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    [Granulomatous reaction pattern of the skin : Interstitial granulomatous dermatitis - lymphoma - vasculitis].
    Hautarzt 2017 Jun 12. Epub 2017 Jun 12.
    Konsultations- und Referenzzentrum für Vaskulitis-Diagnostik, MVZ am Marienkrankenhaus gGmbH, Hamburg, Deutschland.
    A particular diagnostic situation in the classification of a granulomatous dermatitis results when no circumscribed granulomas but instead a diffuse interstitial histiocytic inflammatory infiltrate, a granulomatous vasculitis or a neoplastic lymphocytic infiltrate is found. Interstitial granulomatous dermatitis was originally described in patients with arthritis. Later, it was recognized that there are also associations with other usually immunological diseases. Read More

    Scleroderma-like Disorders.
    Curr Rheumatol Rev 2017 Jun 11. Epub 2017 Jun 11.
    Fortis Escorts Hospital. Bulgaria.
    Scleroderma is a term used to describe diseases that involve hardening and tightening of the skin and the underlying subcutaneous connective tissue. It could be localized to skin and subcutaneous tissue, or may involve the internal organs too in systemic sclerosis. There are disorders that can cause hardening and tightening of skin and mimic scleroderma but are rarely associated with Raynaud phenomenon, sclerodactyly, and autoantibodies in the serum, features specific to scleroderma/systemic sclerosis. Read More

    The efficacy of methotrexate in the treatment of En coup de sabre (linear morphea subtype).
    J Dermatolog Treat 2017 Jun 12:1-8. Epub 2017 Jun 12.
    b Department of Dermatology , Wake forest school of medicine , 4618 country club road Winston Salem , North Carolina 27104.
    Background: En coup de sabre is a rare subtype of linear morphea, located on the forehead or frontoparietal scalp. Systemic treatment of localized morphea with methotrexate has been reported in a few clinical reports. However, there are no case series using methotrexate treatment for En coup de sabre. Read More

    Periostin in Mature Stage Localized Scleroderma.
    Ann Dermatol 2017 Jun 11;29(3):268-275. Epub 2017 May 11.
    Department of Dermatology, SMG-SNU Boramae Medical Center, Seoul, Korea.
    Background: Periostin is a novel matricellular protein expressed in many tissues, including bone, periodontal ligament, and skin. Although its expression is prominent in various fibrotic conditions, studies of periostin in localized scleroderma are rare.

    Objective: To investigate the expression of periostin and other molecules in localized scleroderma. Read More

    Thrombocytopenia Associated with Localized Scleroderma: Report of Four Pediatric Cases and Review of the Literature.
    Pediatr Dermatol 2017 May 23. Epub 2017 May 23.
    Department of Paediatrics, Advanced Pediatrics Centre, Chandigarh, India.
    We report on four children with localized scleroderma (morphea) and thrombocytopenia. All four had the en coup de sabre subtype of morphea and had varying degrees of thrombocytopenia (8 × 10(9) /L to 120 × 10(9) /L). None of them had major bleeding manifestations, and thrombocytopenia resolved with treatment of morphea. Read More

    Metastatic Squamous Cell Carcinoma in a Patient with Disabling Pansclerotic Morphea of Childhood.
    Pediatr Dermatol 2017 May 23. Epub 2017 May 23.
    Department of Dermatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
    Disabling pansclerotic morphea of childhood (DPMC) is a rare disorder that confers a risk of developing ulcer-related squamous cell carcinoma (SCC). We describe a young man with long-standing DPMC and SCC with lung metastasis. Read More

    Bullous morphoea: a retrospective study.
    Clin Exp Dermatol 2017 Jul 22;42(5):532-535. Epub 2017 May 22.
    Section of Dermatology, Mario Aresu Department of Medical Science, University of Cagliari, Cagliari, Italy.
    Bullous morphoea is a rare variant of localized scleroderma whose pathogenesis has been widely discussed. We retrospectively reviewed the records of all histopathologically confirmed cases of morphoea followed from 2005 to 2015 at the Dermatology Clinic and Pathology Institute of the University of Cagliari, Sardinia, Italy. Among 137 patients with morphoea, 2 cases of the bullous variant were identified, which were successfully treated with methotrexate. Read More

    Superficial morphea: case report, look-alikes, pathogenesis, and treatment.
    Dermatol Online J 2017 May 15;23(5). Epub 2017 May 15.
    University of Vermont College of Medicine, Burlington, Vermont University of Vermont Medical Center, Burlington, Vermont.
    Superficial morphea, a rare variant of morphea, is characterized by hypopigmented to hyperpigmented skin lesions located predominantly in a symmetric fashion at intertriginous sites. These patches and plaques typically lack the significant induration, contractures, and atrophy seen in other subtypes of morphea. Histologic examination is key for accurate diagnosis considering the number of similar conditions which may clinically mimic superficial morphea. Read More

    Methotrexate: an effective monotherapy for refractory generalized morphea.
    Clin Cosmet Investig Dermatol 2017 8;10:165-169. Epub 2017 May 8.
    Department of Dermatology and Venereology, Andreas Syggros Hospital, University of Athens, Athens, Greece.
    Introduction: Morphea is an inflammatory skin disorder characterized by excessive collagen deposition. Although treatment algorithms for morphea subtypes have been suggested, no consistent recommendations are available. This study attempts to evaluate the clinical efficacy of methotrexate (MTX) as monotherapy in refractory generalized morphea. Read More

    Systemic sclerosis associated with colliquative necrosis in the cerebellum.
    Surg Neurol Int 2017 5;8:44. Epub 2017 Apr 5.
    Department of Life, Health & Environmental Sciences (MESVA), University of L'Aquila, Italy.
    Background: The scleroderma is a complex autoimmune collagen disorder that can affect many organs simultaneously, as it occurs in the systemic sclerosis (SS), or only the skin, as it occurs in the localized scleroderma (LS). The neurological presentation is extremely uncommon, and even more uncommon are the symptoms of the scleroderma in the cerebellum.

    Case Description: We report the case of a 56-year-old male with cerebellar lesions mimicking a brain abscess. Read More

    Histopathologic Spectrum of Connective Tissue Diseases Commonly Affecting the Skin.
    Surg Pathol Clin 2017 Jun 14;10(2):477-503. Epub 2017 Mar 14.
    Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Amory-3, 75 Francis Street, Boston, MA 02115, USA.
    Connective tissue disorders (CTDs), also known as collagen vascular diseases, are a heterogeneous group of diseases with a common pathogenic mechanism: autoimmunity. Precise classification of CTDs requires clinical, serologic, and pathologic correlation and may be difficult because of overlapping clinical and histologic features. The main contribution of histopathology in the diagnosis of these disorders is to confirm, rule out, or alert clinicians to the possibility of CTD as a disease category, rather than producing definitive diagnoses of specific entities. Read More

    Nodular Sclerodermatous Chronic Cutaneous Graft-Versus-Host Disease (GvHD): A New Clinicopathological Variant of Cutaneous Sclerodermatous GvHD Resembling Nodular/Keloidal Scleroderma.
    Am J Dermatopathol 2017 Apr 25. Epub 2017 Apr 25.
    *Department of Dermatology, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain;†Dermatopathology Research Unit, Department of Dermatology, Medical University of Graz, Graz, Austria; and‡School of Pathology, University of Milan, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy.
    Cutaneous chronic graft-versus-host disease (GvHD) has a broad spectrum of clinicopathological presentations, the most common ones being poikiloderma, lichen planus-like eruptions, lichen sclerosus-like lesions, morphea-like plaques, and deep sclerosis. New forms of chronic cutaneous GvHD with different clinicopathological characteristics have been described, most of them mimicking cutaneous manifestations of autoimmune diseases. We report the case of a 35-year-old man who underwent allogenic stem cell transplantation for a therapy-associated acute myeloid leukemia and developed an acute GvHD with involvement of skin and gastrointestinal tract. Read More

    Scleroderma Mimickers.
    Curr Treatm Opt Rheumatol 2016 Mar 5;2(1):69-84. Epub 2016 Feb 5.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
    Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated. Read More

    Quantitative Assessment of Skin Stiffness in Localized Scleroderma Using Ultrasound Shear-Wave Elastography.
    Ultrasound Med Biol 2017 Jul 28;43(7):1339-1347. Epub 2017 Apr 28.
    Department of Ultrasound, West China Hospital of Sichuan University, Chengdu, China. Electronic address:
    The purpose of this study was to evaluate the usefulness of ultrasound shear-wave elastography (US-SWE) in characterization of localized scleroderma (LS), as well as in the disease staging. A total of 21 patients with 37 LS lesions were enrolled in this study. The pathologic stage (edema, sclerosis or atrophy) of the lesions was characterized by pathologic examination. Read More

    Transcriptional and Cytokine Profiles Identify CXCL9 as a Biomarker of Disease Activity in Morphea.
    J Invest Dermatol 2017 Apr 24. Epub 2017 Apr 24.
    University of Texas Southwestern Medical Center, Department of Dermatology, Dallas, Texas, USA. Electronic address:
    IFN-related pathways have not been studied in morphea, and biomarkers are needed. We sought to characterize morphea serum cytokine imbalance and IFN-related gene expression in blood and skin to address this gap by performing a case-control study of 87 participants with morphea and 26 healthy control subjects. We used multiplexed immunoassays to determine serum cytokine concentrations, performed transcriptional profiling of whole blood and lesional morphea skin, and used double-staining immunohistochemistry to determine the cutaneous cellular source of CXCL9. Read More

    Primary Sjögren's syndrome in patients with celiac disease.
    Turk J Med Sci 2017 Apr 18;47(2):430-434. Epub 2017 Apr 18.
    Division of Rheumatology and Immunology, Department of Internal Medicine, Akdeniz University Faculty of Medicine, Antalya, Turkey.
    Background/aim: Many autoimmune diseases occur concomitantly with celiac disease (CD). We aimed to determine the frequency of Sjögren's syndrome (SS) in CD patients based on SS-specific serology verified by minor labial salivary biopsy.

    Materials And Methods: Eight-two patients with CD were included in the study. Read More

    Th1- and Th17-polarized immune infiltrates in eosinophilic fasciitis-A potential marker for histopathologic distinction from morphea.
    J Cutan Pathol 2017 Jun;44(6):548-552
    Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
    Background: Morphea (localized scleroderma) and eosinophilic fasciitis (EF) are rare fibrosing disorders which may present a diagnostic challenge. While histopathologic features are often distinct, in some cases there may be overlap. T-cells contribute to etiopathogenesis of both autoimmune conditions. Read More

    Erythema multiforme following pneumococcal vaccination.
    Acta Dermatovenerol Alp Pannonica Adriat 2017 Mar;26(1):25-26
    Department of Dermatology, University of Patras, Patras, Greece.
    Erythema multiforme (EM) is an acute and usually self-limited immune-mediated mucocutaneous disorder that is a hypersensitivity reaction to drugs, infections, and vaccines. Clinically, it is characterized by maculopapular, target-like lesions symmetrically distributed on the extremities (minor form) or additionally affecting one or more mucous membranes and causing epidermal detachment involving < 10% of the total body surface area (major form). We report a novel association between pneumococcal vaccination and the development of EM in a 2. Read More

    Pregnancy-associated morphea: a case report and literature review.
    Dermatol Online J 2017 Jan 15;23(1). Epub 2017 Jan 15.
    Department of Internal Medicine, Saint Vincent Hospital, Worcester, MA.
    Morphea, also known as localized scleroderma, is arare fibrosing disorder of the skin, the pathogenesisof which is incompletely understood. It is thought,however, to involve interplay of genetic dispositionand triggering environmental factors, such asinfections and autoimmunity. Pregnancy as a potentialtrigger has only been reported in four cases. Read More

    Morphea and Eosinophilic Fasciitis: An Update.
    Am J Clin Dermatol 2017 Mar 16. Epub 2017 Mar 16.
    Department of Dermatology, Radboud University Medical Centre, Nijmegen, The Netherlands.
    Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. Read More

    [Generalized circumscribed scleroderma with blisters].
    Hautarzt 2017 Mar 16. Epub 2017 Mar 16.
    Klinik für Dermatologie, Allergologie und Phlebologie, Klinikum Bremerhaven Reinkenheide, Postbrookstr. 103, 27574, Bremerhaven, Deutschland.
    The patient suffered from a 20-year course of generalized circumscribed scleroderma and presented with blisters in circumscribed areas of the affected skin. The development of subepidermal blisters has been described in all clinical forms of circumscribed scleroderma. Aetiology and pathogenesis of blister formation have not yet been clarified. Read More

    Histopathological changes in morphea and their clinical correlates: Results from the Morphea in Adults and Children Cohort V.
    J Am Acad Dermatol 2017 Jun 9;76(6):1124-1130. Epub 2017 Mar 9.
    Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address:
    Background: Histopathological features in morphea (localized scleroderma) and their clinical correlates are poorly described.

    Objective: We sought to systematically describe histologic changes of morphea in a large, well-annotated cohort and determine the association between histopathology and clinical features.

    Methods: This was a cross-sectional study of 83 patients enrolled in the Morphea in Adults and Children cohort. Read More

    Localized scleroderma: clinical and epidemiological features with emphasis on adulthood- versus childhood-onset disease differences.
    J Eur Acad Dermatol Venereol 2017 Mar 8. Epub 2017 Mar 8.
    Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
    Background: Localized scleroderma is a rare inflammatory skin disorder that affects the skin and sometimes underlying subcutaneous tissue, muscles or bones. The disease has two modes of onset: juvenile- (JLS) and adult-onset (aLoS). Clinical features have impact on diagnostic and treatment recommendations, but no consensus on the disease management depending on the age at diagnosis was given. Read More

    Increased expression of latent TGF-β-binding protein 4 affects the fibrotic process in scleroderma by TGF-β/SMAD signaling.
    Lab Invest 2017 May 6;97(5):591-601. Epub 2017 Mar 6.
    Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China.
    Scleroderma is a fibrosis-related disorder characterized by cutaneous and internal organ fibrosis, and excessive collagen deposition in extracellular matrix (ECM) is a major cause of fibrosis. Transforming growth factor-β (TGF-β)/SMAD signaling has a central role in the pathogenesis of fibrosis by inducing abnormal collagen accumulation in ECM, and latent TGF-β-binding protein 4 (LTBP-4) affects the secretion of latent TGF-β to ECM. A previous study indicated that bleomycin (BLM) treatment increased LTBP-4 expression in lung fibroblasts of Thy-1 knockout mice with lung fibrosis, and LTBP-4 further promoted TGF-β bioavailability as well as SMAD3 phosphorylation. Read More

    Clinical utility of hypo- and hyperpigmentation of skin in diffuse cutaneous systemic sclerosis.
    Int J Rheum Dis 2017 Mar 6. Epub 2017 Mar 6.
    Rheumatology Department, Waikato Hospital, Hamilton, New Zealand.
    Aim: Cutaneous involvement is an early manifestation of systemic sclerosis (SSc). Localized areas of 'salt and pepper skin' (S&P) may develop. We hypothesize that S&P skin occurs frequently in diffuse cutaneous (dc) SSc which can be used in its early diagnosis and may correlate with joint contractures. Read More

    Human endogenous retroviruses and chosen disease parameters in morphea.
    Postepy Dermatol Alergol 2017 Feb 7;34(1):47-51. Epub 2017 Feb 7.
    Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.
    Introduction: Morphea (localized scleroderma) is a relatively rare disease characterized by excessive skin fibrosis. Human endogenous retroviruses (HERV) are largely distributed within the human genome with hundreds of thousands of elements. The HERV have been widely studied in autoimmune disorders, yet hardly ever assessed in diseases with a good prognosis such as morphea. Read More

    [Interferon in combined therapy of human papillomavirus infections associated with sexually transmitted infections. linical observation].
    Urologiia 2016 Nov(5):103-108
    Moscow Regional Clinical Center of Skin and Sexually-Transmitted Diseases, Moscow.
    To investigate the effectiveness of Ingaron (interferon-) in the treatment of HPV infection associated with sexually transmitted infections, the authors analyzed the scientific literature on the association of human papillomavirus infection with other viral and microbial pathogens. A clinical case of the association of human papillomavirus infection, urogenital infections (urogenital chlamydia and genital herpes) and localized scleroderma penis is described. The results of integrated therapy of diseases with the help of Interferon-gamma have been presented. Read More

    Successful infliximab therapy in a patient with comorbid spondyloarthritis, primary biliary cirrhosis and generalized morphea.
    Acta Clin Belg 2017 Feb 23:1-4. Epub 2017 Feb 23.
    a Faculty of Medicine, Department of Physical Medicine and Rehabilitation , Canakkale Onsekiz Mart University , Canakkale , Turkey.
    The patient in this report was diagnosed simultaneously with primary biliary cirrhosis (PBC), spondyloarthritis, and generalized morphea and was started on infliximab therapy. In addition to an improvement in clinical symptoms with this therapy, an improvement was also observed in laboratory parameters such as cholestatic enzymes, C-reactive protein, and erythrocyte sedimentation rate. Infliximab was well tolerated in this 56-year-old patient. Read More

    Multiplex assessment of serum cytokine and chemokine levels in idiopathic morphea and vitamin K1-induced morphea.
    Clin Rheumatol 2017 May 20;36(5):1173-1178. Epub 2017 Feb 20.
    Scleroderma Center and Jefferson Institute of Molecular Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
    The levels of 63 cytokines, chemokines, and growth factors were measured in the serum of four patients with idiopathic morphea and of one patient with vitamin K1-induced morphea employing a multiplex assay to identify the role of inflammatory/immunologic events in their pathogenesis. Full-thickness skin biopsies of affected skin were analyzed by histopathology. Luminex assays for 63 cytokines, chemokines, and growth factors were performed in the sera from four patients with idiopathic morphea and in two different samples of serum obtained in two separate occasions from one patient with vitamin K1-induced morphea. Read More

    Morphea "en coup de sabre": An unusual oral presentation.
    J Clin Exp Dent 2017 Feb 1;9(2):e315-e318. Epub 2017 Feb 1.
    DDS, MSc, Department of Oral Medicine and Oral Pathology, Universidad Andres Bello, Viña del Mar, Chile. Avenida Valparaíso 1560, Viña del Mar, Chile.
    Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Oral involvement is unusual and it may produce white linear fibrotic areas with a scar-like appearance, atrophy of tongue papillae, gingival recession and alveolar bone resorption. We report a case of a 13-year-old girl who consulted for progressive recession on the attached gingiva of her upper left incisors. Read More

    Blockade of p38 Mitogen-Activated Protein Kinase Inhibits Murine Sclerodermatous Chronic Graft-versus-Host Disease.
    Am J Pathol 2017 Apr 9;187(4):841-850. Epub 2017 Feb 9.
    Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
    Bone marrow transplantation (BMT) of B10.D2 mice into sublethally irradiated BALB/c mice across minor histocompatibility loci is a well-established animal model for human sclerodermatous chronic graft-versus-host disease (Scl-cGVHD) and systemic sclerosis (SSc). The p38 mitogen-activated protein kinase (MAPK) pathway is a key regulator of inflammation and cytokine production. Read More

    Pharmacological inhibition of porcupine induces regression of experimental skin fibrosis by targeting Wnt signalling.
    Ann Rheum Dis 2016 Apr 2;76(4):773-778. Epub 2017 Feb 2.
    Department of Medicine 3 for Rheumatology and Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany.
    Objectives: Wnt signalling has been implicated in activating a fibrogenic programme in fibroblasts in systemic sclerosis (SSc). Porcupine is an O-acyltransferase required for secretion of Wnt proteins in mammals. Here, we aimed to evaluate the antifibrotic effects of pharmacological inhibition of porcupine in preclinical models of SSc. Read More

    Sclerosing Dermatofibrosarcoma Protuberans Shows Significant Overlap With Sclerotic Fibroma in Both Routine and Immunohistochemical Analysis: A Potential Diagnostic Pitfall.
    Am J Dermatopathol 2017 Feb;39(2):83-88
    Departments of *Dermatology, and †Pathology, University of California San Francisco, San Francisco, CA.
    Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low-to-intermediate grade sarcoma with several histologic variants, including pigmented (Bednar tumor), sclerosing, myxoid, atrophic, and DFSP with fibrosarcomatous changes. Two patterns of sclerosis in DFSP can be observed, a sclerotic fibroma-like pattern and a morphea/lichen sclerosus-like pattern. Partial biopsies of sclerosing DFSPs with the sclerotic fibroma pattern can be misdiagnosed as sclerotic fibroma or other benign sclerosing tumors (eg, perineurioma, dermatofibroma). Read More

    Effects of UVA1 Phototherapy on Expression of Human Endogenous Retroviral Sequence (HERV)-K10 gag in Morphea: A Preliminary Study.
    Med Sci Monit 2017 Jan 28;23:505-512. Epub 2017 Jan 28.
    Department of Dermatology, Poznań University of Medical Sciences, Poznań, Poland.
    BACKGROUND Morphea, also known as localized scleroderma, is a rare autoimmune connective tissue disease characterized by skin fibrosis. UVA1 phototherapy is an important asset in the reduction of clinical manifestations in morphea. There are studies claiming that UV light modulates the expression of some human endogenous retroviral sequences. Read More

    Panniculitis: A summary.
    Semin Diagn Pathol 2017 May 27;34(3):261-272. Epub 2016 Dec 27.
    Section of Dermatopathology,Dermatopathology, Division of Surgical Pathology & Cytopathology, University of Virginia Medical Center, Charlottesville, VA, USA. Electronic address:
    The diagnosis of panniculitis is felt to be a confusing topic by some pathologists. This summarical article presents inflammatory diseases of the subcutis in a systematic fashion, based on whether they are centered on fibrovascular septa or the adipose lobules, and whether morphologic vasculitis is present or not. Septocentric, non-vasculitis disorders include erythema nodosum, panniculitis that follows the use of "biological" therapeutic agents, lipodermatosclerosis, post-irradiation panniculitis, morphea profunda, and necrobiosis lipodica profunda. Read More

    Pseudohypertension-Like Presentation: An Exceptionally Rare Presentation in an Athletic Female Patient with Morphea.
    Case Rep Dermatol Med 2016 29;2016:7027352. Epub 2016 Dec 29.
    Novel Psychoactive Substances Research Unit, University of Hertfordshire Doctoral College, Hertfordshire University, Hatfield, UK; Faculty of Medicine, University of Baghdad, Baghdad, Iraq.
    Introduction. Pseudohypertension is a condition which mainly occurs due to thickening-calcification of tunica intima of the arterial wall, leading to a faulty measurement of the intra-arterial blood pressure. To the best of our knowledge, this is the first case report in literature, of a pseudohypertension-like presentation in association with Morphea en plaque. Read More

    Musculoskeletal MRI findings of juvenile localized scleroderma.
    Pediatr Radiol 2017 Apr 14;47(4):442-449. Epub 2017 Jan 14.
    Nemours Children's Health System/Alfred I. duPont Hospital or Children, 1600 Rockland Road, Wilmington, DE, 19803, USA.
    Background: Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes.

    Objective: There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. Read More

    Morphoea with prominent plasma cell endoneuritis.
    Clin Exp Dermatol 2017 Mar 4;42(2):196-199. Epub 2017 Jan 4.
    Department of Dermatopathology, St Thomas' Hospital, London, UK.
    Morphoea (localized scleroderma) is a cutaneous inflammatory condition characterized by the development of indurated and discoloured plaques. The histological features of morphoea typically include a superficial and deep perivascular and periadnexal chronic inflammatory infiltrate associated with variable degrees of dermal and/or subcutaneous sclerosis. The infiltrate is typically composed of lymphocytes, macrophages and conspicuous plasma cells. Read More

    Retinoic acid for treatment of systemic sclerosis and morphea: A literature review.
    Dermatol Ther 2017 Mar 29;30(2). Epub 2016 Dec 29.
    University of California, Los Angeles, Los Angeles, California, 90095.
    Systemic sclerosis and morphea are connective tissue diseases characterized by tightening, thickening, and hardening of the skin, leading to significant morbidity. Unfortunately, current treatment options have limited efficacy for many patients. Cutaneous manifestations of these diseases arise from excess collagen deposition and fibrosis in the skin, through pathogenic mechanisms which have yet to be extensively detailed at the causal immune and cellular levels. Read More

    Specific skin signs as a cutaneous marker of diabetes mellitus and the prediabetic state - a systematic review.
    Dan Med J 2017 Jan;64(1)
    Introduction: Diabetes mellitus and the prediabetic state are associated with a number of skin manifestations. This study is a systematic review of the following manifestations: acanthosis nigricans (AN), skin tags (ST), diabetic dermopathy (DD), rubeosis faciei (RF), pruritus (PR), granuloma annulare (GA), necrobiosis lipoidica (NL), scleroedema diabeticorum (SD) and bullosis diabeticorum (BD). These conditions possibly relate to underlying diabetogenic mechanisms. Read More

    Primary cutaneous anaplastic large cell lymphoma masquerading as large pyogenic granuloma.
    Indian Dermatol Online J 2016 Nov-Dec;7(6):526-528
    Department of Dermatology, Venereology and Leprosy, IGMC, Shimla, Himachal Pradesh, India.
    Primary cutaneous anaplastic large cell lymphoma (pcALCL) forms 9% of the cutaneous T-cell lymphomas. It usually presents as solitary reddish brown ulcerating nodule or indurated plaque. Sometimes, it mimics other dermatological diseases such as eczema, pyoderma gangrenosum, pyogenic granuloma, morphea, and squamous cell carcinoma. Read More

    Autoimmune collagen vascular diseases: Kids are not just little people.
    Clin Dermatol 2016 Nov - Dec;34(6):678-689. Epub 2016 Jul 9.
    Assistant Professor of Dermatology, University of Connecticut School of Medicine, Farmington, CT.
    Morphea, dermatomyositis (DM), and discoid lupus erythematosus (DLE) are autoimmune collagen vascular diseases that can present at any age. In all three of these diseases, the tenants of diagnosis and treatment are largely the same in both children and adults, with a few notable differences. Children with morphea are more likely to present with the linear subtype and have a higher incidence of extracutaneous manifestations. Read More

    The role of local temperature and other clinical characteristics of localized scleroderma as markers of disease activity.
    Int J Dermatol 2017 Jan;56(1):63-67
    Department of Pediatric Medicine, Dermatology Section, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
    Background: Determining the activity of localized scleroderma (LS) remains crucial for decision making, and reliable clinical indicators of activity are lacking. Our objective in this study was to analyze the utility of infrared thermography (IRT) in assessing the activity of LS according to existing clinical scales. A secondary objective was to study whether clinical characteristics of patients and/or IRT have any role in predicting the activity of LS. Read More

    Acral manifestations of paraneoplastic and collagen vascular diseases.
    Clin Dermatol 2017 Jan - Feb;35(1):50-54. Epub 2016 Sep 10.
    Department of Dermatology, Akdeniz University, Faculty of Medicine, Antalya, Turkey. Electronic address:
    The skin often signals a number of systemic disease, making skin findings of paramount significance. Paraneoplastic diseases and collagen vascular diseases are vitally important illnesses. Paraneoplastic diseases and collagen vascular diseases may also occur with many different acral skin findings. Read More

    Lacrimal Drainage System Involvement in Linear Scleroderma.
    Ophthal Plast Reconstr Surg 2016 Dec 7. Epub 2016 Dec 7.
    Govindram Seksaria Institute of Dacryology, L.V. Prasad Eye Institute, Hyderabad-34, India.
    Linear scleroderma is a localized variety of scleroderma characterized by fibrotic areas of the dermis involving head region without systemic features. Ocular involvement has been sparsely reported in the form of episcleritis, dry eye, and uveitis. We describe a 42-year-old man with linear scleroderma, en coup de sabre type with associated nasolacrimal duct obstruction and prolonged dacryocystitis. Read More

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