Clin Exp Dermatol 2017 Sep 19. Epub 2017 Sep 19.

Mansoura Dermatology, Venerology and Leprology Hospital, 5-Amien Alsamanoudy Street, from AbdelsalamAaref Street, Mansoura, Egypt.

Klin Monbl Augenheilkd 2017 Sep 12. Epub 2017 Sep 12.

Zentrum für Augenheilkunde, Universitätsklinikum Essen, Essen.

This article is a review of diagnostic and therapeutic possibilities in common epibulbar malignant tumors, such as basal cell carcinoma, conjunctival lymphoma, squamous cell carcinoma and conjunctival melanoma. Most importantly, for every tumor patient there is a detailed anamnesis, split lamp examination and photo documentation. Further regular controls after therapy are required due to the risk of recurrences. Read More

G Ital Dermatol Venereol 2017 Sep 8. Epub 2017 Sep 8.

Department of Dermatology, University of Rome Tor Vergata, Rome, Italy.

Front Med (Lausanne) 2017 18;4:120. Epub 2017 Aug 18.

Department of Dermatology, University of Cologne, Cologne, Germany.

Sclerosing skin diseases represent a large number of distinct disease entities, which include systemic sclerosis, localized scleroderma, and scleredema adultorum. These pathologies have a common clinical appearance and share histological features. However, the specific interplay between cytokines and growth factors, which activate different mesenchymal cell populations and production of different extracellular matrix components, determines the biomechanical properties of the skin and the clinical features of each disease. Read More

Clin Exp Dermatol 2017 Oct 3;42(7):820-822. Epub 2017 Sep 3.

Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada.

J Am Acad Dermatol 2017 Aug 30. Epub 2017 Aug 30.

Department of Dermatology, Mayo School of Graduate Medical Education, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo School of Graduate Medical Education, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Eosinophilic fasciitis (EF) and morphea profunda (MP) are inflammatory and sclerosing disorders of the subcutis that can exhibit clinical and pathologic presentations that overlap.

Objective: To identify clinicopathologic features that can be used to distinguish EF from MP.

Methods: We performed a retrospective review of 16 patients with EF and 11 patients with MP. Read More

Int J Trichology 2017 Apr-Jun;9(2):92-93

Department of Clinical Research, Fundación Valle del Lili Clinic, Icesi University, Cali, Colombia.

J Eur Acad Dermatol Venereol 2017 Sep 9;31(9):1401-1424. Epub 2017 Aug 9.

Department of Dermatology and Venereology, University Hospital of Cologne, Cologne, Germany.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. Read More

Isr Med Assoc J 2017 Jul;19(7):459-460

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Open Access Maced J Med Sci 2017 Jul 21;5(4):543-544. Epub 2017 Jul 21.

Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior, and Onkoderma Polyclinic for Dermatology and Dermatologic Surgery, Sofia, Bulgaria.

We report the case of a 42 years old male patient suffering from skin changes, which appeared in the last 7-8 years. Two biopsies were performed during the evolution of the lesion. Both showed similar findings that consisted in a busy dermis with interstitial, superficial and deep infiltrates of lymphocytes and histiocytes dispersed among collagen bundles, with variable numbers of neutrophils scattered throughout. Read More

Australas J Dermatol 2017 Aug 3. Epub 2017 Aug 3.

College of Medicine, Chang Gung University, Taoyuan, Taiwan.

Background/objectives: Lymphocytic hidradenitis is a non-specific histopathological feature observed in many dermatoses such as lupus erythematosus, morphea or scleroderma. When it occurs it is usually accompanied by the other distinctive histological features of those conditions. Isolated lymphocytic hidradenitis is uncommon and its clinical features and associated underlying medical conditions are still undetermined. Read More

Ann Dermatol Venereol 2017 Jul 28. Epub 2017 Jul 28.

Service de dermatologie, CHU de Caen, avenue de la Côte-de-Nacre, CS 30001, 14033 Caen cedex 9, France.

Background: Connective tissue nevus (CTN) is a rare condition of the extracellular matrix components that generally presents as papulae of normal skin colour. This condition may be syndromic or sporadic.

Patients And Methods: We report herein two isolated cases of extensive and infiltrative CTN in children at risk for subsequent joint stiffening. Read More

Vaccine 2017 Aug 20;35(35 Pt B):4499-4500. Epub 2017 Jul 20.

Section of Pediatric Dermatology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA 19104, United States.

Multiple groups of patients have been recognized for having high rates of non-responders to the Hepatitis B vaccine including those with HIV, inflammatory bowel disease, and chronic kidney disease. These patients are at increased risk for infection due to both the nature of their underlying diseases and the immunosuppressive drugs they are commonly prescribed. Identification of groups with high non-response rates is essential in order to establish vaccination guidelines and prevent serious infections in already susceptible patients. Read More

J Am Acad Dermatol 2017 Sep 19;77(3):512-517.e5. Epub 2017 Jul 19.

Department of Dermatology, Radboud University, Nijmegen, The Netherlands; Radboud University Medical Centre, Radboud University, Nijmegen, The Netherlands.

Background: Eosinophilic fasciitis (EF) is a connective tissue disease with an unknown long-term course.

Objective: To evaluate presence and determinants of residual disease damage in patients with EF after long-term follow-up.

Methods: Patients with biopsy-proven EF were included for this cross-sectional study. Read More

Australas J Dermatol 2017 Jul 21. Epub 2017 Jul 21.

Department of Dermatology, Medical University of Graz, Graz, Austria.

Br J Dermatol 2017 Jul;177(1):9-10

Department of Dermatology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390-9069, U.S.A.

Clin Rev Allergy Immunol 2017 Jul 16. Epub 2017 Jul 16.

Section of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.

Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. Read More

J Rheumatol 2017 Jul;44(7):1096-1097

Section of Dermatology, The Hospital for Sick Children, and Department of Paediatrics, University of Toronto, Toronto, Ontario, Canada.

Can J Cardiol 2017 Jul 29;33(7):950.e5-950.e6. Epub 2017 Mar 29.

Cardiovascular Department, Santa Corona Hospital, Pietra Ligure (SV), Italy.

A 43-year-old man with systemic sclerosis and chest pain had negative T waves in precordial electrocardiographic leads. The echocardiogram showed a large left ventricular apical accessory chamber. The coronary arteries were normal. Read More

J Dermatol 2017 Jun 30. Epub 2017 Jun 30.

Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan.

Am J Respir Crit Care Med 2017 Jun 27. Epub 2017 Jun 27.

Univerity of Munich-Comprehensive Pneumology Center, Helmholtz Zentrum München, Munich/Neuherberg, Germany ;

Rationale: Analyzing the molecular heterogeneity of different forms of organ fibrosis may reveal common and specific factors and thus identify potential future therapeutic targets.

Objectives: We sought to use proteome-wide profiling of human tissue fibrosis to (1) identify common and specific signatures across endstage interstitial lung disease (ILD) cases, (2) characterize ILD subgroups in an unbiased fashion, and (3) identify common and specific features of lung and skin fibrosis.

Methods: We collected samples of ILD tissue (n=45) and healthy donor controls (n=10), as well as fibrotic skin lesions from localized scleroderma and uninvolved skin (n=6). Read More

PLoS One 2017 26;12(6):e0179917. Epub 2017 Jun 26.

Division of Rheumatology & Immunology, Department of Medicine, Medical University of South Carolina, Charleston, South Carolina, United States of America.

The murine bleomycin (BLM)-induced fibrosis model is the most widely used in systemic sclerosis (SSc) studies. It has been reported that systemic delivery of BLM via continuous diffusion from subcutaneously implanted osmotic minipumps can cause fibrosis of the skin, lungs, and other internal organs. However, the mouse strain, dosage of BLM, administration period, and additional important features differ from one report to the next. Read More

Photodiagnosis Photodyn Ther 2017 Sep 21;19:140-152. Epub 2017 Jun 21.

Wellman Center for Photomedicine, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Dermatology, Harvard Medical School, Boston, MA 02115, USA; Harvard-MIT Division of Health Sciences and Technology, Cambridge, MA 02139, USA. Electronic address:

Photodynamic therapy (PDT) employs a photosensitizer (PS) and visible light in the presence of oxygen, leading to production of cytotoxic reactive oxygen species, which can damage the cellular organelles and cause cell death. In dermatology, PDT has usually taken the form of topical application of a precursor in the heme biosynthesis pathway, called 5-aminolevulinic acid (or its methyl ester), so that an active PS, protoporphyrin IX accumulates in the skin. As PDT enhances dermal remodeling and resolves chronic inflamation, it has been used to treat cutaneous disorders include actinic keratoses, acne, viral warts, skin rejuvenation, psoriasis, localized scleroderma, some non-melanoma skin cancers and port-wine stains. Read More

Hautarzt 2017 Jul;68(7):553-559

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

Curr Rheumatol Rev 2017 06 11. Epub 2017 Jun 11.

Fortis Escorts Hospital, Jaipur, India

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

J Dermatolog Treat 2017 Jun 29. Epub 2017 Jun 29.

b Department of Dermatology, Wake Forest School of Medicine , Winston Salem , NC , USA.

Background: En coup de sabre is a rare subtype of linear morphea, located on the forehead or frontoparietal scalp. Systemic treatment of localised 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

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

Pediatr Dermatol 2017 Jul 23;34(4):e174-e178. 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

Pediatr Dermatol 2017 Jul 23;34(4):e164-e167. 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

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

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

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

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

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

Br J Dermatol 2017 May 6. Epub 2017 May 6.

Dermatology Department, Nancy University Hospital, Vandoeuvre-les-Nancy, France.

Acrodermatitis chronica atrophicans (ACA) is the late cutaneous form of Lyme borreliosis. The early inflammatory phase manifests with a bluish-red discoloration and doughy swelling of the skin. The atrophic phase represents a late-phase process with red discoloration, and a thin and wrinkled appearance of the skin. Read More

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

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

J Rheumatol 2017 May;44(5):711-712

Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

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

J Invest Dermatol 2017 Aug 24;137(8):1663-1670. 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

J Invest Dermatol 2017 Aug 19;137(8):1671-1681. Epub 2017 Apr 19.

Arthritis Center, Boston University School of Medicine, Boston, Massachusetts, USA. Electronic address:

Systemic sclerosis (SSc) is a multi-organ fibrotic disease with few treatment options. Activated fibroblasts are the key effector cells in SSc responsible for the excessive production of collagen and the development of fibrosis. Platelet-derived growth factor (PDGF), a potent mitogen for cells of mesenchymal origin, has been implicated in the activation of SSc fibroblasts. Read More

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

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

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

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

Am J Clin Dermatol 2017 Aug;18(4):491-512

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

Hautarzt 2017 Jul;68(7):566-570

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

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

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