vesiculobullous diseases in dermatology11-1.pptx

vesiculobullous disease dermatologic

1. Kibrom Tekleab Autoimmune bullous disorders

2. Pemphigus - is a group of autoimmune disease characterized by blisters, erosion of the skin & mucous membrane and autoantibodies against cell surface antigen detected by immunofluorescence

3. Epidemiology Worldwide distribution Incidence 1-5 cases / million /year PV > PF except in Tunisia, Brazil M=F Mean age of onset 40-60 years

4. Clinical feature Pemphigus vulgaris Flaccid blisters occurring anywhere on the skin surface on normal or erythematous skin Lesions are often painful and rarely pruritic Positive Nikolsky sign - shearing away of the epidermis after applying lateral pressure at periphery Painful mucous membrane erosions From mucous membranes, oral mucosa – most often affected, may involve the pharynx, larynx

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7. Pemphigus foliaceous Scaly, crusted lesions on erythematous base The primary lesion is a flaccid blister In localized form, well demarcated & in seborrheic distribution Generalized- may result in exfoliative erythroderma Mucosal involvement- not seen

8. CLINICAL… 8/1/2014 11 Diagnosis Clinical Nikolsky sign: firm sliding pressure with a finger separates normal-appearing epidermis, producing an erosion. This sign is not specific for PV and is found in other active blistering diseases. Asboe-Hansen sign: Lateral pressure on the edge of a blister may spread the blister into clinically unaffected skin. Immunofluorescence

9. Treatment Rx started at the onset of the disease In PV- systemic Rx In PF- topical /localized/ systemic /generalized/ Systemic administration of glucocorticoids is the mainstay of Rx in pemphigus Moderate dose of predinsolone with immunosuppressive drugs in some cases

10. Once disease activity is controlled it is tapered Add adjuvant Rx If : ▫ contraindications ▫ steroid alone didn’t control it ▫ dosage not effective - Predinsolone + immunosuppressive Immunosuppressive agents : Azathioprine Mycophenolate mofetil Cyclophosphamide

11. Prognosis & clinical course Without Rx PV- fatal PF- ~70% of cases fatal / especially in elderly/ Cause of death- sepsis/Rx is a contributing factor/ With glucocorticoids & immunosuppressive, mortality ~ 10 % in PV & less in PF

12. Paraneoplastic pemphigus An autoimmune disease linked to lymphoproliferative disorders Non-Hodgkin lymphoma, CLL & castleman disease The disease is characterized by : Polymorphous cutaneous eruption Mucocutaneous ulceration Underlying neoplasm

13. Clinical features Intractable stomatitis: Earliest manifestation Erosion & ulceration Prefer the lateral border of the tongue, extend to involve the vermillion of the lips Persist throughout the course of the disease Resistant to treatment

14. Related findings ~ 30-40% of patients develop pulmonary injury Early- dyspnea/progressive/, normal CXR Late- abnormal pulmonary function test - deteriorates despite treatment Endoscopic biopsy – acantholysis of the bronchial respiratory epithelium

15. Rx If tumor is benign or encapsulated, surgical removal Disease improves substantially or go into remission/1-2 years/ In pts with malignant tumor- no regimen which is consistently effective Oral glucocorticoids

16. Bullous pemphigoid Introduction Chronic , autoimmune, subepidermal, blistering skin disease -that rarely involves mucous membrane. IgG autoantibodies specific for the Bullous pemphigoid antigens Epidemiology Majority -> 60 yrs at the age of onset Peak - >80 yrs Rarely -in children No known racial,ethnic or sexual predilection

17. Clinical feature Non- bullous phase Sn and Sx - are frequently non-specific Mild to severe intractable pruritus alone or In association with excoriated, eczematous, papule and/or urticarial lesions that may persist for several weeks or months. Often starts on the limbs When urticarial, the prodrome lasts 1-3 weeks before blisters occur When eczematous the blisters may not develop for several months

18. Bullous phase Large, tense blister- arising on normal skin or on an erythematous base Most common-lower abdomen, inner or anterior thighs, and flexor forearm Bullae usually are filled with clear fluid but may be hemorrhagic Lesions typically heal without scarring or with milia formation.

19. Course and Prognosis Usually self-limited over a 5- to 6-year period. This period is generally a year or less in children. In most patients who are treated, BP remits within 1.5-5 years and relapse occurs in 10% to 15% of patients once therapy is discontinued Poor prognosis is associated with Greater age Higher daily steroid dosage at discharge Low serum Albumin ESR >30mm/hr

20. Treatment Mild BP sometimes resolves without Rx but resolution usually takes months or years Localized bullous pemphigoid Topical corticosteroids alone Topical calcineurin inhibitor More extensive disease Usually treated with oral predinsolone 0.5-1 mg/kg/day)-usually controls the disease within 1 or 2 weeks, progressively tapered over a period of 6-9 months.

21. Linear IgA dermatosis and chronic bullous dermatosis of childhood Introduction Linear lgA dermatosis and chronic bullous dermatosis of childhood are rare immune-mediated blistering skin diseases that is defined by the presence of homogeneous linear deposits of IgA at the cutaneous basement membrane

22. Epidemiology The true incidence of LABD is unknown The average age of onset of LABD is after 60 years of age. Childhood LABD occurs at a mean age of 4.5 years Pathogenesis Linear IgA dermatosis and CBDC are defined by the presence of a homogeneous linear band of IgA at the dermal-epidermal basement membrane zone

23. Clinical Features Are variable Patients may present with combinations of annular( string of pearls) or grouped papules, vesicles, and bullae. The trunk is almost always involved, and the limbs, face and scalp, hands and feet are commonly affected. Typically, these lesions are distributed symmetrically

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25. The clinical presentation of CBDC is characterized most often by the development of tense bullae, often on an inflammatory base. Lesions occur most frequently in the perineum and perioral region and often occur in a " cluster of jewels“ appearance

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27. Treatment The majority of patients with LABD respond to either oral Dapsone or Sulfapyridine therapy (250 mg/day to 3 g/day) Most patients with LABD have a clinical response within 48-72 hours. The average dose of Dapsone required to control LABD is 100 mg daily, but doses as high as 300 mg daily may be needed.

28. Prognosis CBDC remits within 2-4 years. Occasionally , the disease persists well into puberty but often is less severe than the initial eruption CBDC after remission it often does not recur.

29. Dermatitis herpetiformis Introduction Dermatitis herpetiformis (DH) is an autoimmune blistering disorder associated with a gluten-sensitive enteropathy (GSE). Characterized by an intensely itchy, chronic, papulovesicular eruptions

30. Epidemiology Internationally Prevalence as high as 10 cases per 100,000 population has been reported Onset of DH is in the second to fourth decade Pathophysiogy IgA deposition within the papillary dermis in a granular fashion

31. Clinical features Typical lesion are flesh-colored to erythematous excoriated papules or plaques with herpetiform vesicles. Patients may also have many individual non-grouped lesions. Symmetrically distributed over extensor surfaces, including the elbows, knees, buttocks, and shoulders.

32. Diagnosis Clinical Histopathology Immunoflorescence Management Cornerstones of management are Dietary measures and drug therapy Gluten free diet Dapsone is the first line therapy for DH Sulfapyridine is indicated in patients intolerant of dapsone ,