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Ophthalmic Plast Reconstr Surg 2022 Jun 14. Epub 2022 Jun 14.

Department of Ophthalmology, Ophthalmic Plastic Surgery, Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts.

A 62-year-old man presented with diffuse, painless, left-sided preseptal edema, erythema, and woody induration extending to the left temple. The induration generated an orbital compartment syndrome with markedly elevated intraocular pressure necessitating lateral canthotomy and cantholysis. Although atypical for an infectious etiology, empiric broad-spectrum intravenous antibiotics were initiated with no improvement. Read More

Medicine (Baltimore) 2022 Jun 3;101(22):e29274. Epub 2022 Jun 3.

Division of Endocrinology, Shin-Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.

Rationale: Pituitary apoplexy occurs in about 8% of those with nonfunctioning pituitary adenoma. Subsequent hormone deficiency, especially corticotropic deficiency, is the most common finding. We describe the unusual manifestations of adrenal insufficiency that are usually overlooked in such cases, with the aim of raising awareness of this disease. Read More

J Med Invest 2022 ;69(1.2):145-147

Department of Urology, Nippon Medical School, Tokyo, Japan.

Robot-assisted laparoscopic prostatectomy (RALP) for prostate cancer was introduced in 2000 and rapidly gained popularity. The Da Vinci Surgical System? can ensure improved local control of cancer and fewer perioperative complications. However, RALP is performed in the steep-Trendelenburg position (a combination of lithotomy and head-down tilt position/Lloyd-Davies position) to obtain a good surgical view, and as a result, well leg compartment syndrome (WLCS) can become a serious complication of RALP. Read More

Zhonghua Xin Xue Guan Bing Za Zhi 2022 Apr;50(4):401-403

Department of Cardiology, Shanghai Institute of Cardiovascular Diseases, Zhongshan Hospital, Fudan University, Shanghai 200032, China.

J Allergy Clin Immunol 2022 Apr;149(4):1137-1149

Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy; Internal Medicine, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. Electronic address:

Urticarial vasculitis (UV) is a rare cutaneous vasculitis of small vessels characterized by recurrent episodes of wheal-like lesions that tend to last more than 24 hours, healing with a residual ecchymotic postinflammatory hyperpigmentation. The histopathologic pattern of UV is that of leukocytoclastic vasculitis, consisting of fibrinoid necrosis of dermal vessels' walls and neutrophil-rich perivascular inflammatory infiltrates. Although its etiopahogenesis remains still undefined, UV is now regarded as an immune complex-driven disease with activation of the complement cascade, leading to exaggerated production of anaphylatoxins that are responsible for neutrophil recruitment and activation. Read More

Cureus 2022 Feb 27;14(2):e22657. Epub 2022 Feb 27.

Dermatology Unit, Cheikh Khalifa International University Hospital, Mohammed VI University of Health Sciences, Casablanca, MAR.

Eosinophilic annular erythema (EAE) is a rare dermatosis. Its relationship with Wells syndrome (WS) is debated. We report a case treated with hydroxychloroquine. Read More

Clin Rheumatol 2022 Jul 22;41(7):2233-2237. Epub 2022 Mar 22.

Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.

There have been hundreds of reports on mutations in the NLRP3 gene related to NLRP3-associated autoinflammatory disease, but few of these mutations have occurred as both germline and somatic mosaic mutations. In this case-based review, we report a 68-year-old man with an NLRP3-associated autoinflammatory disease. He developed secondary amyloidosis, including a renal and colorectal presentation in his 50 s. Read More

JAAD Case Rep 2022 Apr 9;22:11-13. Epub 2022 Feb 9.

Department of Dermatology, Centre Hospitalier Universitaire de Québec, Université Laval, Québec, Canada.

Rheumatology (Oxford) 2022 Mar 9. Epub 2022 Mar 9.

Sorbonne Université, internal medicine department, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, France.

Objective: Cryopyrin-associated periodic syndrome (CAPS) is a rare but treatable inherited autoinflammatory condition including familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and chronic infantile neurologic cutaneous articular syndrome (CINCA). Our objective was to describe the main features of CAPS AA amyloidosis (AA-CAPS) associated and the efficacy of interleukin-1 inhibitors in this indication.

Methods: Retrospective study in France associated with a systematic literature review. Read More

JAAD Case Rep 2022 May 3;23:27-30. Epub 2022 Mar 3.

Department of Dermatology, Warren Alpert Medical School of Brown University, Providence, Rhode Island.

Am J Dermatopathol 2022 Feb 2. Epub 2022 Feb 2.

University of California Davis School of Medicine, Sacramento, CA; Dermatology Department, Loma Linda University, Loma Linda, CA; and Dermatology Section, Geisinger Medical Center, Wilkes-Barre, PA.

Abstract: Tattoo reactions have become more common as tattoos gain visibility and popularity worldwide. A variety of inflammatory patterns have been described in association with tattoos- more commonly fibrosing, granulomatous, lichenoid, pseudolymphomatous, and spongiotic patterns. To date, there has been one case report of a Wells syndrome-like inflammatory pattern associated with a temporary Henna tattoo. Read More

J Eur Acad Dermatol Venereol 2022 05 31;36(5):e328-e330. Epub 2022 Jan 31.

Department of Dermatology, School of Medicine, Ajou University, Suwon, Korea.

Mod Rheumatol Case Rep 2022 06;6(2):282-287

Center for Preventive Medical Sciences, Chiba University, Chiba, Japan.

Cryopyrin-associated periodic syndrome (CAPS) is a rare inherited autoinflammatory disease caused by gain-of-function mutations in the NLRP3 gene, with a genotype-phenotype correlation. The clinical presentation of each mutation has been previously studied. However, very few studies have reported on the clinical characteristics and treatment effectiveness across different generations within a family with the same mutation. Read More

Life (Basel) 2021 Dec 19;11(12). Epub 2021 Dec 19.

Department of Pediatrics, Okayama City Hospital, Okayama City 700-8557, Japan.

The aim of this study is to describe bilateral optic disc swelling in three consecutive patients with Blau syndrome or cryopyrin-associated periodic syndrome at a single institution. Case 1 was a 30-year-old woman receiving 25 mg etanercept twice weekly who had been diagnosed as early-onset sarcoidosis by biopsy of skin rashes at 5 months old and genetically diagnosed with Blau syndrome with / mutation (N670K) at 13 years old. At 10 years old, she began to have uveitis with optic disc swelling in both eyes, resulting in macular degeneration and optic disc atrophy at 17 years old only when etanercept was introduced. Read More

Arerugi 2021 ;70(10):1391-1397

Department of Infectious Diseases and Respiratory Medicine, Fukushima Medical University Aizu Medical Center.

A-68-year-old man, who has allergic rhinitis with peripheral blood eosinophilia, hospitalized because of fever of unknown origin in May 2020. Five days after antibiotics were given, itchy exanthema occurred, followed by gland glass opacity on both lungs with bilateral pleural effusions. Since acute respiratory failure developed, bronchoscopy was hard to carry out. Read More

Lancet 2021 12;398(10316):2101

McGill University Health Centre Research Institute, Montreal, QC, Canada. Electronic address:

Curr Rheumatol Rev 2021 Nov 15. Epub 2021 Nov 15.

Onco-Hematologist, Department of Internal Medicine No.2, V.I. Vernadsky Crimean Federal University, Simferopol. Russian Federation.

Background: Cryopyrin-Associated Periodic Syndrome (CAPS) is a variety of clinical variants of autoinflammatory diseases. The pathology is based on a mutation in the NLRP3 gene encoding the cryopyrin protein, which leads to the uncontrolled production of interleukin-1β. Particular attention should be paid to the rarity of this disease and the lack of clinical knowledge about it in therapeutic and rheumatological practice, which leads to an erroneous diagnosis and the appointment of ineffective treatment for a long time, leading to the progression of the disease and disability of the patient. Read More

Ann Dermatol Venereol 2022 Jun 27;149(2):123-127. Epub 2021 Oct 27.

Department of Dermatology, CHU Lyon, Université Claude Bernard Lyon I, 69000 Lyon, France. Electronic address:

Background: Eosinophilic annular erythema (EAE) is a rare eosinophil-related skin disease which typically manifests with annular erythematous plaques and severe pruritus. Besides the diagnosis, the treatment of EAE is challenging since relevant published data are sparse.

Methods: The aim of this study was to assess the underlying diseases, treatments and outcomes of patients with EAE. Read More

Ital J Pediatr 2021 Sep 28;47(1):196. Epub 2021 Sep 28.

Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties "G. D'Alessandro", University of Palermo, Palermo, Italy.

Introduction: Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome, also known as Hay-Wells syndrome, is a rare genetic syndrome with ectodermal dysplasia. About 100 patients have been reported to date. It is associated to a heterozygous mutation of the tumor protein p63 (TP63) gene, located on chromosome 3q28. Read More

J Dtsch Dermatol Ges 2021 11 22;19(11):1653-1655. Epub 2021 Sep 22.

Department of Dermatology and Allergy, Hannover Medical School, Hannover, Germany.

J Eur Acad Dermatol Venereol 2022 Jan 5;36(1):e26-e28. Epub 2021 Oct 5.

Department of Dermatology, Cliniques universitaires Saint-Luc, Université catholique de Louvain (UCLouvain), Brussels, Belgium.

JAAD Case Rep 2021 Oct 18;16:33-36. Epub 2021 Aug 18.

Hospital Corporation of America Healthcare/University of South Florida Morsani College of Medicine Graduate Medical Education Programs, Department of Dermatology, Largo Medical Center, Largo, Florida.

Clin Cosmet Investig Dermatol 2021 24;14:1029-1031. Epub 2021 Aug 24.

Department of Dermatology, The Fifth People's Hospital of Hainan Province, Branch of National Clinical Research Center for Skin and Immune Disease, Haikou, Hainan, People's Republic of China.

Wells syndrome (WS), also known as eosinophilic cellulitis, is a rare inflammatory dermatosis of unknown etiology that typically presents with pruritic cellulitis-like plaques. The first line treatment options for WS are topical or systemic corticosteroids, however, the development of side effects of systemic corticosteroids usually led to a switch to the second line therapy. Here, we reported a rare case of facial Wells syndrome misdiagnosed with bacterial cellulitis. Read More

Am J Case Rep 2021 Aug 31;22:e932732. Epub 2021 Aug 31.

Department of Surgery, Evangelismos Hospital, Paphos, Cyprus.

BACKGROUND Wells syndrome is an uncommon inflammatory dermatosis that presents as tender or mildly pruritic cellulitis-like eruptions. The clinical presentation can include papular and nodular eruptions, annular plaques, vesicles, bullae, and urticaria. This syndrome can be promoted by inappropriate eosinophilic stimulation by factors that affect normal eosinophil cellular systems. Read More

Ocul Oncol Pathol 2021 Jun 28;7(3):190-193. Epub 2021 Jan 28.

Department of Ophthalmology, University Hospital of Antwerp, Edegem, Belgium.

We present a case of a woman with a swollen upper eyelid in ptosis, conjunctival nodules, and chemosis present for a couple of weeks. She did not respond to therapy for hordeolum or allergy. A biopsy followed by histopathological examination showed a large infiltration of eosinophilic granulocytes and flame figures, so the diagnosis of Wells syndrome was made. Read More

Int J Dermatol 2022 06 9;61(6):e227-e228. Epub 2021 Jul 9.

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

J Dermatol 2021 Sep 25;48(9):e438-e439. Epub 2021 May 25.

Department of Dermatology, Osaka City University Graduate School of Medicine, Osaka, Japan.

JGH Open 2021 May 9;5(5):629-631. Epub 2021 Mar 9.

Faculty of Health and Medical Sciences, The University of Adelaide Adelaide South Australia Australia.

Cryopyrin-associated Periodic Syndrome (CAPS) is a rare, genetic autoinflammatory condition associated with NLRP3 gene mutations, causing upregulated innate immunity. CAPS manifests as systemic inflammation, causing a constellation of symptoms on a clinical spectrum of phenotypical severity: Familial Cold Autoinflammatory Syndrome being the mildest, Muckle-Wells Syndrome moderate, and Neonatal Onset Multisystem Inflammatory Disease the most severe, with phenotype spectrum overlap. The treatment in Australia for CAPS is interleukin-1 blockade with receptor antagonist, anakinra. Read More

RMD Open 2021 05;7(2)

Division of Pediatric Rheumatology, Department of Paediatrics and Autoinflammation Reference Center Tuebingen, University Hospital Tuebingen, Tübingen, Germany.

Objective: To report the long-term safety and effectiveness of canakinumab, a fully human anti-interleukin 1β monoclonal antibody, in patients with cryopyrin-associated periodic syndromes (CAPS), including familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID), in a real-world setting.

Methods: From December 2009 to December 2015, the β-Confident Registry prospectively enrolled patients with CAPS and non-CAPS conditions who received canakinumab per routine care and were prospectively followed for up to 6 years. The registry protocol did not mandate specific visits or procedures; however, all observed adverse events (AEs) and serious adverse events (SAEs) had to be recorded. Read More

Indian J Nephrol 2021 Jan-Feb;31(1):64-66. Epub 2021 Jan 27.

Department of Nephrology, Manipal Hospitals, Bengaluru, Karnataka, India.

Hearing impairment in a patient with renal failure is an important clue towards etiologic diagnosis of kidney disease. Variety of hereditary diseases, developmental defects, and toxins involve these two organs. However, additional retinopathy is seen in quite a few diseases which include Alport's syndrome and Muckle-Wells syndrome (MWS). Read More