No abstract

Introduction Eosinophilic fasciitis (EF) is a rare autoimmune disease causing progressive induration of dermal, hypodermal, and muscularis fascia. The exact pathogenesis is yet to be fully understood, and a validated therapy protocol still lacks. We here aimed to realize a clinical-functional characterization of these patients.Materials and methods A total of eight patients (five males, 45 years average) were treated with adjuvant high-dose UVA-1 phototherapy (90 J/cm), after having received the standard systemic immunosuppressive protocol (oral methylprednisolone switched to methotrexate). Body lesion mapping, Localized Scleroderma Assessment Tool (LoSCAT), Dermatology Life Quality Index (DLQI), High-Resolution Ultrasound (HRUS) (13-17MHz), and ultra HRUS (55-70 MHz) were performed at each examination time taking specific anatomical points. Gene expression analysis at a molecular level and in vitro UVA-1 irradiation was realized on lesional fibroblasts primary cultures. ResultsThe LoSCAT and the DLQI showed to decrease significantly starting from the last UVA-1 session. A significant reduction in muscularis fascia thickness (À50% on average) was estimated starting from 3 months after the last UVA-1 session and maintained up to 12 months follow-up. Tissues was detected by HRUS. The UVA-1 in vitro irradiation of lesional skin sites cells appeared not to affect their viability. Molecular genes analysis revealed a significant reduction of IL-1ß and of TGF-ß genes after phototherapy, while MMPs 1,2,9 gene expression was enhanced.Comment These preliminary in vivo and in vitro findings suggest that UVA-1 phototherapy is a safe and useful adjuvant therapy able to elicit anti-inflammatory effects and stimulate tissue matrix digestion and remodeling at lesional sites.

The differential diagnosis of lichenoid dermatoses of the childhood (ie lichen nitidus [LN], lichen spinulosus [LSpi], lichen striatus [LStr], keratosis pilaris [KP]) can be challenging. Many of these uncommon cases remain indeed misdiagnosed, also because of overlapping forms, coexistence of atopic dermatitis or previous inappropriate treatments. [1][2][3][4] Line-field confocal optical coherence tomography (LC-OCT) is a recently developed non-invasive imaging technique able to provide real-time, high-resolution images of the skin up to a 500µm depth, both within the normal 5 and pathological spectrum. [6][7][8][9][10] We examined 10 patients (8 males and 2 females; median age 8.4 [3-16] years) affected by lichenoid dermatoses of the childhood, namely LN (3), LSpi (1), LStr (1) and KP (5). Non-invasive examinations LC-OCT, lichen nitidus, lichen planus, lichen spinulosus, RCM