Turkderm-Turk Arch Dermatol Venereol. 2013; 47(4): 209-213 | DOI: 10.4274/turkderm.05657

The clinical features, laboratory findings, treatment and follow-up results of patients with morphea

Nehir Parlak¹, Bengü Nisa Akay², Hatice Erdi Þanlý², Aynur Akyol^2
1Prof. Dr.. Celal Ertuð Etimesgut State Hospital For Skin And Venereal Diseases Clinic, Ankara,
²Ankara University School Of Medicine, Department Of Dermatology, Ankara, Turkey

Objective: Morphea, also known as localized scleroderma, is a rare skin disease of unknown pathogenesis, characterized by fibrosis in the skin and subcutaneous tissue. In this study, we aim to evaluate the demographic features, clinical characteristics, laboratory findings, and response to treatment in patients diagnosed with morphea.
Materials and Methods: The findings of fifty eight patients diagnosed with morphea were retrospectively evaluated between 1995-2011. All patients' clinical symptoms, concomitant diseases, symptoms, immunological features and presence of peripheral eosinophilia were investigated. Treatment methods, response to therapy of 40 patients whose treatment continued for 2-12 months were examined. Fourty nine patients (84.5%) were female and 9 patients (15.5%) were male of 58 patients who were diagnosed with morphea. The mean age of patients was 42.33±18.44 years (range: 7-75 years). Diagnosis was made histopathologically in all cases. Borrelia antibodies were negative in all patients enrolling the study. Thirty six patients (62.1%) had plaque type, 17 patients (29.3%) had generalized type, 3 patients (5.2%) had mixed type (linear + plaque) and 2 patients (3.4%) had linear type of morphea. ANA was found to be positive in 12 (26.2%) of 46 patients. Considering the relationship between the clinical types of morphea with ANA, 38.5% of plaque type, 53.8% of generalized type, 7.7% of mixed type patients showed ANA positivity. ANA positivity was statistically significant in patients with generalized morphea (p=0.027). Peripheral eosinophilia was detected in one case in whom lesions were generalized (2.1%). Colchicine therapy was given to 23 cases. Complete and partial response rates are 47.8% and 26.1%, respectively. However, 17.4% of patients remained stable and progression was noted in 8.7% of the cases.
Conclusion: In conclusion, plaque type morphea is the most common type of morphea. ANA positivity was statistically significant in patients with generalized skin lesions. Colchicine is an effective and safe treatment option.

Keywords: autoantibodies, kolþisin, localized scleroderma, morphea

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Morfealý hastalarda klinik özellikler, laboratuvar bulgularý, seçilen tedavi yöntemi ve takip sonuçlarý

Nehir Parlak¹, Bengü Nisa Akay², Hatice Erdi Þanlý², Aynur Akyol^2
1Prof. Dr. Celal Ertuð Etimesgut Devlet Hastanesi, Deri Ve Zührevi Hastalýklar Kliniði, Ankara, Türkiye
²Ankara Üniversitesi Týp Fakültesi, Deri Ve Zührevi Hastalýklar Ana Bilim Dalý, Ankara, Türkiye

Amaç: Morfea lokalize skleroderma olarak da bilinen, deri ve deri altý dokuda fibrozis ile karakterli, patogenezi bilinmeyen nadir bir deri hastalýðýdýr. Bu çalýþmada, morfea tanýsý alan olgularda, demografik özellikler, klinik seyir, laboratuvar bulgularý ve tedavi yanýtlarýnýn deðerlendirilmesi amaçlanmýþtýr.
Gereç ve Yöntem: Çalýþma 1995-2011 yýllarý arasýnda morfea tanýsý konulan 58 hastanýn retrospektif olarak incelenmesiyle gerçekleþtirilmiþtir. Tüm hastalarýn klinik bulgularý, eþlik eden hastalýklarý, semptomlarý, immünolojik antikor profilleri, periferal eozinofili varlýðý ve tedavi yanýtlarý araþtýrýlmýþtýr. 2-12 ay süre ile tedavisi devam etmiþ olan 40 hastanýn tedavi yöntemleri ve tedaviye yanýtlarý incelenmiþtir.
Bulgular: Morfea tanýsý alan 58 olgunun 49’u (%84.5) kadýn, 9’u (%15.5) erkekti. Hastalarýn yaþ ortalamasý 42.33±18.44 yýl (aralýk: 7-75yýl) idi. Olgularýn tamamýna morfea tanýsý histopatolojik inceleme ile konulurken, borrelia antikorlarý negatif olarak saptandý. Lezyonlar 36 olguda (%62.1) plak tip, 17’sinde (%29.3) generalize tip, 3’ünde (%5.2) miks (lineer + plak) tip, 2’sinde (%3.4) lineer tip morfea olarak sýnýflandýrýldý. ANA seviyesi 46 olgunun 12’sinde (%26.2) pozitif olarak bulundu. Morfeanýn klinik tipleri ile ANA arasýndaki iliþki incelendiðinde plak tipi olanlarýn %38.5’inde, generalize tipi olanlarýn %53.8’inde, miks tip morfealý olgularýn %7.7’sinde ANA pozitifliði saptanmýþtý. Generalize morfeasý olan hastalarda ANA pozitifliðinin anlamlý olduðu görüldü (p=0.027). Anti ds-DNA 1 olguda (%2.7), anti-histon antikor 1 olguda (%1.7) ve anti-ku antikor 3 olguda (%8.6) pozitif olarak saptandý. Periferal eozinofili 1 olguda (%2.1) mevcuttu ve bu olguda lezyonlar generalize idi. Olgularýn 23’üne kolþisin tedavisi verildiði görüldü. Kolþisin tedavisi kullanan hastalarýn %47.8’inde tedaviye komplet yanýt, %26.1’inde parsiyel yanýt alýnýrken, %17.4’ünde hastalýk stabil kalmýþ, %8.7’sinde ise progresyon izlenmiþtir.
Sonuç: Çalýþmamýzýn sonucunda plak tip morfeanýn diðer tiplere oranla daha sýk görüldüðü, generalize tip morfeada ise ANA pozitifliðinin anlamlý olduðu saptanmýþtýr. Kolþisinin tedavide etkili ve güvenilir olduðu ve yeni lezyon çýkýþýný durdurduðu gözlenmiþtir

Anahtar Kelimeler: otoaantikorlar, colchicine, lokalize skleroderma, morfea

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