Association between Localized Scleroderma Cutaneous Assessment Tool and clinicopathologic characteristics in patients with morphea/ Morfeali hastalarda Lokalize Skleroderma Kutanoz Degerlendirme Araci ve klinikopatolojik ozellikler arasindaki iliski

• Published in: Türkderm Vol. 56; no. 3; p. 93
• Main Authors: Urun, Yildiz Gursel, Keskin, Elif Usturali
• Format: Journal Article
• Language: English; Turkish
• Published: Galenos Yayinevi Tic. Ltd 01.09.2022
• Subjects: Development and progression; Physiological aspects; Prognosis; Scleroderma (Disease); Systemic scleroderma; Turkey
• ISSN: 1019-214X; 2651-5164
• DOI: 10.4274/turkderm.galenos.2022.07347

Background and Design: Morphea is also known as localized scleroderma. It is a rare autoimmune skin disease characterized by inflammation and sclerosis in the dermis and sometimes in the subcutaneous tissue. Laboratory findings, imaging, and histopathological features facilitate diagnosis and provide sufficient information about disease severity. Clinicopathologic correlations and severity factors in morphea are poorly described. Thus, this study aimed to review the clinical and histopathological features and treatment responses of patients with morphea and compare these features with disease activity and damage scores to identify new tools for assessing disease severity other than clinical findings. The applicability of the Localized Scleroderma Cutaneous Assessment Tool in clinical practice was also evaluated. Materials and Methods: This study reviewed data of 41 patients who had a histopathologically confirmed diagnosis of morphea and had been followed up regularly for at least 6 months. The modified Localized Scleroderma Skin Severity Index (mLoSSI), Localized Scleroderma Skin Damage Index (LoSDI), Physician Global Assessment-Activity (PGA-A), and Physician Global Assessment-Damage (PGA-D) were calculated at baseline and final treatment. Results: Among morphea subtypes, superficial morphea had significantly more sclerosis in the papillary dermis and plaque-type morphea had significantly more sclerosis in the reticular dermis (p<0.05). When positive antinuclear antibody (ANA) and high levels of thyroid autoantibodies were compared with mLoSSI, LoSDI, PGA-A, and PGA-D scores at baseline, no significant correlation was found. Comparison of the subgroups according to the initial mLoSSI and LoSDI scores revealed no significant histopathological differences between the groups. Conclusion: Although the mLoSSI, LoSDI, PGA-A, and PGA-D scores can be successfully used for the follow-up and treatment of patients with morphea, no correlation was found between positive ANA, high levels of thyroid autoantibodies, and histopathological features. Keywords: Localized scleroderma, morphea, Localized Scleroderma Cutaneous Assessment Tool, pathology, autoantibodies Amac: Lokalize skleroderma olarak da bilinen morfea, dermiste ve bazen subkutan dokuda enflamasyon ve skleroz ile seyreden, nadir gorulen, otoimmun bir deri hastaligidir. Laboratuvar bulgulari, goruntuleme yontemleri ve histopatolojik ozellikler taniyi kolaylastirmakla birlikte hastaligin siddeti hakkinda yeterince bilgi vermemektedir. Hastaligin klinikopatolojik ozellikleri ile siddeti arasindaki iliski nadir olarak gosterilmistir. Bu calismanin amaci morfeali hastalarin klinik ve histopatolojik ozelliklerini ve tedavi yanitlarini gozden gecirmek ve bu ozelliklerle hastalik aktivite ve hasar skorlarini karsilastirmak ve klinik bulgular disinda hastalik siddetini degerlendirmek icin yeni araclar gelistirmektir. Ayrica Lokalize Skleroderma Kutanoz Degerlendirme Aracinin klinik pratikte uygulanabilirligi de degerlendirilmistir. Gerec ve Yontem: Bu calisma, histopatolojik olarak morfea tanisi dogrulanmis, en az alti ay duzenli takip edilen, tedavi baslangicinda ve bitiminde Modifiye Lokalize Skleroderma Deri Siddet Indeksi (mLoSSI), Lokalize Skleroderma Deri Hasar Indeksi (LoSDI), Doktorun Global Degerlendirmesi Aktivitesi (PGA-A) ve Doktorun Global Degerlendirmesi Hasari (PGA-H) hesaplanmis olan 41 hastanin mevcut verileri gozden gecirilerek tasarlanmistir. Bulgular: Morfea alt tipleri arasindaki karsilastirmada, yuzeyel tip morfeali hastalarin papiller dermisinde ve plak tip morfeali hastalarin alt retikuler dermisinde anlamli olarak daha fazla skleroza sahip olduklari goruldu (p<0,05). Antinukleer antikor (ANA) pozitifligi ve troid otoantikorlarinin yukseligi ile tedavi baslangic mLoSSI, LoSDI, PGA-A ve PGA-H skorlari karsilastirildiginda anlamli bir korelasyon bulunamadi. Baslangic mLoSSI ve LoSDI skorlari goz onune alinarak olusturulan alt gruplar arasinda anlamli bir histopatolojik farklilik gozlenmedi. Sonuc: Morfeali hastalarin takip ve tedavisinde mLoSSI, LoSDI, PGA-A ve PGA-D skorlari basarili bir sekilde kullanilabilmekle birlikte, pozitif ANA, yuksek tiroid otoantikor duzeyleri ve histopatolojik ozellikler arasinda bir iliski bulunamadi. Anahtar Kelimeler: Lokalize skleroderma, morfea, Lokalize Skleroderma Kutanoz Degerlendirme Araci, patoloji, otoantikorlar