Relationship of Serum Levels of Anti-Desmoglein Antibodies and Direct Immunofluorescence Findings with Clinical Activity of Pemphigus

Y�lmaz, Mediha; B�lb�l Ba�kan, Emel; Budak, Ferah; Sar�cao�lu, Hayriye; Tunal�, ��kran

Relationship of Serum Levels of Anti-Desmoglein Antibodies and Direct Immunofluorescence Findings with Clinical Activity of Pemphigus [Turkderm-Turk Arch Dermatol Venereol]

Turkderm-Turk Arch Dermatol Venereol. 2011; 45(2): 77-82 | DOI: 10.4274/turkderm.45.18

Relationship of Serum Levels of Anti-Desmoglein Antibodies and Direct Immunofluorescence Findings with Clinical Activity of Pemphigus

Mediha Y�lmaz¹, Emel B�lb�l Ba�kan¹, Ferah Budak², Hayriye Sar�cao�lu¹, ��kran Tunal�¹
¹Department Of Dermatology, Uluda� University Medical Faculty, Bursa, Turkey
²Department Of Microbiology, Uluda� University Medical Faculty, Bursa, Turkey

Background and Design: Pemphigus is an autoimmune disease that results in blistering of the skin and mucous membranes. In this study, we investigated the relationship between disease activity and remission with ELISA scores and direct immunofluorescence (IF) - two methods used for the detection of antibodies against desmoglein-1 (dsg-1) and desmoglein-3 (dsg-3) that are responsible for blister formation.
Material and Method: Twenty-three pemphigus vulgaris patients and two pemphigus foliaceus patients were enrolled in the study. The serum levels of anti-dsg-1 and anti-dsg-3 antibodies were measured with ELISA before therapy and at 3, 6, and 12 month of clinical remission. Concurrently, direct IF was performed on perilesional skin during active disease and on normal buttock skin/lower lip mucosa in remission. The tests were repeated if relapse has occured.
Results: Anti-dsg-1 was detected in 17 (73.9%) pemphigus vulgaris patients and anti-dsg-3 in 23 (100%) pemphigus vulgaris patients. In two pemphigus foliaceus patients, anti-dsg-1 values were positive, while anti-dsg-3 values were negative. A statistically significant correlation was seen between anti-dsg-1 antibody serum levels and skin severity scores (r: 0.577; p: 0.003), as well as between anti-dsg-3 antibody serum levels and oral mucosa severity scores (r: 0.539; p: 0.008). Direct IF results in 16 patients (84.2%) who achieved complete remission were negative. In 9 patients who relapsed, elevated serum values of anti-dsg-1 and/or anti-dsg-3 were also found. Increase in serum antibody levels was detected 1-4 months before the relapse in three of them.
Conclusion: In this study, we observed that serum desmoglein antibody levels correlated with disease severity and activity. In clinical remission, serial measurements of desmoglein antibodies can provide a guide for clinical follow-up and treatment modification.

Keywords: Pemphigus vulgaris, pemphigus foliaceus, direct immunofluorescence, desmoglein antibodies, disease activity

Pemfigusta Desmoglein Antikor Serum D�zeyleri ile Direkt �mm�nofloresan Bulgular�n�n Hastal��n Klinik Aktivitesi ile �li�kisi

Mediha Y�lmaz¹, Emel B�lb�l Ba�kan¹, Ferah Budak², Hayriye Sar�cao�lu¹, ��kran Tunal�¹
¹Uluda� �niversitesi T�p Fak�ltesi, Deri Ve Z�hrevi Hastal�klar Anabilim Dal�, Bursa
²Uluda� �niversitesi T�p Fak�ltesi, Mikrobiyoloji Anabilim Dal�, Bursa

Ama�: Pemfigus deri ve mukozalarda b�l olu�umuyla seyreden, otoimm�n bir hastal�kt�r. Bu �al��mada b�l olu�umunda rol� olan desmoglein-1 (dsg-1) ve desmoglein-3 (dsg-3)�e kar�� olu�mu� antikorlar�n saptanmas�nda kullan�lan iki y�ntem olan direkt imm�nfloresan (IF) inceleme ve ELISA y�nteminin hastal�k aktivitesi ve remisyonla ili�kisi ara�t�r�lmaktad�r.
Gere� ve Y�ntem: �al��maya 23�� pemfigus vulgaris, 2�si pemfigus foliaseus tan�s� alm�� toplam 25 hasta al�nd�. Hastalar�n tedavi �ncesi ve klinik remisyonun 3., 6. ve 12. aylar�ndaki anti-dsg-1 ve anti-dsg-3 serum antikor d�zeyleri ELISA ile ara�t�r�ld�. E� zamanl� olarak aktif hastal�kta lezyon kenar�ndan, remisyonda ise sa�lam kal�a derisi/alt dudak mukozas�ndan direkt IF inceleme yap�ld�. N�ks halinde tetkikler tekrarland�.
Bulgular: Pemfigus vulgaris hastalar�n�n tedavi �ncesi 17�sinde (%73,9) anti-dsg-1 antikoru, hepsinde (%100) anti-dsg-3 antikoru pozitif saptand�. �ki pemfigus foliaseus olgusunda tedavi �ncesi anti-dsg-1 pozitif de�erlerde iken anti-dsg-3 negatif saptand�. Anti-dsg-1 antikor serum d�zeyleri deri �iddet skoru ile (r: 0,577; p: 0,003), anti-dsg-3 antikor serum d�zeyleri ise oral mukoza �iddet skoru ile korele idi (r: 0,539; p: 0,008). Tam remisyona giren hastalar�n 16�s�nda (%84,2) tedavi �ncesi direkt IF�da saptanan birikim remisyonla birlikte negatifle�ti. N�ks g�zlenen 9 hastan�n hepsinde n�ks s�ras�nda anti-dsg-1 ve/veya anti-dsg-3 serum d�zeylerinde art�� saptand�. Dokuz olgunun 3��nde ise klinik remisyon halinde iken n�ksten 1-4 ay �ncesinde serum antikor d�zeylerinde y�kselme tespit edildi.
Sonu�: Bu �al��mada serum desmoglein otoantikor de�erlerinin hastal�k �iddeti ve aktivitesi ile ili�kili olabilece�ini saptad�k. Klinik remisyon esnas�nda desmoglein antikorlar�n�n seri �l��mleri takip ve tedavi modifikasyonunda yol g�sterici olabilir.

Anahtar Kelimeler: Pemfigus vulgaris, pemfigus foliaseus, direkt imm�nfloresan, desmoglein antikorlar�, hastal�k aktivitesi

Mediha Y�lmaz, Emel B�lb�l Ba�kan, Ferah Budak, Hayriye Sar�cao�lu, ��kran Tunal�. Relationship of Serum Levels of Anti-Desmoglein Antibodies and Direct Immunofluorescence Findings with Clinical Activity of Pemphigus. Turkderm-Turk Arch Dermatol Venereol. 2011; 45(2): 77-82

Corresponding Author: Emel B�lb�l Ba�kan, T�rkiye

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