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
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
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
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
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
*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
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
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
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
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
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
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
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
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
An 89-year-old man with advanced renal failure, polymyalgia rheumatica and a past history of tuberculosis was admitted with a high fever. Erythema and swelling appeared in the femoral region. Since the cellulitis failed to respond to antibiotic therapy, a skin biopsy was performed. Read More
We report the case of a 45-year-old female patient previously diagnosed with subacute cutaneous lupus erythematosus who presented with a one-week history of fever, tender erythematous nodules on her limbs, and palpable lymphadenopathy. Two incisional biopsies showed histiocytic infiltrates with abundant nuclear debris in the dermis and at the dermosubcutaneous junction with absence of neutrophils, characteristic of Kikuchi-Fujimoto disease (KFD). The dermatologic and dermopathologic details of KFD are very heterogeneous and yet poorly described. Read More
Adult-onset Still's disease (AOSD) is a systemic inflammatory condition, characterized by a high spiking fever, leukocytosis with neutrophilia, arthralgia, and skin rash. Typical skin rash is an evanescent, salmon-pink erythema predominantly involving extremities, which is included as one of the diagnostic criteria; however, recent findings show that not only typical evanescent rash but also various skin lesions are associated with AOSD. The representative characteristic skin lesion among the non-classical skin rash is called persistent pruritic papules and plaques, which presents erythematous, slightly scaly papules with linear configuration on the trunk. Read More
Histiocytic necrotizing lymphadenitis (HNL), or Kikuchi's disease, is a benign and self-limiting lymphadenopathy that typically affects young Asian females. It presents with lymphadenopathy, usually cervical, accompanied by fever, chills and leukopenia. Although the association between systemic lupus erythematosus (SLE) and HNL is rare, the number of reports of HNL in SLE patients is increasing. Read More
Erythrasma is a superficial skin disease caused by Gram-positive Corynebacterium species. Coral-red fluorescence under Wood's light, strongly suggestive of erythrasma, can be attributed to the presence of porphyrins. Fractionated porphyrin analysis in erythrasma lesions is yet to be reported. Read More
Kaposi sarcoma is an acquired immunodeficiency syndrome-related disease that mainly involves the skin, gastrointestinal gut, and lungs. Whole-body 18F-fluorodeoxyglucose-positron emission tomography and computed tomography (FDG-PET/CT) scanning is useful for simultaneous detection of multiple lesions of Kaposi sarcoma. We present a 67-year-old man with a history of infection with human immunodeficiency virus who presented with numerous cutaneous lesions. Read More
We report a case of a 63-year-old woman who developed acute right heart failure and an achalasia-like syndrome with limited cutaneous systemic sclerosis (lcSSc) and primary biliary cirrhosis. Intravenous administration of diuretics improved her acute heart failure. Anti-centromere antibodies and anti-mitochondria antibodies were present. Read More
The skin is the second most frequently affected organ system in lupus erythematosus. Although only very rarely life threatening--an example is lupus erythematosus-associated toxic epidermal necrolysis--skin disease contributes disproportionally to disease burden in terms of personal and psychosocial wellbeing, vocational disability, and hence in medical and social costs. Since several manifestations are closely associated with the presence and activity of systemic lupus erythematosus, prompt and accurate diagnosis of cutaneous lupus erythematosus is essential. Read More
Kikuchi's disease (KD), or histiocytic necrotizing lymphadenitis, is a rare, self-limited lymphadenopathy, typically in young women, that usually remits spontaneously and does not recur. KD is clinically characterized by cervical lymphadenopathy and a high fever. Extranodal involvement (skin, arthritis, meningitis) rarely occurs. Read More
A WHO position paper states that allergen immunotherapy is an effective treatment for allergic diseases, and well characterized allergens should be used in immunotherapy. The house dust mite is a major cause of allergic disease. However, the biological activity of the mite extracts currently used cannot be clearly determined, since these extracts contain various impurities. Read More
Kikuchi's disease is a self-limited necrotizing lymphadenitis that is characterized by cervical lymphadenopathy and fever. Although it has been reported that some patients with Kikuchi's disease have cutaneous manifestations, the specific skin changes of patients with Kikuchi's disease have not been fully described. We report here on 16 patients of Kikuchi's disease with cutaneous manifestations. Read More
Varicella pneumonia is one of the serious complications of primary varicella zoster virus (VZV) infection in adults. A 36-year-old woman with end-stage renal disease underwent renal transplantation from a living donor in 1998, receiving immunosuppressive treatment with cyclosporine, mycophenolate mofetil, and methylprednisolone. She had a history of VZV infection during childhood. Read More
Histologic examination of lesions plays a key role in the diagnostics of cutaneous lupus erythematosus (LE). LE has a broad spectrum of histopathological signs, which are related to the stages of the lesions. In addition to the main subtypes of LE, we report on special manifestations like Rowell's-syndrome and Chilblain LE, and give an account of Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis), which may be associated with systemic LE. Read More
Kikuchi's disease (KD) is a self-limiting histiocytic necrotizing lymphadenitis (HNL). Cutaneous manifestations are frequent and usually show histopathological findings similar to those observed in the involved lymph nodes. HNL with superposed histological features to KD has been described in patients with lupus erythematosus (LE), and a group of healthy patients previously reported as having HNL may evolve into LE after several months. Read More
A 43-year-old woman presented with a persistent high fever of 39 degrees C and edematous erythema accompanied by pustules on the face, trunk and extremities. Conjunctivitis and nodules were also observed in the right eye. On the basis of the clinical symptoms and histopathological findings. Read More
Kikuchi's disease, also known as histiocytic necrotizing lymphadenitis, is a systemic illness with classic clinical findings of cervical lymphadenopathy and fever. Diagnosis is confirmed by lymph node histology, which reveals paracortical foci of necrosis and a histiocytic infiltrate. Kikuchi's disease has been associated with a number of infections, but no single source has been identified. Read More
We examined the possibility of expanding applications of rasH2 mice, which are genetically manipulated mice for short-term carcinogenicity tests, to percutaneous application. A 26-week short-term carcinogenicity study was performed on a total of 300 mice including 75 male and female rasH2 mice each, and 75 male and female non-Tg mice each from the same litter as the rasH2 mice divided into untreated group, an ethanol group, a white Vaseline group, an acetone group, and a phorbol 12-myristate 13-acetate (TPA) group. Only shaving of dorsal skin was performed on the untreated mice. Read More
Kikuchi's disease (KD) is a necrotizing lymphadenopathy with a self-limiting course commonly affecting young people. We conducted a clinico-pathological study of 20 cases of KD received at our department between July 2000 and December 2003. Our study confirmed that KD commonly involves the cervical lymph nodes of young adults, with a female predominance. Read More
The antibiotic-induced eruption of infectious mononucleosis is a well-known clinical phenomenon. Latent viral infection with herpesviridae (eg, human herpes virus 6 (HHV-6) and Epstein-Barr virus (EBV)) is suspected to play a role in the drug hypersensitivity syndrome. The cutaneous pathologic findings have not been reported in the former, and are infrequently reported in the latter entity. Read More
Background: Kikuchi-Fujimoto's necrotizing lymphadenitis (KFNL) is a rare, benign, self-limited condition characterized by constitutional symptoms, lymphadenopathy, and skin lesions.
Objective: We report a case of KFNL in a 43-year-old East Indian woman with a ten-year history of discoid lupus erythematosus (DLE) of the scalp and a three-month history of a erythematous plaque on the left nasal bridge, cervical lymphadenopathy, and fever. Skin biopsy samples were taken from the face and lymph node. Read More
Background: Basic fibroblast growth factor (bFGF) and vascular endothelial growth factor (VEGF) are angiogenic cytokines, that have been reported to elevate serum levels in patients with collagen diseases such as systemic sclerosis and dermatomyositis as well as in those with inflammatory bowel diseases.
Objective: In this study, the serum levels of bFGF and VEGF were measured by using enzyme-linked immunosorbent assay in 20 patients with polyarteritis nodosa (PAN; 5 with systemic PAN and 15 with cutaneous PAN) and in 20 control subjects. We also investigated the expression of bFGF and VEGF in cutaneous lesions of patients by using immunohistochemical methods. Read More
Introduction: Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto's disease is a rare anatomoclinical entity whose etiology remains unknown. It is mainly reported in young adult female, presenting with cervical lymphadenopathies, fever and asthenia. The diagnosis is based on the histological examination of a lymph node biopsy. Read More
Background: Chronic urticaria is caused by a complement fixing, IgG antibody directed to the alpha-subunit of the IgE receptor, which is present in 35% to 45% of patients. This autoimmune subgroup can be identified by an autologous skin test or histamine release from human basophils or cutaneous mast cells. However, binding assays do not correlate with these functional assays. Read More
Background: Acne vulgaris is a chronic inflammatory disease involving colonization of Propionibacterium acnes (P. acnes), activation of neutrophils and lymphocytes. Circumstantial evidence suggests that antigen-independent and -dependent immune responses against P. Read More
Therapeutic administration of efalizumab, a humanized antibody to CD11a, induces a marked but reversible increase of peripheral lymphocytes in psoriasis patients. In this study, 13 patients were treated with 12 weekly subcutaneous doses (2 mg/kg/week) of efalizumab, and all 13 patients had increases in leukocyte counts. This increased white blood cell count was mainly due to a 3- to 4-fold increase in the number of circulating CD3(+) lymphocytes during active treatment. Read More
Necrotizing histiocytic lymphadenopathy (Kikuchi's disease) is a rarely observed clinical entity characterized by fever, and solitary or multiple lymphadenopathy predominantly in the posterior cervical region. Kikuchi's disease has been reported to precede, coexist with or follow the diagnosis of systemic lupus erythematosus. In only rare instances has its association with cutaneous lupus erythematosus without systemic involvement been reported. Read More