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Dermatol Ther (Heidelb) 2020 Jun 23. Epub 2020 Jun 23.

Department of Dermatology, University of Rzeszow, Rzeszow, Poland.

Eosinophilic annular erythema (EAE) is a rare condition with a chronic relapsing and remitting course, characterized by the presence of annular or polycyclic erythematous and plaque lesions and prominent tissue eosinophilia on histopathology. There is an ongoing discussion on whether EAE is a subset of Wells syndrome (eosinophilic cellulitis) or a separate entity. To date, few cases of EAE have been reported in the literature; of these, about 40 cases were in adults and fewer than ten cases were in children. Read More

Am J Clin Dermatol 2020 May 11. Epub 2020 May 11.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Eosinophilic dermatoses encompass a broad spectrum of diseases of different etiologies hallmarked by eosinophilic infiltration of the skin and/or mucous membranes, with or without associated blood eosinophilia. The wide range of dermatological manifestations of this spectrum, including nodules and plaques, pustules, blisters, ulcers, and urticarial lesions, is reflected in a non-univocal classification system. We identified six groups of eosinophilic dermatoses based on the predominant anatomic level of involvement: (1) epidermal; (2) of the dermal-epidermal junction; (3) dermal; (4) of the hypodermis and muscle fascia; (5) of the pilosebaceous unit; and (6) vascular/perivascular. Read More

J Pediatr Genet 2020 Jun 30;9(2):109-113. Epub 2019 Sep 30.

Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.

Histiocytosis-lymphadenopathy plus syndrome (H syndrome) is caused by mutations in the gene that result in histiocytic infiltration of numerous organs. Patients suffering from this disorder can be easily mistaken for similar conditions such as Muckle-Wells syndrome. We present a 9. Read More

Pediatr Int 2020 May 17;62(5):635-637. Epub 2020 Apr 17.

Department of Pediatrics, Medical Research Institute, Pusan National University School of Medicine, Pusan National University Hospital, Busan, Korea.

J Dermatolog Treat 2020 Apr 22:1-14. Epub 2020 Apr 22.

Department of Dermatology and Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

Anakinra (Kineret) is an interleukin-1 receptor antagonist (IL-1Ra) FDA approved for use in rheumatoid arthritis and in neonatal-onset multisystem inflammatory disease (NOMID). It has been used off-label for a variety of dermatologic conditions. A review of the available studies and cases of these off-label uses would be valuable to the dermatologist considering alternative treatments for these oftentimes poorly studied conditions. Read More

World J Pediatr 2020 Apr 3. Epub 2020 Apr 3.

Rheumatology Department, Unidade Local de Saúde Do Alto Minho, Hospital Conde Bertiandos, 4990-041, Ponte de Lima, Portugal.

Rev Med Interne 2020 Feb 20. Epub 2020 Feb 20.

Service de dermatologie et vénéréologie, CHU de Ibn Rochd, Casablanca, Maroc.

Wells syndrome (WS), also called eosinophilic cellulitis is a rare inflammatory skin disease with about 200 cases reports in the literature. It is part of eosinophilic dermatoses and presents a diagnostic challenge. We report a case of WS presenting as bacterial cellulitis. Read More

Contact Dermatitis 2020 Jun 20;82(6):401-402. Epub 2020 Feb 20.

Division of dermatology, CHU de Québec, Laval University, Québec, Canada.

Reumatismo 2020 Jan 28;71(4):226-229. Epub 2020 Jan 28.

Department of Internal Medicine, Groupe Hospitalier Sud Ile de France, Melun.

Wells' syndrome, also called eosinophilic cellulitis, is a rare eosinophilic dermatosis characterized by an unspecific inflammatory erythematous eruption often associated with systemic symptoms. Here we report the case of a 57-year-old female with bilateral painful pitting and pruritic feet progressive for two weeks despite one week of oral antibiotics. Skin biopsy was performed showing dermal eosinophilic infiltration. Read More

J Dermatol 2020 Mar 21;47(3):e75-e77. Epub 2020 Jan 21.

Department of Dermatology, Fukushima Medical University, Fukushima, Japan.

Harefuah 2020 Jan;159(1):34-37

Department of Dermatology, Emek Medical Center, Afula, Israel.

Aims: This study aims to critically review the pros and cons of biological drugs as treatments and triggers of eosinophilic dermatoses.

Background: Eosinophilic dermatoses syndromes are rare diseases with a prominent eosinophilic infiltration mechanism. These syndromes have several known treatments with limited success. Read More

J Postgrad Med 2020 Jan-Mar;66(1):59

[This corrects the article DOI: 10.4103/0022-3859.153107]. Read More

Clin Dermatol 2019 Sep - Oct;37(5):516-519. Epub 2019 Jul 17.

Medical College, Kolkata, West Bengal, India.

Greenish staining of human skin may result from a gamut of causes, such as chlorosis, exogenous copper, resolving ecchymosis, drugs, green textile dyes, green tattoos, apocrine and eccrine chromhidrosis, hyper biliverdinemia, chloromas, use of green dyes during tube feeding in patient with multiorgan failure, Pseudomonas infections, and Wells' syndrome in its second stage. Physicians may rarely encounter patients with green skin, hair, nails, or mucosae. Read More

Turk J Biol 2019 13;43(6):349-359. Epub 2019 Dec 13.

Department of Molecular Biology and Genetics, Apoptosis and Cancer Immunology Laboratory (AKIL), Boğaziçi University, İstanbul Turkey.

Inflammasomes are supramolecular protein complexes implicated in the detection of pathogens or danger-associated molecules and are responsible for mounting the first line of innate immune response to counteract these signals and restore tissue homeostasis. Among different inflammasomes identified so far, NLRP3 is of main interest since mutations in Nlrp3 gene are associated with autoinflammatory diseases such as Muckle-Wells syndrome, neonatal onset multisystem inflammatory disease, and familial cold urticaria/autoinflammatory syndrome. On the other hand, whereas other inflammasomes are mainly detectors of specific molecular motifs, NLRP3 is acting as a general sensor of cellular perturbations including potassium efflux, lysosomal damage, and ROS production. Read More

Int J Rheum Dis 2020 Feb 19;23(2):262-270. Epub 2019 Dec 19.

Division of Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

Cryopyrin-associated periodic fever syndrome (CAPS) represents an increasingly recognized disease group entity, with varied presentations. CAPS includes 3 clinical entities, namely, familial cold-induced autoinflammatory syndrome (FCAS; MIM #120100), Muckle-Wells syndrome (MWS; MIM #191900) and chronic inflammatory neurologic cutaneous and articular syndrome (CINCA; MIM #607115); which share several overlapping clinical features. These patients often present with early-onset episodes of fever and rash, and variable systemic signs and symptoms, making it a great mimicker of other systemic autoimmune diseases. Read More

Open Access Maced J Med Sci 2019 Sep 30;7(18):3002-3005. Epub 2019 Jun 30.

Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital, Dresden, Germany.

Background: Wells syndrome is a rare idiopathic dermatosis of the eosinophilic spectrum. Diagnostic criteria include cutaneous eruptions of variable morphology with eosinophilic infiltrates, peripheral blood eosinophilia, a relapsing, remitting course, and exclusion of systemic disease. Diagnosis is often delayed. Read More

J Int Adv Otol 2019 Dec;15(3):454-458

Department of Otolaryngology, Gifu University, Gifu, Japan.

Muckle-Wells syndrome (MWS), a subclass of cryopyrin-associated periodic syndrome (CAPS), sometimes includes complications of bilateral progressive sensorineural hearing loss. A 48-year-old woman had been diagnosed with pediatric rheumatic arthritis at aged 6 years; however, systematic therapy with prednisolone and methotrexate showed limited efficacy for her general fatigue and arthritic pain, and it never improved the hearing level. She underwent a cochlear implant surgery for progressive profound bilateral hearing loss. Read More

ACR Open Rheumatol 2019 Jun 6;1(4):267-276. Epub 2019 Jun 6.

Sorbonne Université INSERM, Hôpital Trousseau Paris France.

Objective: To determine the molecular and cellular bases of autoinflammatory syndromes in a multigenerational French family with Muckle-Wells syndrome and in a patient originating from Portugal with familial cold autoinflammatory syndrome.

Methods: Sequencing of exon 3 was performed in all accessible patients. Microsatellite and whole-genome single nucleotide polymorphism genotyping was used i) to test the intrafamilial segregation of the identified variant and ii) to look for a founder effect. Read More

Br J Nurs 2019 Oct;28(18):1180-1186

Honorary Research Associate, National Amyloidosis Centre, Division of Medicine, University College London.

Cryopyrin-associated periodic syndrome (CAPS) is a group of rare hereditary autoinflammatory diseases characterised by recurrent flares of mild to severe systemic inflammation and fever. CAPS is the umbrella term for a spectrum of individual conditions, namely familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID), also known as chronic infantile neurologic, cutaneous and articular (CINCA) syndrome. The flare symptoms include fever, fatigue, rashes, headaches, arthralgia and myalgia that can last for a few hours or for several days. Read More

J Dtsch Dermatol Ges 2019 10 27;17(10):1039-1051. Epub 2019 Sep 27.

Department of Dermatology, University Hospital, Jena, Germany.

Eosinophilic dermatoses are a heterogeneous group of diseases, characterized by an eosinophil-rich infiltrate and/or degranulation of eosinophils. Blood eosinophilia may be an associated feature. Typical, albeit not specific histological findings include 'flame figures', which are caused by the accumulation of cationic proteins released by eosinophils and subsequent collagen denaturation. Read More

J Investig Allergol Clin Immunol 2020;30(1):58-59. Epub 2019 Sep 18.

Division of Immunology and Allergology, Department of Internal Medicine, University Hospital and Medical Faculty, Geneva, Switzerland.

J Dermatol 2019 Dec 9;46(12):e451-e452. Epub 2019 Sep 9.

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

Dis Colon Rectum 2019 10;62(10):1150-1152

Department of Colorectal and Intestinal Failure Surgery, Salford Royal National Health Service Foundation Trust, University of Manchester, Manchester, United Kingdom.

Am J Dermatopathol 2020 May;42(5):329-336

Clinical Department of Dermatology and Venereology, Medical University Innsbruck, Innsbruck, Austria.

Erythema nodosum (EN) is considered to represent a septal panniculitis. In a period from January 2000 until June 2018, we clinically and histopathologically investigated 124 patients with EN, 13 (10.5%) of them in an early stage demonstrating features of a leukocytoclastic vasculitis (LCV) around postcapillary venules of the subcutaneous fat. Read More

Dtsch Arztebl Int 2019 Jul;116(27-28):470

An Bras Dermatol 2019 Jul 29;94(3):370-372. Epub 2019 Jul 29.

RSP Dermatologia, Ribeirão Preto (SP), Brazil.

Cutis 2019 Jun;103(6):E25-E27

Riddle Memorial Hospital, Media, Pennsylvania, USA.

Ann Agric Environ Med 2019 Jun 30;26(2):256-259. Epub 2018 Oct 30.

Faculty of Medicine, Jan Kochanowski University, Kielce, Poland.

Introduction: The study presents the case report of a Wells Syndrome in a 18-year -ld female. Wells Syndrome is a rare inflammatory skin disorder which seems to present an abnormal eosinophilic response to a number of triggers.

Objective: The aim of the study is to discuss several problems related to the diagnosis and treatment of Wells Syndrome. Read More

Arthritis Rheumatol 2019 11 9;71(11):1955-1963. Epub 2019 Sep 9.

University of Toronto and the Hospital for Sick Children, Toronto, Ontario, Canada.

Objective: To assess long-term efficacy and safety of canakinumab and the response to vaccination in children ages ≤5 years with cryopyrin-associated periodic syndrome (CAPS).

Methods: CAPS patients (ages ≤5 years) received 2 mg/kg canakinumab subcutaneously every 8 weeks; patients with neonatal-onset multisystem inflammatory disease (NOMID) received a starting dose of 4 mg/kg in this open-label trial. Efficacy was evaluated using physician global assessment of disease activity and serum levels of C-reactive protein (CRP) and amyloid A (SAA). Read More

J Eur Acad Dermatol Venereol 2019 10 3;33(10):e388-e391. Epub 2019 Jun 3.

Department of Dermatology, Hospital del Mar, IMIM, Universitat Autònoma, Barcelona, Spain.

J Clin Immunol 2019 04 10;39(3):277-286. Epub 2019 May 10.

Division of Pediatric Allergy, Immunology, and Rheumatology, Rady Children's Hospital of San Diego, University of California, San Diego, San Diego, CA, USA.

Cryopyrin-associated periodic syndrome (CAPS) is a rare inherited autoinflammatory disorder characterized by systemic, cutaneous, musculoskeletal, and central nervous system inflammation. Gain-of-function mutations in NLRP3 in CAPS patients lead to activation of the cryopyrin inflammasome, resulting in the inappropriate release of inflammatory cytokines including IL-1β and CAPS-related inflammatory symptoms. Several mechanisms have been identified that are important for the normal regulation of the cryopyrin inflammasome in order to prevent uncontrolled inflammation. Read More

Front Immunol 2019 16;10:802. Epub 2019 Apr 16.

Paediatric Rheumatology and Autoinflammatory Diseases Unit, Department of Paediatrics and Adolescent Medicine, First Faculty of Medicine, General University Hospital in Prague, Charles University, Prague, Czechia.

Muckle-Wells syndrome (MWS) represents a moderate phenotype of cryopyrinopathies. Sensorineural hearing loss and AA amyloidosis belong to the most severe manifestations of uncontrolled disease. Simultaneous discovery of MWS in four generations of one large kindred has enabled us to document natural evolution of untreated disease and their response to targeted therapy. Read More

Eur J Paediatr Neurol 2019 May 28;23(3):466-472. Epub 2019 Mar 28.

Department of Pediatric Neurology, Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Istanbul, Turkey.

Objective: Neurologic complications of chronic infantile neurologic, cutaneous and articular syndrome (CINCA) are well-known, whereas there are scarce data regarding neurologic features of milder cryopyrin-associated periodic syndrome (CAPS) phenotypes. We aimed to review the neurologic features in detail and summarize the other CAPS-related manifestations in 12 children.

Methods: All children with CAPS that have been followed-up from pediatric rheumatology outpatient clinic, were enrolled to the study. Read More

World Allergy Organ J 2019 14;12(3):100019. Epub 2019 Mar 14.

Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Germany.

Background: Systemic autoinflammatory diseases (SAIDs) are rare debilitating disorders of which there is limited awareness and a significant delay in diagnosis. There is no uniform approach in the diagnosis and treatment of these disorders and the real life state of SAID patient care is poorly characterized. The aim of this study was to obtain data on the epidemiology, state of care and the perception of physicians who are involved in the care of SAID patients. Read More

JAMA Dermatol 2019 May;155(5):617-618

Department of Dermatology, Xijing Hospital, Fourth Military Medical University, Xi'an, China.

J Dermatol 2019 May 27;46(5):422-425. Epub 2019 Feb 27.

Department of Dermatology, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.

A Chinese female infant presented with ectodermal dysplasia, cleft palate and severe skin erosions at birth. Although all the typical clinical features of ankyloblepharon-ectodermal dysplasia-clefting (AEC) syndrome were present, the ankyloblepharon was not very marked. We misdiagnosed epidermolysis bullosa and congenital ichthyosiform erythroderma at first and confirmed the diagnosis of AEC syndrome only when she presented with the typical clinical manifestation of recurrent infected scalp erosions at 1 year of age. Read More

Rheumatol Int 2019 05 19;39(5):911-919. Epub 2019 Feb 19.

Department of Molecular Biology-Genetics and Biotechnology, Dr. Orhan Öcalgiray Molecular Biology-Biotechnology and Genetics Research Centre, Graduate School of Science, Engineering and Technology, İstanbul Technical University, Ayazağa Campus, Maslak, 34469, Istanbul, Turkey.

Systemic autoinflammatory diseases (sAIDs) are a heterogeneous group of disorders, having monogenic inherited forms with overlapping clinical manifestations. More than half of patients do not carry any pathogenic variant in formerly associated disease genes. Here, we report a cross-sectional study on targeted Next-Generation Sequencing (NGS) screening in patients with suspected sAIDs to determine the diagnostic utility of genetic screening. Read More

Isr Med Assoc J 2019 Jan;21(1):65

Department of Dermatology, Emek Medical Center, Afula, Israel.

Ocul Immunol Inflamm 2020 17;28(1):70-78. Epub 2018 Dec 17.

Pediatric Allergy and Clinical Immunology, Uludag University School of Medicine, Bursa, Turkey.

: To present the ocular findings of the members of a family that has the diagnosis of Muckle Wells syndrome, a form of cryopyrin associated periodic syndrome (CAPS).: Nine patients with MWS were included in this study. Each study participant underwent a systemic evaluation, comprehensive ophthalmic examination, and auxillary testings. Read More

Vet Clin North Am Small Anim Pract 2019 Jan;49(1):47-51

Department of Pathobiology, University of Pennsylvania, School of Veterinary Medicine, 3900 Delancey Street, Philadelphia, PA 19104-6051, USA. Electronic address:

Canine acute eosinophilic dermatitis with edema is an uncommon syndromic disorder in dogs with a unique clinical presentation. Most but not all dogs have a history of gastrointestinal upset preceding or concomitant with skin lesion onset. Affected dogs present with macular to generalized erythema that is most evident on the glabrous skin of the abdomen. Read More

Acta Dermatovenerol Croat 2018 Oct;26(3):262-263

Assoc. Prof. Suzana Ljubojević Hadžavdić, MD, PhD, Department of Dermatology and Venereology University Hospital Center Zagreb School of Medicine University of Zagreb, Šalata 4, 10000 Zagreb, Croatia;

Dear Editor,Eosinophilic annular erythema (EAE) is a rare figurate dermatitis of unknown etiology with prominent tissue eosinophilia. A 59-year-old male patient presented with a one-month history of itchy, polycyclic, annular, and partially serpiginous lesions involving the back, the gluteal region, and the extremities (Figure 1, a, b). There was no medical history of drug intake. Read More

J Dermatol 2019 04 26;46(4):e146-e147. Epub 2018 Oct 26.

Division of Dermatology, Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.

Clin Rheumatol 2019 Mar 18;38(3):943-948. Epub 2018 Oct 18.

Department of Pediatrics, Yamaguchi University Graduate School of Medicine, 1-1-1 Minamikogushi, Ube, Yamaguchi, 755-8505, Japan.

Cryopyrin-associated periodic syndrome (CAPS) is one of the autoinflammatory disorders caused by mutations in NLRP3 gene. The over-production of interleukin (IL)-1β induced by NLRP3 gene mutations plays an important role in the pathophysiology of CAPS. We diagnosed 3 patients with CAPS, who were lineal family members having a novel mutation of NLRP3 gene. Read More

Eur J Dermatol 2018 Oct;28(5):700-701

Department of Dermatology, Ehime University Graduate School of Medicine, Toon, Ehime, Japan.

Ann Pathol 2018 Dec 17;38(6):401-406. Epub 2018 Sep 17.

Service d'anatomie et cytologie pathologiques, CHU de Pontchaillou, 2, rue Henri-le-Guilloux, 35033 Rennes cedex 9, France.

Introduction: Cryopyrin associated periodic syndrome is a rare auto inflammatory disease including three clinical entities with a common genetic cause. Among these three entities, Muckle-Wells syndrome is described as an intermediate phenotype associated with a progressive sensorineural hearing loss and AA amyloidosis. The present case reports a renal AA amyloidosis associated with an IgA nephropathy, revealing a Muckle-Wells syndrome. Read More

J Dtsch Dermatol Ges 2018 Oct 29;16(10):1250-1252. Epub 2018 Aug 29.

Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Berlin, Germany.

Cutis 2018 Jun;101(6):400;405;406

Department of Dermatology, Oregon Health and Science University, Portland, USA.

J Dermatol 2019 Feb 31;46(2):e57-e59. Epub 2018 Jul 31.

Department of Dermatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.

Indian J Dermatol Venereol Leprol 2018 Sep-Oct;84(5):594-599

Department of Pathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.