小動物疾病系統

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落葉型天疱瘡
作者
陳柏達、賴政宏、鄭豐邦
中文病名 落葉型天疱瘡
英文病名 Pemphigus foliaceus
疾病描述
病因:
    落葉型天疱瘡是一種自體免疫性疾病。正常皮膚表面之角質細胞(eratinocyte)之間具有黏著分子複合物-橋粒(desmosome),使細胞彼此緊密連接。落葉型天疱瘡,為其中的Dsg 1蛋白(desmoglein 1)會被自體抗體IgG所攻擊,進而形成抗原抗體複合物,沉積於細胞間隙,最終使的細胞間的黏著力喪失而自表皮淺層脫落,即所謂的表皮棘層鬆解(acantholysis)。
    落葉型天疱瘡是犬貓天疱瘡中最常見的一型,可發生於任何年齡層、性別、品種,其中又以秋田犬(Akitas)、鬆獅犬(Chow Chows)、杜賓犬(Doberman)、可利牧羊犬(Collie)、長鬚牧羊犬(Bearded Collie)、紐芬蘭犬(Newfoundland)、舒柏奇犬(Schipperke)、英國激飛獵犬(English Springer Spaniel)、中國沙皮犬(Chinese Shar Pei)等為好發品種。
    落葉型天疱瘡大多屬於自發性疾病(idiopathic),少數病例可經由藥物引起,或是經慢性炎症皮膚病所產生的後遺症。
臨床症狀:
    最初之病灶為表層的膿疱,但由於皮毛的遮蔽或是膿疱的破裂,臨床上往往較難發現完整之膿疱病灶。繼發的病灶則包括了表層的糜爛、結痂、鱗屑、頸圈型病變、脫毛。典型的好發部位為鼻鏡至鼻樑、耳廓、足墊。在貓的病例中,尚有指甲床及乳頭等獨特好發部位。此外,一般落葉型天疱瘡病例中,顏面病灶也常會涉及至眼部周圍,或是伴隨鼻子的脫色素變化(depigmentation)。少數病例中,足墊的過度角化現象(hyperkeratosis)可能是唯一的症狀。相較於尋常型天疱瘡(pemphigus vulgaris),落葉型天疱瘡較沒有口腔及皮膚黏膜的病灶。

    除了皮膚病灶外,可能還有淋巴腫大(lymphadenomegaly)、四肢水腫(limb edema)、發燒(fever)、厭食(anorexia)以及抑鬱(depression)等情形。

診斷方法
1.            經由病史、好發品種初步懷疑。
2.            經由臨床症狀、好發部位初步懷疑。
3.            細胞學檢查(cytology):病灶採樣以原發病變的膿皰較為理想,若膿皰皆已破裂,可以捎刮之鱗屑代替。以25G 針頭吸取膿皰內滲出液,製成塗抹片,以顯微鏡檢查。鏡檢結果可發現大量嗜中性球,伴隨大量具診斷特徵性之圓形至橢圓形角質細胞,即棘狀細胞(acantholytic cells)。偶而可見少數嗜酸性球(eosinophils)及細菌。
4.            排除其他類症病變,如:毛囊蟲症(demodicosis)、表層膿皮症(superficial pyoderma)、皮癬菌症(dermatophytosis)、其他自體免疫性疾病、嗜酸性膿皰病(eosinophilic pustulosis)、皮肌炎(dermatomyositis)、藥疹(drug eruption)、皮膚型淋巴瘤(epitheliotropic lymphoma)、鋅反應性皮膚病(zinc-responsive dermatosis)等。
5.            皮膚組織病理學檢查(dermatohistopathology):將病灶區進行活體組織採樣(biopsy)後送檢,經由皮膚組織病理學家將其製成組織病理切片後鏡檢並診斷。臨床上百分之八十的病例可經此檢查提出有利證據之診斷。

免疫螢光染色(immunofluorescence)或免疫組織化學染色(immunohistochemistry):直接檢驗細胞間之抗體沉積物。可用於皮膚組織病理學檢查仍無法確診之病例,可提供最有力證據之診斷。

治療方法
1.          外用洗毛精全身性治療:目的為去除身上之痂皮、鱗屑,保持體表清潔。
2.          治療或是預防繼發感染的膿皮症(針對狗):給予長期全身性抗生素,至少持續4周,建議持續投藥至免疫抑制治療達到控制為止。研究統計,免疫抑制治療合併給予抗生素之犬隻,其存活率往往會比單獨以免疫抑制治療之犬隻高。
3.          免疫抑制治療:目的在於抑制症狀,控制病情。一般採用合併多藥物治療計畫,以降低單一藥物之副作用。而治療劑量的強度,則視病情嚴重程度而定,往往治療初期為了達到病情緩解的作用,會使用較高之劑量,但控制病情後的2-3月需要逐量的降低劑量,直至達到最低有效劑量為止,以便未來長期性的給藥。以下介紹四種類型建議之治療藥物:(建議之藥物種類及劑量,請參考下方之附表一)
A.        局部性治療藥物:
    每12小時於病灶處及其周圍塗抹適量之含steroid或tacrolimus之乳劑藥物,以減少病灶處的炎症反應。病情達控制後,再藉由降低塗抹頻率或劑量以減少藥物之局部副作用。若於全身性治療時搭配使用,則可降低投與全身治療藥物之劑量。
B.         保守性全身性治療藥物:
    目的在於全身治療的情況下,以產生較少之副作用為前提,以降低病灶炎症反應及降低其他副作用藥物較大之藥物劑量,如steroids或其他化療制劑(chemotherapeutics)。此類藥物多為輔助治療藥物,如:抗生素tetracycline、維生素E、必須脂肪酸等營養補充物。
C.         非固醇類免疫抑制藥物:
    建議的有效藥物包含了cyclosporine, azathioprine, chlorambucil, cyclophosphamide, mycophenolate mofetil, leflunomide等,往往在投與8-12周後病情會有明顯之改善。於病情緩解時需逐量的降低劑量與投藥頻率。
D.        固醇類免疫抑制藥物:
    對自體免疫性的皮膚疾病而言,固醇類藥物是治療強度最強,最有效的維持症狀緩解的藥物,但同樣也是副作用最大的藥物,特別是對於犬隻,所以於病情緩解時逐量的降低劑量與投藥頻率是絕對必要的。一般也會建議與上述的非固醇類藥物併用以降低劑量,才作為長期性的給藥劑量。
   prednisone與methylprednisolone為常用之第一線口服藥物,給予此藥物需要定期的進行血清生化值的監控,以避免副作用傷害的擴大。達到症狀緩解的劑量時,建議維持此劑量2-8周後,逐量減低劑量約8-10周內調整至最低有效劑量。若給予投藥治療後的2-4周內仍無明顯改善,則要可慮更換或是加入別種免疫抑制藥物。
    對於prednisone與methylprednisolone具抵抗性之病畜,常改用triamcinolone或dexamethasone作為治療藥物。對於貓而言,此兩種藥物的治療效度往往會比prednisone與methylprednisolone來的好。triamcinolone或dexamethasone同樣需要進行定期監控與逐量減低劑量等工作。
     若給予投藥治療後有產生嚴重的副作用,則要可慮更換別種醣皮質固醇類藥物,或是使用非固醇類之藥物。

經治療療程結束後,一般預後判定為尚可至良好,但絕大多數之患畜於停止治療後會有復發之情況,所以正規之治療計畫是需要終身投藥的,並且於投藥期間需進行定期血清生化監控。

預防控制方法

並無有效之預防控制方法。患畜需要終身投藥,且長期監控病情。

相關圖片 <p><span style="font-size: 12pt"><strong>各種治療計畫之建議藥物種類及劑量</strong></span></p>
Close<p><span style="font-size: 12pt"><strong>各種治療計畫之建議藥物種類及劑量</strong></span></p>

<p><span style="font-size: 12pt"><strong>各種治療計畫之建議藥物種類及劑量</strong></span></p>


<div><b>圖一、落葉型天疱瘡</b>。患犬臉部之肉眼病變,病灶多分布於鼻鏡至鼻樑、眼窩周圍及耳廓。可見皮膚表層的脫毛、糜爛、結痂及鼻頭的脫色素變化。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>
Close<div><b>圖一、落葉型天疱瘡</b>。患犬臉部之肉眼病變,病灶多分布於鼻鏡至鼻樑、眼窩周圍及耳廓。可見皮膚表層的脫毛、糜爛、結痂及鼻頭的脫色素變化。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>

<div><b>圖一、落葉型天疱瘡</b>。患犬臉部之肉眼病變,病灶多分布於鼻鏡至鼻樑、眼窩周圍及耳廓。可見皮膚表層的脫毛、糜爛、結痂及鼻頭的脫色素變化。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>


<p>&nbsp;</p>
<div><b>圖二、落葉型天疱瘡</b>。患貓臉部之肉眼病變,,病灶多分布於鼻鏡至鼻樑、眼窩周圍及耳廓,可見皮膚表層的脫毛、糜爛、結痂及鼻頭的脫色素變化。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>
Close<p>&nbsp;</p>
<div><b>圖二、落葉型天疱瘡</b>。患貓臉部之肉眼病變,,病灶多分布於鼻鏡至鼻樑、眼窩周圍及耳廓,可見皮膚表層的脫毛、糜爛、結痂及鼻頭的脫色素變化。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>

<p>&nbsp;</p> <div><b>圖二、落葉型天疱瘡</b>。患貓臉部之肉眼病變,,病灶多分布於鼻鏡至鼻樑、眼窩周圍及耳廓,可見皮膚表層的脫毛、糜爛、結痂及鼻頭的脫色素變化。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>


<div><b>圖三、落葉型天疱瘡。</b>患犬足墊之肉眼病變,可見明顯的過度角化現象。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>
Close<div><b>圖三、落葉型天疱瘡。</b>患犬足墊之肉眼病變,可見明顯的過度角化現象。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>

<div><b>圖三、落葉型天疱瘡。</b>患犬足墊之肉眼病變,可見明顯的過度角化現象。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>


<div><b>圖四、落葉型天疱瘡。</b>患貓趾甲床之肉眼病變,可見明顯的紅腫、脫毛、痂皮等現象(甲溝炎paronychia)。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>
Close<div><b>圖四、落葉型天疱瘡。</b>患貓趾甲床之肉眼病變,可見明顯的紅腫、脫毛、痂皮等現象(甲溝炎paronychia)。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>

<div><b>圖四、落葉型天疱瘡。</b>患貓趾甲床之肉眼病變,可見明顯的紅腫、脫毛、痂皮等現象(甲溝炎paronychia)。<span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">(</span><span lang="EN-US" style="font-size: 12pt; font-family: &quot;Times New Roman&quot;,&quot;serif&quot;; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA; mso-fareast-font-family: 標楷體">Keith, 2011</span><span style="font-size: 12pt; font-family: 標楷體; mso-ascii-font-family: 'Times New Roman'; mso-hansi-font-family: 'Times New Roman'; mso-bidi-font-family: 'Times New Roman'; mso-font-kerning: 1.0pt; mso-ansi-language: EN-US; mso-fareast-language: ZH-TW; mso-bidi-language: AR-SA">)</span></div>


參考文獻
1.          陳朝澧。診斷方法與設備。引自:陳朝澧,臨床小動物圖譜,第一版。台北,合記圖書出版社,11-29,1997。
2.          Keith AH. Autoimmune and immune mediated skin disorders. In: Keith AH. Small animal dermatology: a color atlas and therapeutic guige, 3rd ed, Elsevier Saunders, Philadelphia, 227-238, 2011.

Tim Nuttall, Richard GH, Patrick JM. Papular and pustular dermatoses. In: Tim Nuttall, Richard GH, Patrick JM. A colour handbook of skin dieases of the dog and cat, 2nd ed, Manson Publishing, London, 155-158, 2009.

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