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Pediatr Dermatol 2019 Apr 17. Epub 2019 Apr 17.

University of Vermont Larner College of Medicine, Burlington, Vermont.

Lichen aureus is a pigmented purpuric dermatosis that tends to occur on the lower extremities, and linear morphea is an inflammatory disease of the dermis and subcutaneous fat that leads to a scarlike induration. We present a pediatric case of linear morphea developing in the same anatomic distribution as prior lichen aureus. Due to the possible progression of lichen aureus to morphea described herein and the difficulty in diagnosing early morphea, close follow-up may be considered for pediatric patients who present with this pigmented purpuric dermatosis. Read More

Dermatol Ther 2019 Apr 9:e12896. Epub 2019 Apr 9.

Dermatology Hospital, Southern Medical University, Guangzhou, China.

Morphea Profunda (MP) is a variant of Morphea that presents clinically as localized fbrotic plaques. Based on previous reports, there was no unified standard treatment for MP. Here we report a rare case of MP, which is the first report about treatment by hydroxychloroquine in combination with superficial X-ray. Read More

JAAD Case Rep 2019 Apr 25;5(4):300-302. Epub 2019 Mar 25.

Division of Dermatology, Department of Medicine, University of Calgary, Calgary, Canada.

J Invest Dermatol 2019 Mar 13. Epub 2019 Mar 13.

Institute of Biochemistry, Emil-Fischer-Center, Friedrich-Alexander University of Erlangen-Nürnberg, Erlangen, Germany; Comprehensive Cancer Center (CCC) Erlangen-EMN, Erlangen, Germany. Electronic address:

Bone morphogenetic protein-6 (BMP6) is known to be crucial for regulating embryonic skin development. This study assessed the role of BMP6 in dermal fibrosis. We detected that BMP6 is significantly increased in skin-derived fibroblasts of patients with localized scleroderma. Read More

Arch Argent Pediatr 2019 Apr;117(2):e137-e141

Servicio de Dermatología, Hospital Universitario de Cabueñes, Gijón,

Solitary morphea profunda is an unusual form of localized scleroderma in childhood. It is characterized by a single, poorly defined and indurate plaque often located on the upper trunk near the spine. Solitary morphea profunda is frequently asymptomatic and shows no associated systemic involvement. Read More

Clin Case Rep 2019 Feb 18;7(2):258-263. Epub 2018 Dec 18.

Division of Dermatology McGill University Health Centre Montreal Québec Canada.

Idiopathic Atrophoderma of Pasini and Pierini should be considered on the differential in a patient presenting with an asymptomatic atrophic plaque on the skin. Differentiation from Linear Atrophoderma of Moulin and morphea remains a challenge; however, features of the presentation and tissue biopsy can help establish the diagnosis. Read More

Rheumatol Int 2019 Mar 5. Epub 2019 Mar 5.

Paediatric Rheumatology Unit, The Royal Children's Hospital, Parkville, VIC, Australia.

Disabling pansclerotic morphea of childhood (DPMC) is a rare subtype of juvenile localized scleroderma (JLS) characterized by pansclerosis mainly affecting children under the age of 14. This aggressive disease has a poor prognosis due to the rapid progression of deep musculoskeletal atrophy resulting in cutaneous ulceration and severe joint contractures. We describe the challenges in treating a previously well 5-year-old male who has refractory symptoms of DPMC. Read More

Acta Derm Venereol 2019 02 27. Epub 2019 Feb 27.

Reference Centre of Rare Skin Diseases, Larrey Hospital, Paul Sabatier University, 31400 Toulouse, France.

Methotrexate has demonstrated its efficiency for the treatment of juvenile localized scleroderma but some patients may be resistant. The aim of our study was to define the profile of such patients. We performed an observational retrospective multicenter study between 2007 and 2016 and included all children seen in the French Paediatric Dermatology and Rheumatology departments with active localized scleroderma treated by methotrexate for a minimum of 4 months. Read More

JAAD Case Rep 2019 Mar 12;5(3):209-212. Epub 2019 Feb 12.

Department of Dermatology, University of Massachusetts Medical School, Worcester, Massachusetts.

J Am Acad Dermatol 2019 Feb 21. Epub 2019 Feb 21.

Department of Dermatology; Yale University School of Medicine. Electronic address:

Actas Dermosifiliogr 2019 Feb 19. Epub 2019 Feb 19.

Servicio de Dermatología, Hospital Universitario La Paz, Madrid, España.

Indian Dermatol Online J 2019 Jan-Feb;10(1):92-93

Department of Dermatology, Government Medical College Srinagar, Karan Nagar Srinagar, Jammu and Kashmir, India.

Neth J Med 2019 Jan;77(1):29-31

Department of Dermatology, Mohammed V Military Hospital, Mohammed V University of Rabat, Rabat, Morocco.

Radiation-induced morphea (RIM) is a rare complication of radiotherapy of unknown etiology. We report the case of a woman with a history of vitiligo and Hashimoto's thyroiditis, who developed RIM following radiotherapy for breast carcinoma. We suggest that a history of autoimmune disease is a possible risk factor for the development of RIM. Read More

Autoimmun Rev 2019 Apr 14;18(4):369-381. Epub 2019 Feb 14.

Center for Autoimmune Diseases Research (CREA), School of Medicine and Health Sciences, Universidad del Rosario, Bogota, Colombia.. Electronic address:

Overt polyautoimmunity (PolyA) corresponds to the presence of more than one well-defined autoimmune disease (AD) manifested clinically in a single patient. The current study aimed to describe the main characteristics of juvenile PolyA in a pediatric rheumatology setting and analyze the chronological aspects, index cases, familial autoimmunity, and clustering pattern. This was a cross-sectional and multicenter study in which 313 children with overt PolyA were included. Read More

Cutis 2019 Jan;103(1):34-36

St. Joseph Mercy Hospital System, Reichert Health Center, Ypsilanti, Michigan, USA.

En coup de sabre (ECDS) is a rare form of localized scleroderma that typically manifests in children and women. It presents as a fibrous pansclerotic plaque extending in a bandlike distribution on the frontoparietal scalp with surrounding scarring alopecia. Many patients have comorbid central nervous system involvement in addition to the cutaneous findings. Read More

Curr Neurol Neurosci Rep 2019 Feb 12;19(3):11. Epub 2019 Feb 12.

NYU Multiple Sclerosis Comprehensive Care Center, Department of Neurology, New York University School of Medicine, New York, NY, USA.

Purpose Of Review: To describe diverse neurologic and neuroradiologic presentations of two rare, immunologically mediated skin conditions: Sweet disease and localized scleroderma (morphea).

Recent Findings: Core syndromes of neuro-Sweet disease (NSD) are steroid responsiveness, recurrent meningitis, and encephalitis. Focal neurologic, neuro-vascular, and neuro-ophthalmologic syndromes have been reported recently in NSD. Read More

Pediatr Dermatol 2019 Mar 10;36(2):223-226. Epub 2019 Feb 10.

Pediatric Dermatology, Phoenix Children's Hospital, Phoenix, Arizona.

Parry-Romberg syndrome (PRS) is characterized by hemiatrophy of facial structures, including skin, subcutaneous fat, muscle, bone, and cartilage. Complications associated with PRS include headaches, seizures, and chronic facial pain. Protocol for the treatment of chronic facial pain is not clear; reports on the use of botulinum toxin A injections for pain reduction in adults but not in the pediatric/adolescent population are available. Read More

JAAD Case Rep 2019 Feb 25;5(2):144-146. Epub 2019 Jan 25.

Department of Dermatology, University of Texas McGovern Medical School, Houston, Texas.

Int J Dermatol 2019 Apr 1;58(4):E90-E92. Epub 2019 Feb 1.

Department of Dermatology, Charles Nicolle Hospital, Tunis, Tunisia.

JAAD Case Rep 2019 Feb 16;5(2):127-130. Epub 2019 Jan 16.

Department of Dermatology, Amiens University Hospital, Amiens, France.

J Am Acad Dermatol 2019 Jan 28. Epub 2019 Jan 28.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Few studies support treating morphea (localized scleroderma) with hydroxychloroquine.

Objective: To assess the efficacy of hydroxychloroquine treatment of morphea.

Methods: We conducted a retrospective study of 84 patients who had morphea and were treated with hydroxychloroquine monotherapy for at least 6 months at our institution from 1996 through 2013. Read More

J Dermatol 2019 Apr 31;46(4):354-357. Epub 2019 Jan 31.

Department of Dermatology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan.

Morphea profunda refers to inflammatory and sclerotic lesions that start primarily from the deep dermis, subcutaneous fat and fascia. Its pediatric case published work is limited. Here, we report the case of an 8-year-old girl with a 5-year history of multiple subcutaneous nodules on her extremities and a right wrist joint contracture who had been previously diagnosed with juvenile idiopathic arthritis and treated with salazosulfapyridine, low-dose prednisolone (PSL) and methotrexate. Read More

JAMA Dermatol 2019 Jan 16. Epub 2019 Jan 16.

Department of Dermatology and Pediatrics (Pediatric Dermatology), Medical College of Wisconsin, Milwaukee, Wisconsin.

Skin Res Technol 2019 Jan 14. Epub 2019 Jan 14.

Chair and Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.

Background: Clinical characteristics of the lesions are used to identify activity and damage in localized scleroderma (LoS). For high-frequency ultrasound (HF-US), the features of active lesions were described.

Materials And Methods: Clinical signs of activity and damage in LoS lesions were assessed with the use of Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and HF-US by two examiners independently. Read More

Arch Rheumatol 2018 Sep 18;33(3):344-351. Epub 2018 Jan 18.

Department of Pediatric Rheumatology, İstanbul University, Cerrahpasa Medical School, İstanbul, Turkey.

Objectives: This study aims to evaluate the demographic and clinical features, laboratory data, treatment modalities, and outcomes of juvenile systemic sclerosis (JSS) and juvenile localized scleroderma (JLS) patients at a referral pediatric rheumatology center in Turkey.

Patients And Methods: Medical records of a total of 57 patients, including 29 with JSS (1 male, 28 females; mean age 18.3±3. Read More

Postepy Dermatol Alergol 2018 Dec 13;35(6):638-640. Epub 2018 Nov 13.

Department of Dermatology, Pediatric Dermatology and Oncology, Medical University of Lodz, Lodz, Poland.

Postepy Dermatol Alergol 2018 Dec 13;35(6):614-619. Epub 2018 Nov 13.

Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.

Introduction: Scleroderma is a chronic connective tissue disease resulting in fibrosis.

Aim: The aim of the study was to determine the connection between sE-selectin and sIL-2R and the severity of skin lesions in various subtypes of LoS. Evaluation of disease severity, the location of skin lesions, the duration of symptoms and disease activity were assessed in relation to the three different LoS subtypes in patients with localized scleroderma. Read More

J Invest Dermatol 2019 Mar 25;139(3):715-718. Epub 2018 Oct 25.

Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA; University of Pittsburgh, Pittsburgh, Pennsylvania, USA. Electronic address:

Cutis 2018 Nov;102(5):E10-E12

Division of Dermatology, Department of Medicine, Albert Einstein College of Medicine, Bronx, New York, USA.

Pediatr Rheumatol Online J 2018 Dec 18;16(1):80. Epub 2018 Dec 18.

Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Eaton Road, Liverpool, L12 2AP, UK.

Objective: To describe current United Kingdom practice in assessment and management of patients with juvenile localised scleroderma (JLS) compared to Paediatric Rheumatology European Society (PRES) scleroderma working party recommendations.

Methods: Patients were included if they were diagnosed with JLS and were under the care of paediatric rheumatology between 04/2015-04/2016. Retrospective data was collected in eleven UK centres using a standardised proforma and collated centrally. Read More

Medicine (Baltimore) 2018 Dec;97(49):e13404

Department of Acupuncture and Moxibustion Medicine, Dongguk University Ilsan Oriental Hospital, Goyang, Republic of Korea.

Rationale: Bee venom has been reported to demonstrate antinociceptive and anti-inflammatory effects in experimental studies, but there remain questions regarding the clinical use of bee venom, especially for scleroderma. This case report shows the successful outcome of bee venom acupuncture for circumscribed morphea in a patient with systemic sclerosis, which is considered to be a rare condition.

Patient Concerns: A 64-year-old Korean woman had circular white areas (3 and 1 cm diameter) with severe itch in the right lateral iliac crest. Read More

J Dtsch Dermatol Ges 2018 Dec;16(12):1480-1482

Dermatology, Venereology and Leprosy, Ellahi Medicare Clinic, Kashmir, India.

Cytokine 2018 Dec 6. Epub 2018 Dec 6.

Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI 48109, USA. Electronic address:

Type I interferons (IFNs) are an immunomodulatory class of cytokines that serve to protect against viral and bacterial infection. In addition, mounting evidence suggests IFNs, particularly type I but also IFNγ, are important to the pathogenesis of autoimmune and inflammatory skin diseases, such as cutaneous lupus erythematosus (CLE). Understanding the role of IFNs is relevant to anti-viral responses in the skin, skin biology, and therapeutics for these IFN-related conditions. Read More

Asian Pac J Allergy Immunol 2018 Dec 9. Epub 2018 Dec 9.

Department of Allergy, Immunology and Rheumatology, Mackay Children's Hospital, Taipei, Taiwan.

Generalised morphea (GM) is a subtype of localised scleroderma that usually manifests with bilateral involvement. Unilateral generalised morphea (UGM) is a rare variant of GM. This is a case report of a Taiwanese girl with UGM over the left side of her body. Read More

Case Rep Dermatol 2018 Sep-Dec;10(3):226-230. Epub 2018 Oct 18.

Department of Dermatology, Nara Medical University School of Medicine, Kashihara, Japan.

We report the case of a 45-year-old female with generalized morphea (GM), who exhibited positivity for the anti-centromere antibody (Ab). She frequently developed multiple sclerotic skin lesions, whose histological findings were compatible with morphea. She demonstrated favorable responses to topical and oral steroids. Read More

Int Med Case Rep J 2018 12;11:313-318. Epub 2018 Nov 12.

Department of Infectious Diseases, Regional Hospital of Bellinzona e Valli, 6500 Bellinzona, Ticino, Switzerland.

Tularemia is a zoonosis caused by the infection of (a gram-negative aerobic bacterium). Transmission to other animals or humans usually occurs through insect or tick bites, direct contact with a contaminated environment (mud or water), infected animals - mainly lagomorphs - or by ingesting undercooked meat or inhaling contaminated dust (hay or soil). This paper discusses the case of a 32-year-old man, who came to our Emergency Room presenting with persistent fever, inguinal lymphadenopathy, and an ulcer on his left lower limb on a linear morphoea lesion that had been there for some time. Read More

J Dermatol 2019 Feb 3;46(2):131-138. Epub 2018 Dec 3.

Fudan University, Department of Radiation Oncology, Zhongshan Hospital, School of Medicine, Fudan University, Shanghai, China.

There is no established diagnostic criteria or widely accepted severity classification of localized scleroderma (LS) by imaging. Acoustic radiation force impulse (ARFI) technology by normalized mean shear wave velocity (SWV) may be as a probing tool for diagnosing and staging LS accurately and objectively. Fifty-six patients with LS of inflammatory (n = 21), sclerotic (n = 24) and atrophic (n = 11) stage and 30 healthy controls were evaluated on the basis of pathological results. Read More

Plast Reconstr Surg 2018 11;142(5):1275-1283

Madison, Wis.; and Livingston, N.J. From the Division of Plastic and Reconstructive Surgery, University of Wisconsin School of Medicine and Public Health; and Summit Medical Group.

Background: Parry Romberg disease (hemifacial atrophy) and linear scleroderma (coup de sabre) are progressive, usually unilateral facial atrophies of unknown cause. The gold standard treatment for these patients has been microsurgical reconstruction following the "burning out" of the facial atrophy and stable contour for 2 years.

Methods: The authors report their experience treating patients with hemifacial atrophy and linear scleroderma with free tissue transfers between 1989 and 2016. Read More

Pediatr Dermatol 2019 Jan 28;36(1):e56-e57. Epub 2018 Nov 28.

Division of Rheumatology, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire.

A 12-year-old female with oligoarticular juvenile inflammatory arthritis developed an atrophic linear plaque involving the left medial forearm and proximal arm 7 months after intra-articular triamcinolone injection for arthritis. The plaque spontaneously resolved without treatment over approximately one year. It is important to recognize this rare complication of intra-articular steroid injection in order to avoid potential misdiagnosis as linear scleroderma and subsequent immunosuppressive treatment. Read More

Am J Dermatopathol 2018 Dec;40(12):873-878

Professor of Clinical Dermatology and Pathology Director, UC Davis Dermatopathology Service Executive Vice Chair of Academic Affairs, Department of Dermatology, University of California Davis School of Medicine, Sacramento, CA.

Histologic clues that facilitate rapid diagnosis of morphea at scanning magnification have been described but not well studied. We examined 73 cases of morphea and 42 control cases to determine the sensitivity and specificity of a novel histopathological feature-"the line sign (LS)"-a prominent, straight interface between subcutis and adjacent collagen. The sensitivity of LS was shown to be the most sensitive feature among 4 other existing histopathological features. Read More

Dermatol Clin 2019 Jan 1;37(1):37-48. Epub 2018 Nov 1.

Departments of Dermatology and Pathology, Cleveland Clinic Lerner College of Medicine, 9500 Euclid Avenue A61, Cleveland, OH 44195, USA. Electronic address:

Connective tissue diseases often prominently affect the skin, requiring dermatologists to play an important role in diagnosis and treatment of these patients. Herein we describe updates on the pathogenesis, clinical features, and treatment of 4 major connective tissue diseases: dermatomyositis, cutaneous lupus erythematosus, limited scleroderma (morphea), and cutaneous vasculitis. Many of these updates promise to improve clinical care of patients who suffer from dermatologic involvement of these diseases and are the result of research performed by dermatologists who have expertise in these conditions. Read More

J Dtsch Dermatol Ges 2018 Dec 22;16(12):1480-1482. Epub 2018 Nov 22.

Dermatology, Venereology and Leprosy, Ellahi Medicare Clinic, Kashmir, India.

Eur J Dermatol 2018 Oct;28(5):718-720

Centre Hospitalier Régional Universitaire (CHRU) Tours, Department of Dermatology, 37044 Tours Cedex 9, France, Universities of Nantes and Tours, SPHERE-INSERM 1246, 37000 Tours, France.

Eur J Dermatol 2018 Nov 13. Epub 2018 Nov 13.

Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.

Postepy Dermatol Alergol 2018 Oct 19;35(5):470-473. Epub 2018 Jul 19.

Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.

Introduction: Morphea, also known as localized scleroderma, is an autoimmune skin disease which is characterized by excessive accumulation of collagen that leads to the thickening of the dermis and subcutaneous tissue. There is an unclear relationship between morphea and other autoimmune diseases, especially related to the thyroid gland.

Aim: To determine the occurrence of increased antithyroid antibodies in patients with morphea in relation to the clinical manifestations of the disease. Read More

BMJ Case Rep 2018 Nov 14;2018. Epub 2018 Nov 14.

Department of Dermatology, Southend University Hospital NHS Foundation Trust, Westcliff-on-Sea, UK.

Parry-Romberg syndrome (PRS) is characterised by progressive but self-limiting facial hemiatrophy. We describe a 48-year-old woman with a 3-year history of gradually worsening right facial hemiatrophy on a background of scleroderma. Her initial primary concern was alopecia. Read More

J Dtsch Dermatol Ges 2018 Nov;16(11):1323-1327

Department of Dermatology, Venereology and Dermato-Oncology, Faculty of Medicine, Semmelweis University, Budapest, Hungary.

Background And Objectives: Morphea is frequently associated with other autoimmune disorders. Little is known about the association of morphea and primary biliary cholangitis (PBC). The objective of this case series was to study the possible association of morphea with PBC and to identify risk factors. Read More

Actas Dermosifiliogr 2019 Mar 30;110(2):153-159. Epub 2018 Oct 30.

Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.

Postirradiation morphea is an uncommon entity that has been mostly described in women with breast cancer. The increasing use of radiotherapy to treat breast cancer and the clinical similarities between morphea and other conditions, such as radiodermatitis, postirradiation fibrosis, and tumor recurrence, highlights the need for dermatologists to be familiar with this entity. We present a series of 6 women with a mean age of 64. Read More

Biofactors 2018 Nov 27;44(6):507-517. Epub 2018 Oct 27.

Department of Regulatory Toxicology, National Institute of Pharmaceutical Education and Research (NIPER), Balanagar, Hyderabad, Telangana, 500037, India.

Scleroderma is an inflammatory autoimmune disease which begins with inflammation due to tissue injury and advances to progressive accumulation of extracellular matrix resulting in scarring and hardening of the skin. Inflammation is a salutary response to tissue injury caused by varied factors. While inflammation is required for systematic wound healing, dysregulated chronic inflammation often leads to tissue scarring. Read More