Presence of eosinophil extracellular trap cell death in the affected skin of eosinophilic granulomatosis with polyangiitis.

Eosinophil activation in tissue might be associated with disease severity. Eosinophil cytolysis, a process of active cell death, has been referred to as eosinophil extracellular trap cell death (EETosis). In the present study, the authors investigated EETosis in the affected skin of four patients with eosinophilic granulomatosis with polyangiitis (EGPA) using an immunofluorescence staining method. Immunofluorescence staining for myelin basic protein, galectin-10, and DNA revealed various degrees of EETosis and Charcot-Leyden crystals in skin tissue, suggesting the different degree of eosinophil activation status. The histopathological characteristic features may help physicians establish an earlier diagnosis of intractable eosinophilic-related disease including EGPA. Furthermore, ETotic eosinophil infiltration in perineurium of skin tissue might play a primary role in peripheral neuropathy of this disorder.

Heliotrope-like manifestation of adult-onset Still disease with macrophage activation syndrome: A case-based review.

Adult-onset Still disease (AOSD) has been typically associated with an evanescent skin rash that appears during febrile episodes. Subsequently, reports of a more persistent rash have appeared in the literature, referred to as the atypical rash of AOSD. The atypical nonevanescent rash can be usually divided into dermographism-like, lichenoid, and dermatomyositis-like lesions. Some authors have suggested that AOSD with the atypical rash could be severe, with a poor prognosis. We describe the case of a Japanese woman with AOSD characterized by persistent pruritic lesions resembling those observed in heliotrope manifestation of dermatomyositis. We conducted a literature review of clinical cases of AOSD on MEDLINE and the Web of Science. We identified nine cases of atypical rash of the eyelids, heliotrope-like manifestation of AOSD in addition to our case. All nine patients were female and they had a mean age of 39.3 ± 2.8 years. Four (44.4%) patients had macrophage activation syndrome (MAS) or disseminated intravascular coagulation (DIC) as complications and our case was the only one associated with both MAS and DIC. When a clinician encounters a female patient with heliotrope-like rash resembling those observed in dermatomyositis, the underrecognition of the skin manifestations may result in delayed diagnosis of AOSD. We believe that physicians should identify this type of cutaneous lesion to diagnose AOSD earlier and administer adequate treatment. Although the contribution of tocilizumab to the occurrence of MAS has not been determined, careful observation should be considered during tocilizumab therapy in patients with active AOSD.

Presence of neutrophil extracellular traps in superficial venous thrombosis of Behçet's disease.

Behçet's disease (BD) has a heterogeneous spectrum of disease manifestations featuring the involvement of different organs and can be characterized with different symptoms depending on the clinical department in charge. We retrospectively reviewed BD patients seen at our hospital and investigated the presence of neutrophils producing neutrophil extracellular traps (NET) in those patients. Immunolabeling of myeloperoxidase and histone citrullination proteins was performed on skin biopsies from three BD patients who had skin biopsy-proven superficial vein thrombophlebitis in their erythema nodosum-like lesions. We observed a higher proportion of female patients and a higher incidence of acne-like eruptions among BD patients seen at our dermatology department, while there was a higher incidence of ocular and gastrointestinal involvement among BD patients treated in other departments. We suggest that sex statistical trends could lead to the co-development of different manifestations and may help clinicians choose the best therapeutic approaches, tailoring them to the specific phenotype of the patient rather than one based on single disease manifestations. NET were found in neutrophils of panniculitis concurrent with superficial vein thrombophlebitis. We suggest that the pathogenesis of BD-related thrombosis could be associated with neutrophil activation and NET are released in the panniculitis of affected skin lesions, erythema nodosum-like lesions.

Early add-on administration of mepolizumab and intravenous immunoglobulin effective in treating eosinophilic granulomatosis with polyangiitis.

Treatment of eosinophilic granulomatosis with polyangiitis (EGPA) remains a challenge because currently available therapies, corticosteroids and immunomodulators, do not always control symptoms and are often associated with significant morbidity and relapse. Mepolizumab has been demonstrated to be an effective add-on therapy with steroid-sparing effect in cases of relapsing or refractory EGPA. Intravenous immunoglobulin (IVIG) therapy is effective against mononeuritis multiplex or heart failure in patients with EGPA who do not respond to corticosteroid-cyclophosphamide treatment. We present two cases of EGPA in which earlier add-on administration of adjunct mepolizumab and IVIG led to significant improvement in EGPA symptoms and prevention of flare-up of the disease. We suggested that earlier add-on combination administration of IVIG and mepolizumab might be a useful adjunct treatment to induce clinical remission of EGPA and improve the rate of remission, decrease relapse rate, and allow for reduced glucocorticoid use without any serious adverse drug effects.

Tinea faciei caused by Trichophyton mentagrophytes (molecular type Arthroderma benhamiae ) mimics impetigo : a case report and literature review of cases in Japan.

A 36-year-old female elementary schoolteacher presented with aggregated serous papules surrounded by mild erythema, extending from both nasal wings/nostrils down to the upper lip. No improvement was seen following treatment of the lesions with topical antibiotics for impetigo. Potassium hydroxide (KOH) direct microscopy confirmed the presence of mycelia, and the infection was diagnosed as tinea faciei. The isolate was identified as Trichophyton mentagrophytes using morphological analysis and as Arthroderma benhamiae using genetic analysis. Here we describe that case and summarize the clinical features of other cases of A. benhamiae infection in Japan that have been reported in the literature.