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J Cutan Pathol 2021 03 20;48(3):343-346. Epub 2021 Jan 20.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

Lupus 2021 Mar 8;30(3):473-477. Epub 2020 Dec 8.

Sorbonne Université, Medecine, Service de dermatologie et allergologie, APHP, Hôpital Tenon, Paris, France.

Introduction: Kikuchi-Fujimoto disease (KFD) is a self-limited histiocytic necrotizing lymphadenitis sometimes affecting the skin. "Kikuchi disease-like inflammatory pattern" (KLIP) has been described in cutaneous lesions as similar pathological features in patients without lymph node involvement and as a potential clue for the diagnosis of lupus. We aimed to describe KLIP-associated clinical and immunological features in lupus patients with a retrospective case-control study. Read More

Intern Med 2021 Apr 16;60(8):1311-1315. Epub 2020 Nov 16.

Department of General Internal Medicine, Hiroshima University Hospital, Japan.

We herein report a case of aortitis induced by granulocyte colony-stimulating factor (G-CSF) that coincided with lung injury, splenomegaly, and cutaneous manifestations during treatment for recurrent extraosseous mucinous chondrosarcoma. Computed tomography revealed large-vessel vasculitis, splenomegaly, and pulmonary interstitial changes. Treatment with prednisolone was successful. Read More

Dermatol Online J 2020 Aug 15;26(8). Epub 2020 Aug 15.

Geisel School of Medicine at Dartmouth College, NH Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, NH.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare disorder that must be distinguished from systemic lupus erythematosus (SLE). Although a minority of patients with KFD develop SLE, most patients have a self-limited disease. Importantly, KFD can have skin manifestations resembling cutaneous lupus. Read More

Rheumatology (Oxford) 2021 02;60(2):617-628

NDORMS, Rheumatology Department, University of Oxford, Oxford, UK.

Objectives: ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- and older-onset patients are still incompletely understood.

Methods: We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. Read More

JAMA Oncol 2020 07 9;6(7):e201250. Epub 2020 Jul 9.

Department of Respiratory Medicine and Medical Oncology, Yokohama Municipal Citizen's Hospital, Yokohama, Japan.

Importance: Although the efficacy of epidermal growth factor receptor tyrosine kinase inhibitors for EGFR gene mutation-positive non-small cell lung cancer is well established, optimal dosing remains to be established, especially in elderly or frail patients.

Objective: To investigate the efficacy and safety of low-dose erlotinib in elderly or frail patients with EGFR mutation-positive non-small cell lung cancer.

Design, Setting, And Participants: Single-arm phase 2 trial with the Southwest Oncology Group (SWOG) 2-stage design that enrolled frail patients from 21 Japanese institutions after meeting the inclusion criteria. Read More

Cureus 2020 Mar 18;12(3):e7312. Epub 2020 Mar 18.

Department of Dermatology, College of Medicine, King Saud University, Riyadh, SAU.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a benign, self-limiting disorder with unknown etiology. The most frequent clinical manifestations include lymphadenopathy, fever, cutaneous lesions, arthritis, fatigue, and hepatosplenomegaly. Cutaneous manifestations of KFD, occurring in about one-third of patients, are typically non-specific, rarely presenting as symmetrically distributed lesions. Read More

Parasitol Int 2020 Apr 10;75:102036. Epub 2019 Dec 10.

Department of Infectious Diseases, Division of Parasitology, Faculty of Medicine, University of Miyazaki, Japan. Electronic address:

Proliferative sparganosis is one of the most bizarre and mysterious parasitic diseases ever described. The causative parasite is Sparganum proliferum, which is a pseudophyllidean cestode distinct from Spirometra tapeworms. Here we overview this rare but fascinating disease with the all original case reports on human patients published in the last 115 years. Read More

J Clin Rheumatol 2021 Apr;27(3):e96-e97

Department of Rheumatology, University Medical Center.

Actas Dermosifiliogr (Engl Ed) 2019 Nov 4;110(9):710-727. Epub 2019 Jun 4.

Servicio de Anatomía Patológica, Hospital El Bierzo, Ponferrada, León, España; Instituto de Investigación Biomédica de A Coruña, Grupo de Investigación CellCOM-SB, A Coruña, España; Servicio de Anatomía Patológica, Hospital de la Reina, Ponferrada, León, España. Electronic address:

The skin is the largest and most exposed organ in the human body and the ideal place to look for signs that aid in the early diagnosis of systemic diseases with cutaneous effects. As the concepts that underpin our understanding of many of these diseases have evolved or expanded in recent years, there have also been changes in the criteria we use for early diagnosis, including our approaches to skin biopsy and dermatopathologic evaluation. This review focuses on some of the systemic processes with skin manifestations for which our basic understanding has changed most in recent decades. Read More

Rheumatology (Oxford) 2019 03;58(3):554-556

Department of Internal Medicine, APHP, Saint Antoine Hospital, Sorbonne Université, France.

Indian J Pathol Microbiol 2018 Jan-Mar;61(1):113-115

Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Wardha, Maharashtra, India.

Kikuchi's disease (KD) also known as histiocytic necrotizing lymphadenitis is rare, idiopathic, generally self-limited cause of lymphadenitis. We present a case of twenty year young female who presented in critically ill state with fever, cervical and axillary lymphadenopathy, rash, vomiting and altered sensorium and found to have neurological, hepatic, renal and dermatological involvement. Kikuchi's disease should be considered in differential diagnosis of fever and lymphadenopathy and though benign can sometimes present with multi-organ involvement. Read More

Clin J Gastroenterol 2017 Oct 3;10(5):420-425. Epub 2017 Aug 3.

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

Cutaneous metastasis of an internal malignancy is uncommon and is estimated to occur in 0.7-9% of patients with internal cancer including autopsy cases. We would like to report a case of long survival of sigmoid colon adenocarcinoma diagnosed as an instance of facial cutaneous metastasis. Read More

Eur J Dermatol 2017 04;27(2):212-213

Department of Dermatology.

Arthritis Care Res (Hoboken) 2016 12 21;68(12):1883-1887. Epub 2016 Oct 21.

Centre Hospitalier Universitaire Pierre Zobda Quitman, Fort de France, Martinique.

Objective: To provide an epidemiologic description of Kikuchi-Fujimoto disease (KFD), and to describe its relationship with systemic lupus erythematosus (SLE) in a population of sub-Saharan origin.

Methods: Patients were retrospectively included on the basis of lymph node histology compatible with KFD reported in Martinique from 1991 until 2013. In order to describe the characteristics of the disease in a larger cohort, we subsequently included more patients of Afro-Caribbean origin from Guadeloupe and French Guiana. Read More

Int J Dermatol 2016 Oct 21;55(10):1069-75. Epub 2016 May 21.

Dermatology, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA.

Kikuchi-Fujimoto disease is a benign, self-limiting condition characterized by lymphadenopathy and fever. Its diagnosis can be challenging, as the disease must be differentiated from lymphoma and systemic lupus erythematosus. Patients can present with nonspecific cutaneous lesions. Read More

Eur J Dermatol 2016 Apr;26(2):208-9

Department of Dermatology The Jikei University School of Medicine 3-25-8 Nishishimbashi, Minato-ku, Tokyo, JAPAN 106-8461.

Mod Rheumatol 2018 Mar 4;28(2):369-372. Epub 2016 Mar 4.

a Nephrology Center, Toranomon Hospital , Japan.

A 69-year-old Japanese man was presented with hypertensive crisis. Renal histology revealed malignant nephrosclerosis, including an onion skin pattern with fibrinoid necrosis of the small arteries from arterioles up to interlobular arteries. Immunological investigation clarified positive anti-RNA polymerase (RNAP) III antibody, and limited cutaneous systemic sclerosis (Lc SSc) was diagnosed by skin biopsy as the underlying disease causing scleroderma renal crisis (SRC). Read More

BMC Pulm Med 2016 Feb 11;16:30. Epub 2016 Feb 11.

Department of Respiratory Medicine, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.

Background: Paradoxical inflammations during anti-TNF-α therapy are defined as adverse effects such as psoriasiform skin lesions, uveitis and sarcoidosis-like granulomas induced by immune reactions, not by infectious agents. Here, we report a very rare case of the simultaneous development of sarcoidosis and cutaneous vasculitis in a patient with refractory Crohn's disease during infliximab therapy and both of which resolved spontaneously without the cessation of infliximab.

Case Presentation: In September 2000, 23-year old Japanese male was diagnosed with Crohn's disease. Read More

Am J Dermatopathol 2016 Jun;38(6):e77-80

*Division of Dermatology, Department of Medicine, University of Washington, Seattle, WA; †Division of Hematology, Department of Medicine, University of Washington, Seattle, WA; ‡Division of Hematopathology, Department of Pathology, University of Washington, Seattle, WA; and §Division of Dermatopathology, Department of Pathology, University of Washington, Seattle, WA.

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a variant of cutaneous T-cell lymphoma that can mimic inflammatory panniculitis, particularly lupus erythematosus panniculitis, clinically and histologically. Patients with SPTL generally have a benign course, though SPTL can be complicated by hemophagocytic syndrome, which can negatively impact prognosis. We report a patient with recurrent SPTL and hemophagocytic syndrome accompanied by a necrotizing lymphadenitis consistent with Kikuchi-Fujimoto disease. Read More

Medicine (Baltimore) 2015 Nov;94(46):e2065

From the Department of Internal Medicine (L-HT, BG, NL); Department of Dermatology (SI-H-O, PW, OC); Department of Pathology (NO), AP-HP, CHU Henri-Mondor, UPEC University; EA EpiDermE (Epidemiology in Dermatology and Evaluation of Therapeutics), INSERM, CIC 1430 (SI-H-O, PW, OC); INSERM U955 team 9, Créteil (NO); C. Cap Orléans Laboratory, Orléans (LR, JK); and Department of Dermatology, CHG de Dreux, Dreux, France (VP).

Kikuchi-Fujimoto disease (KFD) is a rare and benign disorder that usually occurs in young adults with enlarged lymph nodes containing infiltrate of cytotoxic T cells and nuclear debris. It can be a manifestation of systemic lupus erythematosus (SLE) although the strength of this association has varied among studies. Although specific KFD cutaneous lesions are well described, pure cutaneous lesions have never been reported. Read More

An Bras Dermatol 2015 Mar-Apr;90(2):245-7

Hospital de Braga, Braga, Portugal.

Kikuchi's disease is a benign, self-limiting disease, whose pathogenesis remains unknown. Patients most often present with cervical lymphadenopathy, sometimes associated with fever and leukopenia. It has been reported that up to 40% of patients with Kikuchi's disease have also cutaneous eruptions, but no specific skin changes have been described. Read More

J Immunol 2015 Feb 19;194(4):1996-2003. Epub 2015 Jan 19.

Division of Gene Therapy Science, Graduate School of Medicine, Osaka University, Suita 565-0871, Japan;

Recessive dystrophic epidermolysis bullosa (RDEB) is an intractable genetic blistering skin disease in which the epithelial structure easily separates from the underlying dermis because of genetic loss of functional type VII collagen (Col7) in the cutaneous basement membrane zone. Recent studies have demonstrated that allogeneic bone marrow transplantation (BMT) ameliorates the skin blistering phenotype of RDEB patients by restoring Col7. However, the exact therapeutic mechanism of BMT in RDEB remains unclear. Read More

Rheumatol Int 2015 Jun 25;35(6):1111-3. Epub 2014 Nov 25.

Department of Dermatology, Kitasato University School of Medicine, 1-15-1 Kitasato, Sagamihara, Kanagawa, 252-0374, Japan,

J Dermatol 2014 Aug 9;41(8):736-8. Epub 2014 Jul 9.

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

We report a 38-year-old male patient who presented with cutaneous lesions mimicking widespread discoid lupus erythematosus with high-grade fever, arthralgia and lymphadenopathy. Additional lymph node and skin biopsies, however, revealed karyorrhectic debris without neutrophils and numerous CD68-positive cells, a characteristic finding of Kikuchi's disease (KD). Comparing skin biopsies on different occasions, we could see different forms of histopathology. Read More

J Dermatol 2014 May 15;41(5):407-10. Epub 2014 Mar 15.

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

Typical skin rash, which appears and disappears along with respective rise and fall of fever, is well-known, and included as one of the major criteria of adult-onset Still's disease (AOSD) (Yamaguchi's criteria). In addition, various skin lesions are occasionally observed in association with AOSD. Persistent pruritic eruptions present with some clinical features, such as urticarial erythema, flagellate erythema, erythematous, slightly scaly or crusted papules, and/or plaques on the trunk and extremities. Read More

Acta Derm Venereol 2014 Nov;94(6):729-30

Department of Dermatology, Sapporo City General Hospital North 11, West 13, Chou-ku, Sapporo 060-8604, Japan.

Dermatol Pract Concept 2014 Jan 31;4(1):47-9. Epub 2014 Jan 31.

Pathology Unit, Arcispedale Santa Maria Nuova-IRCCS, Reggio Emilia, Italy.

Kikuchi-Fujimoto disease (KFD) is an uncommon clinicopathological entity characterized by fever and lymphadenopathy, predominantly involving cervical lymph nodes, accompanied by chills and leukopenia. The diagnosis relies primarily on the presence of typical morphological features in the swelling lymph nodes. KFD can occur as a benign and self-limiting lymphadenopathy, but it can sporadically precede, postdate or coincide with the diagnosis of systemic lupus erythematosus (SLE). Read More

Am J Clin Pathol 2013 Apr;139(4):536-51

AS-255C, UCLA Medical Center, CHS, 10833 LeConte Ave, Los Angeles, CA 90095, USA.

The Society for Hematopathology and European Association for Haematopathology workshop, from October 27 to 29, 2011, in Los Angeles, CA, exhibited many exemplary skin biopsy specimens with interesting inflammatory changes mimicking features of cutaneous lymphoma. This article reviews features observed in cutaneous lymphoid hyperplasia, cutaneous drug reactions, lupus-associated panniculitis, pityriasis lichenoides, hypereosinophilic syndrome, histiocytic necrotizing lymphadenitis, traumatic ulcerative granuloma with stromal eosinophils, and pigmented purpuric dermatosis, as well as a brief review of the pertinent literature and discussion of submitted conference cases. For the pathologist, it is important to be aware of diagnostic pitfalls as well as the limitations of ancillary testing (eg, clonality studies). Read More

Rev Med Interne 2013 Jun 29;34(6):373-6. Epub 2012 Nov 29.

Service de médecine interne A, hôpital Lariboisière, université Paris Cité-Diderot, AP-HP, 2, rue Ambroise-Paré, 75010 Paris, France.

Introduction: Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) is a rare clinical entity characterized by the association of enlarged lymph nodes in the posterior cervical region and fever. The disease is more frequent in young women.

Case Report: We report a 41-year-old African patient who presented with atypical features of Kikuchi's disease including cutaneous lupus, haemophagocytosis, and lymphocytic meningitis. Read More