HandE and DIF

-Blood tests for circulating antibodies

- high dose steroids

-immunosuppressive agents: azathioprine, cyclophosphamide, methotrexate, rituximab

- 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.

- biopsy of skin histolgy and DIF

- serology IIF ab in blood or blister fluid

- potent topical steroids

-oral steroids

immunosuppressive agents: methotrexate, azathioprine, mycophenolate

- early adult life

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

gluten sensitive enteropathy and other autoimmune diseases like

thyroid disease, diabetes

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

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  

Can epidermolysis affect nails?

Which joints does it usually affect on the feet?

Yes

IPJ joints

• 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

-  Lesional for histopathology

  (drugs, gluten sensitivity, IgA disease)

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

- friction

-thermal injury

-chemicals

- diabetic acral bulla

-coma

-renal failure, porphyrias

-acute dermatitis

-impetigo

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,

- 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

[image]

Cause of this blistering?

[image]

Cause of this blistering?

[image]

Cause of this blistering?

[image]

What is causing this blisterning at the IPJ joints?

[image]

- disorder of keratinisation

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

smooth discoloured, annular lesions that are asymptomatic

limbs and trunk

females mainly, all ages

self resolving

topical - steroids, TCNI

Intralesional steroids

phototherapy

systemci retinoids, steroids, hydroxychloroquine