Assessment of epidermal atrophy in localized scleroderma (morphea)

The skin surface of scleroderma skin becomes glossy as a result of epidermal atrophy. In this study, the skin relief was studied in 12 patients with localized scleroderma (morphea). Measurements by a stylus method showed smoothening of the skin in all plaques studied (p less than 0.01). Ultrasound measurement showed increased skin thickness (p less than 0.01). There was no correlation between smoothening of the skin surface and increase in skin thickness, and no correlation to the duration of the plaques. It is concluded that glossy appearance of plaques of scleroderma is an early sign, which may be useful in the clinical diagnosis of scleroderma.     

系统性硬皮病合并局限性硬皮病六例报告

关于系统性硬皮病与局限性硬皮病的关系,意见尚不一致.一般多认为局限性硬皮病不侵犯内脏,预后良好,而与系统性硬皮病有所不同(1,2).但亦有局限性硬皮病出现内脏病变,可能已从局限转化为系统性.     

Elastic fiber pattern in scleroderma/morphea

Background: Scleroderma/morphea is characterized by expansion of the dermis with thickened collagen bundles and loss of CD34⁺ dermal dendrocytes. Variable elastic fiber changes have been described, but to our knowledge, no systematic study of the elastic fiber pattern correlated with CD34 expression has been reported.
Methods: To better define the typical elastic fiber morphology, we examined seven cases of normal skin and 28 cases of scleroderma/morphea ranging from inflammatory to sclerosing stages. All but four biopsies were submitted with a clinical impression of either scleroderma or morphea. CD34 immunohistochemistry was performed on 26 biopsies with available tissue.
Results: Elastic van Gieson stain showed preservation of elastic fibers in all cases. In addition, straightening with parallel orientation and compression between thickened collagen bundles was frequently present and was graded as limited in 46% and diffuse in 54% of cases. The extent of elastic fiber alteration correlated with the degree of sclerosis. A variable loss of CD34⁺ dermal dendritic cells was seen in all cases.
Conclusion: This study confirms the preservation and frequent presence of parallel, straightened and compressed elastic fibers in scleroderma/morphea and suggests that the elastic fiber pattern, in addition to CD34 immunohistochemistry, may serve as a useful diagnostic adjunct.     

Histological study on localized scleroderma

To define stage-specific and type-specific histological findings in morphea, 21 (14 plaque, 2 linear, and 5 en coup de sabre type) patients were examined. A) Fibrotic changes; 1) Every case had the fibrotic change of various degrees. 2) Nodular sclerotic fibrosis was found in 7 cases with morphea, namely in 5 of 11 cases with a plaque type and 2 of 4 en coup de sabre type morphea. And the above mentioned 7 cases were within two years since the onset, and no nodular fibrosis was found in the morphea with the longer duration than two years in history. Nodular fibrosis was located in the middle or lower dermis adjacent to the typical sclerotic dermis. It was strongly suggested that this nodular fibrosis is as an initial change of localized scleroderma. 3) Nodular fibrosis expanded to the neighboring area and made a typical histological feature of morphea which is completely different from that of diffuse scleroderma. B) Inflammatory findings; 1) Inflammatory changes were divided into, a perivascular, a diffuse or non-perivascular, and a mixed type. 2) The pure perivascular type was found in 10, the pure diffuse type only in one, the mixed type in 6 cases, and the other 4 cases had no inflammation. 3) Marked or moderate inflammation was found in cases with short history of the disease except for one case. 4) Inflammatory cells in the morphea were mainly composed of lymphocytes and histiocytes, but occasionally of plasma cells in 11 of 17 cases. C) Pigmentary changes; Incontinentia pigmenti was found in 18 of 21 cases.     

Systemic and localized scleroderma

Sclerosing conditions of the skin are manifested by a full spectrum of presentations that includes skin-limited forms as well as those which can involve internal organs and result in death. At this point, we are just beginning to understand the mechanisms of tissue fibrosis, and it is likely that the fibrotic processes are a heterogeneous group of disorders in which perturbation of multiple molecular pathways, including vascular and immunologically mediated pathways, can lead to fibrosis. We now have some moderately effective therapies for vascular aspects of systemic sclerosis (eg, bosentan for pulmonary arterial hypertension, calcium-channel blockers for Raynaud's, or angiotensin-converting enzyme inhibitors for renal crisis). We also are beginning to find treatments interrupting the immunologic pathways that manifest as systemic sclerosis (eg, methotrexate for the skin or cyclophosphamide for the lungs). The basic process of fibrosis, however, awaits proven, effective therapy.     

局限性硬皮病发病机制的研究进展

局限性硬皮病是一种以局部皮肤及皮下组织纤维化为特征的疾病.病因不明,发病机制可能涉及小血管的损伤、T细胞的活化以及结缔组织生成增加等多个过程.T细胞活化释放各种细胞因子,趋化因子,主要包括肿瘤坏死因子α、转化生长因子β、可溶性白细胞介素受体2和可溶性白细胞介素受体6等.血管内皮细胞的损伤也可介导纤维化相关的细胞因子释放.局限性硬皮病和系统性硬皮病在发病机制上有一定的区别和联系,概述局限性硬皮病发病机制的最新进展.     

Perineural inflammation in morphea (localized scleroderma): systematic characterization of a poorly recognized but potentially useful histopathological feature

The association between morphea and perineural inflammation has been reported sporadically but never studied systematically. To assess the prevalence and nature of perineural inflammation in various clinicopathologic stages of morphea and a cohort of other inflammatory dermatoses, 80 morphea and 36 control skin biopsies were studied using hematoxylin/eosin and S100 stains. Perineural inflammation was semiquantitatively analyzed (scored), which along with the pattern (concentric vs. marginal) and cellular composition was compared in the two groups. Perineural inflammation was identified in 84% and 61% of morphea and control cases, respectively. Examination of only routinely stained sections could still detect this feature in 58% of morphea and 33% of control biopsies. Mean perineural inflammation score in morphea (0.65) was significantly higher than in the control group (0.23) (p < 0.0001) and the inflammation tended to show a concentric pattern with plasma cell neurotropism. Intraneural inflammation was limited to four morphea cases. Although perineural inflammation is common in morphea, it is not unusual to find this feature in other inflammatory conditions. Nevertheless, perineural inflammation can serve as an important diagnostic adjunct in difficult cases of morphea if one considers its greater intensity, predominantly concentric pattern and the tendency to show plasma cell neurotropism.     

Evidence for spirochetal origin of circumscribed scleroderma (morphea)

Acrodermatitis chronica atrophicans (ACA) and morphea are clinically distinct skin diseases with some common features and possible coexistence. We found antibodies to Borrelia burgdorferi in eight of fifteen patients with morphea. Six of them had IgG antibodies and two both IgG and IgM antibodies. Four of the eight seropositive and five of the seven seronegative patients had been treated with high dose penicillin previously. Spirochetal organisms could be cultured in Barbour-Stoenner-Kelly's medium from a skin biopsy of one seropositive untreated patient. Spirochetes were recovered from histological sections in three of eight, two seropositive and one seronegative morphea and in one of three erythema chronicum migrans patients by an avidin-biotin immunoperoxidase method. The similar clinical picture of ACA and morphea, the response to penicillin therapy in both entities, the presence of antispirochetal antibodies, the isolation of spirochetes in culture and the detection of spirochetal organisms on histological sections suggest a close relationship among these diseases. We conclude that morphea may represent a Borrelia infection. The correlation to ACA is discussed.     

Longitudinal melanonychia in localized scleroderma

Longitudinal melanonychia associated with scleroderma is rare. This is paradoxical, as increased skin pigmentation is frequent in the latter disease. This article reports 4 cases of longitudinal melanonychia that were found concurrently with various types of localized scleroderma. These 4 cases may be a coincidental finding despite the fact that pigmentation is so frequent a manifestation of scleroderma. In 2 cases, biopsy specimen of the nail matrix showed well-defined melanocytes without cellular atypia. This, in keeping with the pigmentation of scleroderma, appears to result from the functional activation of the matrix melanocytes.     

Treatment options for localized scleroderma

Localized scleroderma, or morphea, is a chronic disease that causes a thickening and induration of the skin. For plaque type morphea, the treatments of choice include super-potent corticosteroids and calcipotriol. For the more generalized forms, as well as the linear forms, UVA is currently the best therapeutic modality. Patients with localized scleroderma are managed by both rheumatologists and dermatologists. There is still much therapeutic uncertainty in this disease.