水皰病 羅珮瑜醫師

天皰瘡 (Pemphigus) 定義 免疫性水皰病 表皮內水皰  Immunoglobulins and complements are found bound to the intercellular substance in the perilesional epidermis

天皰瘡 (Pemphigus) 致病因  World-wide distribution  M=F, middle age  藥物 : D-penicillamine, etc.

天皰瘡 (Pemphigus) 臨床特徵  全身散在性 : 皺扁的水泡 Flaccid vesicles and bullae  Nikolsky‘s sign 陽性  口腔潰瘍 : > 50% of patients, as the initial lesions --generalized bullous phase in most patients occurs some 5 or more months after the onset of oral oral lesions.  好發區 :face, trunk, pressure points, groin, axillae  癒合情形 : without scarring but transient hyperpigmentation common.

天皰瘡 (Pemphigus)  病理 : Suprabasal separation with acantholysis  免疫螢光檢查 :  -- 直接 (D I F): IgG & C3 at intercellular space -- 間接 (I I F): -Intercellular antibody : 80-90% -Titers often parallel disease activity

天皰瘡 (Pemphigus) 治療  口服類固醇 : high dose  Immunosuppressive therapy -- Imuran, diminish the need for corticoseroids

類天皰瘡 (Bulous Pemphigoid) 定義  免疫性水皰病 : -starts with urticaria-like and pruritic erythematous lesions, later, large dense blisters in both erythematous and normal skin.  表皮下水皰 : intact epidermis forms the roof.

類天皰瘡 (Bulous Pemphigoid)  Age: 65~ 75 y/o  Sex: M=F  Bullous pemphigoid antigens are hemidesmosomal components, located below the basal epithelial cells.

類天皰瘡 (Bulous Pemphigoid) 臨床特徵  初始病狀 :Commonly starts with non-specific rashes on the limbs - 蕁痲疹狀 : 1-3 weeks before blisters occur - 濕疹狀 : several months. -General eruption of pemphigoid follows the prodromal phase and most of the body may be affected within a week. -arise on erythematous or normal skin and may be associated with subcutaneous edema.  飽滿的水皰 : flexural aspects of limbs and abdomen.  口腔潰瘍 : 少見

類天皰瘡 (Bulous Pemphigoid)  病理 : subepidermal blister, mainly eosinophils  直接 (D I F): IgG and C3 along the basement membrane zone.  間接 (I I F): circulating IgG attack basement membrane zone

類天皰瘡 (Bulous Pemphigoid) 治療  局部病灶 : topical or intralesional steroids  Systemic corticosteroid  Tetracycline, Erythromycin, Niacinamide  Azathioprine,dapsone

多形性紅斑 (erythema multiforme EM) 種類  EM minor,EM major or SJS, and TEN. 致病因  感染 : herpes simplex or mycoplasma, less severe  藥物 : 10%. severe

Stevens-Johnson syndrome(SJS)  好發 : young adult males  症狀 : high fever, asthenia, muscular pains, diarrhea, vomiting, arthralgias, and pharyngitis precede mucosal involvement by several days.  皮膚病灶 : 2 or more mucosal sites

Toxic epidermal necrolysis(TEN)  症狀 :skin tenderness that resembles a severe sunburn.  皮膚病灶 : 20% to 100% TBSA. Flaccid bullae may form. Nikolsky sign is positive. Two or more mucosal sites are involved in 85-95% of patients with TEN.  死亡率 : ~30%.

可能引起的藥物  antibiotics (sulfonamides, trimethoprim- sulfamethoxazole, penicillins, cephalosporins, chloramphenicol, clindamycin, griseofulvin, rifampin, streptomycin, tetracycline)  nonsteroidal anti-inflammatory agents (ibuprofen, acetylsalicylic acid, ketotifen, naproxen, piroxicam, sulindac)  antihypertensives,  anticonvulsants (phenobarbital, carbamazepine, phenytoin)  allopurinol.

病理變化  表皮細胞壞死 : interface dermatitis with necrotic keratinocytes  TEN shows massive and confluent necrosis of the basal cells

治療 支持性治療 預防性抗生素 短期服用類固醇 : may abort the process, but it will not restore epidermis that is already necrotic. Burn unit care

預後  EM most often has a good prognosis  SJS and TEN can be lethal depending on the extent of skin involvement and the age of the patient.