Localized scleroderma (morphea). Diagnostic and therapeutic recommendations of the Polish Dermatological Society

Abstract

Localized scleroderma (morphea) is a chronic autoimmune disease of the connective tissue. Its etiopathogenesis is unknown. Most often the disease affects the skin, but may also involve the subcutis, muscles, and the osteoarticular system. The clinical presentation and the course of disease may be diverse. This article presents clinical characteristics of various types of the disease, the classification of its subtypes, recommendations for differential diagnosis and treatment. STReSzCzenie Twardzina ograniczona (morphea) jest przewlekłą chorobą zapalną tkanki łącznej o podłożu autoimmunologicznym i o niewyjaśnionej etiopatogenezie. Najczęściej zajęta jest skóra, ale proces chorobowy może zajmować tkankę podskórną, mięśnie i układ kostno-stawowy. Zróżnicowany obraz kliniczny choroby wymaga prawidłowego rozpoznania i zastosowania odpowiedniej terapii. W artykule przedstawiono charakterystykę kliniczną oraz zalecenia dotyczące klasyfikacji, postępowania diagnostycznego i leczenia.

The Correction of Facial Morphea Lesions by Hyaluronic Acid: A Case Series and Literature Review

“…Despite the suggestions of developmental origins of morphea (due to frequent pattern of lesions alongside Blaschko's lines), numerous evidence confirms its autoimmune background (the correlation with personal and family history of autoimmune diseases, the presence of human leukocyte antigen (HLA) class II molecules found also in other autoimmune disorders) [1].…”

“…Morphea (localized scleroderma, LoSc) is a chronic inflammatory disease of connective tissue which presents with a wide spectrum of clinical symptoms depending on the activity of the disease, involved region and the depth of the lesions. The lesions most often affect dermis and subcutaneous tissue and show tendency for spontaneous remission, yet causing skin atrophy and dyspigmentation [1]. Less often the process affects deeper tissues (fascia, muscles and bones), leading to deep atrophy and deformations.…”

“…The genetic and environmental factors, as well as microchimerism, are considered to activate keratinocytes to release inflammatory mediators (interleukin-4 (IL-4), IL-6, IL-10, IL-17A, IL-27, interferon-γ), which subsequently stimulate lymphocytes, endothelial cells and fibroblasts. Of particular significance, the activation of endothelial cells and lymphocytes provokes fibroblasts to increase collagen synthesis, which clinically manifests as focal skin hardening and active inflammatory border (lilac ring) [ 1 – 4 ].…”

“…The facial lesions lead to the deformation of the face, lowering patients’ quality of life, especially in adults [ 5 , 6 ]. It is most pronounced in en coup de sabre type, which is characterized by linear atrophy and hardening of the skin, subcutaneous tissue, muscles and bones, usually extending from the upper limit of the eyebrow to the scalp, often with concomitant alopecia and eyebrow loss [ 1 ]. The therapeutic options proposed by consensuses aim at silencing active forms, hindering the progression of the disease, but usually they do not discuss methods of correction of the defects generated by the lesions [ 1 , 7 – 9 ].…”

Laser Therapy for the Treatment of Morphea: A Systematic Review of Literature

“…After several months or years, sclerotic plaques resolve, but skin and/or deeper tissues atrophy and dyspigmentation remain as an atrophic stage. Some severe types, particularly generalized and linear, may be associated with various extracutaneous manifestations [ 5 , 6 ]. It is worth emphasizing that histopathological features mirror the disease phase, although a diagnostic skin biopsy should be taken only in case of an unclear clinical presentation.…”