[Multimodal therapy for peristomal pyoderma gangrenosum with topical cyclosporine]. / Multimodale Therapie des peristomalen Pyoderma gangraenosum mit topischem Ciclosporin.

Pyoderma gangrenosum (PG) is a rare neutrophilic disease. Clinically it shows a rapidly evolving painful ulceration with undermined violaceous wound edges. Peristomal PG is particularly resistant to treatment due to mechanical irritation. Two cases illustrate a multimodal therapeutic concept based on topical cyclosporine, hydrocolloid dressing and systemic glucocorticoids. In one patient re-epithelialization was attained after 7 weeks and the other patient experienced downsizing of the wound edges over 5 months.

Clinical guidance of pyoderma gangrenosum 2022.

Pyoderma gangrenosum (PG) is a rare, neutrophilic skin disease. For the purpose of accurate diagnosis and proper treatment of PG, the Japanese clinical practice guidance for PG developed by the Japanese Dermatological Association was published in 2022. In this guidance, clinical aspects, pathogenesis, current therapies, and clinical questions on PG are described from the viewpoints of current knowledge and evidence-based medicine. Here, the English version of the Japanese clinical practice guidelines for PG is presented and is intended to be widely referred to in the clinical examination and treatment of PG.

Osteomielitis aséptica craneal asociada a pioderma gangrenoso / Cranial aseptic osteomyelitis associated with pyoderma gangrenosum

Las dermatosis neutrofílicas (DN) constituyen un grupo heterogéneo de enfermedades. Excepcionalmente, las DN pueden acompañarse de acúmulos de neutrófilos estériles en otros tejidos distintos a la piel. Una paciente de 34 años consultó por una cefalea que no respondía al tratamiento analgésico. Una TAC demostró una lesión osteolítica a nivel parietal derecho, cuyo estudio histopatológico sugería una osteomielitis. Un año después del inicio de la cefalea desarrolló un pioderma gangrenoso en cara anterior de ambas piernas. Tras tratamiento con corticoterapia sistémica se resolvieron las lesiones cutáneas y la cefalea. La afectación ósea en las dermatosis neutrofílicas es excepcional. Habitualmente afecta a la población infantil en el contexto de una osteomielitis crónica recurrente multifocal (OCRM). Solo se han descrito dos casos en adultos, una paciente de 26 años, con una OCRM desde la infancia, y un varón de 67 años que desarrolló una osteomielitis aséptica en continuidad de un pioderma gangrenoso (AU)

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the other one in contiguity with the cutaneous lesions of pyoderma gangrenosum (AU)

Cranial aseptic osteomyelitis associated with pyoderma gangrenosum.

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the other one in contiguity with the cutaneous lesions of pyoderma gangrenosum.

Neuro-Behçet Disease, Neuro-Sweet Disease, and Spectrum Disorders.

Behçet disease and its related disorder, Sweet disease, are multifactorial disorders whose susceptibility loci have been identified in the genes of various immunological factors aside from human leukocyte antigens. The neurological involvement of these diseases, including encephalitis, myelitis, and meningitis, referred to as neuro-Behçet disease (NBD) and neuro-Sweet disease (NSD) respectively, is sometimes difficult to diagnose, especially when the characteristic mucocutaneous symptoms do not precede neurological symptoms or when characteristics of both diseases are present in a single patient. NBD and NSD constitute a spectrum of diseases that are differentiated according to the combination of risk factors, including the genetic background. Encephalitis, myelitis, and meningitis similar to NBD or NSD can be diagnosed as spectrum disorders, even if the characteristic mucocutaneous symptoms fail to be detected. Understanding these conditions as a disease spectrum may help elucidate the disease pathogenesis and assist in the development of therapeutic agents.

Cranial aseptic osteomyelitis associated with pyoderma gangrenosum. / Osteomielitis aséptica craneal asociada a pioderma gangrenoso.

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the other one in contiguity with the cutaneous lesions of pyoderma gangrenosum.

The great imitator with no diagnostic test: pyoderma gangrenosum.

Pyoderma gangrenosum is a rare, neutrophil-mediated, auto-inflammatory dermatosis that wound care specialists must be prepared to recognise. This condition has clinical features analogous to infectious processes. There is no specific diagnostic test and the diagnosis is usually obtained from exclusion. Its early recognition and proper management with prompt initiation of immunosuppressive therapy are essential to improve the quality of life and the prognosis of patients.

A case of seronegative longitudinally extensive transverse myelitis with possible neuro sweet disease.

•Neuro sweet disease (NSD) rarely exhibits spinal cord involvement.•We experienced a case of NSD with longitudinally extensive transverse myelitis.•The shape of gadolinium enhanced lesion of spinal MRI was characteristic.•Above mentioned finding might be useful for diagnosis of neuro sweet disease.

T-cell Lymphoma Presenting Neutrophilic Inflammation in the Cerebrospinal Fluid.

A 66-year-old woman presented with upper abdominal pain and weakness in the limbs. She had bilateral uveitis and gastric ulcers. A neurological examination revealed tetraparesis and sensory disturbance in the right arm. A cerebrospinal fluid (CSF) examination showed polymorphonuclear pleocytosis with elevated pro-inflammatory cytokine levels. Magnetic resonance imaging showed brain lesions and a long spinal cord lesion. She was initially diagnosed with neuro-Behçet's disease and was treated with corticosteroids, resulting in no improvement. A gastric mucosa biopsy indicated T-cell lymphoma colocalizing with neutrophils. The cytokine-mediated neutrophilic inflammation probably caused characteristic CSF and histopathological features. It is noteworthy that T-cell lymphoma may present with CSF neutrophilic inflammation.

[The etiology, diagnosis, and treatment of neurological complications in Behçet disease and its related disorder Sweet disease].

Behçet disease, and its related disorder Sweet disease, are multisystem inflammatory conditions characterized by muco-cutaneous symptoms. When neuropsychiatric symptoms appear, the two conditions are referred to as neuro-Behçet disease and neuro-Sweet disease. While diagnosing these conditions according to their diagnostic criteria, muco-cutaneous symptoms must be observed; however, neuropsychiatric symptoms may precede muco-cutaneous symptoms. In these conditions the dysregulation of cytokines, following the onset of oral muco-cutaneous bacterial infection, may induce an abnormal chemotaxis of neutrophils causing ectopic encephalitis and meningitis. Thus, an initial treatment targeting neutrophils should be considered based on the diagnosis of neuro-neutrophilic disease when symptoms indicating neutrophil hyperactivity are observed, even without muco-cutaneous symptoms. In addition to human leukocyte antigen-B51 and -A26, genome-wide association analyses have identified new susceptibility loci on the genes of various immunological factors in Behçet disease. These findings may help elucidate disease pathogenesis and assist the development of diagnostic modalities and therapeutic agents for neuro-neutrophilic disease.

Vasculitis with superior ophthalmic vein thrombosis compatible with neuro-neutrophilic disease.

PURPOSE: To present a unique case of neuro-neutrophilic disease with inflammation and thrombosis of the superior ophthalmic vein (SOV). OBSERVATIONS: A 43-year-old Japanese man with past histories of oculomotor paralysis, auditory disorder, ischemic enteritis, and recurrent oral ulceration was referred to our hospital because of blurred vision in his right eye. Ophthalmic examination revealed decreased best corrected visual acuity and central scotoma in his right eye. Orbit magnetic resonance imaging (MRI) revealed an enlarged SOV in the right eye, with Gadolinium (Gd) enhancement in the wall of the vein but not inside the vein, indicating thrombosis. Multiple Gd-enhanced hyperintense lesions were also observed in the juxtacortical area of the brain. We diagnosed the patient with vasculitis in the right SOV that was adversely affecting the optic nerve. We ruled out systemic thrombophilia, infections, and malignancy by systemic examinations. The human leukocyte antigen (HLA) typing was Cw1-, B54-, B61-, A2-, A24-, and DR4-positive and B51-negative. We treated the patient with systemic steroid and anticoagulant therapy. After three courses of steroid pulse therapy, his symptoms and the MRI findings of the right SOV and brain improved; therefore, we decided to discontinue the anticoagulant therapy. One month after anticoagulant cessation, MRI revealed recurrence of the thrombus and enlargement of the right SOV despite the lack of vision worsening. We restarted the anticoagulant therapy while continuing the oral prednisolone treatment. At the final visit, 14 months after the onset of the disease, the patient was still receiving oral anticoagulation with warfarin potassium and prednisolone (5 mg/day). His symptoms and the right eye's visual function remained normal with a mildly enlarged SOV; there was less Gd enhancement and no brain lesions on MRI. CONCLUSIONS AND IMPORTANCE: We treated a unique case of possible neuro-neutrophilic disease that presented visual disturbances due to right SOV inflammation and thrombosis. Anticoagulation and systemic steroid therapies were required to reduce the inflammation and to prevent the recurrence of thrombosis.

The impact of underlying disease state on outcomes in patients with pyoderma gangrenosum: A national survey.

BACKGROUND: Whether the underlying disease affects the outcomes in pyoderma gangrenosum (PG) is unclear. OBJECTIVES: To determine the impact of comorbid disease associations and concomitant procedural treatments on patient outcomes in hospitalizations of patients with PG. METHODS: A cross-sectional analysis of the National Inpatient Sample for hospitalizations of patients with PG from the years 2002 to 2011, analyzing in-hospital mortality rate and health care resource utilization. RESULTS: Inflammatory bowel disease was the most frequent comorbid association, followed by inflammatory arthritis, hematologic malignancies/dyscrasia, and vasculitis. Multivariable modeling showed that vasculitis and hematologic malignancy/dyscrasia, when compared with inflammatory bowel disease, were associated with a 4-fold to 6-fold increased risk of in-hospital mortality and increasing health care resource utilization. Inpatient procedural interventions, including skin grafts, biopsies, and debridement, did not affect mortality and were associated with an increased length of stay. LIMITATIONS: The database does not account for outpatient follow-up; additionally, there was a low rate of coded comorbid conditions. CONCLUSIONS: Comprehensive evaluation to determine the underlying comorbidity for patients with PG is important for patient risk stratification.

Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation.

We report 2 cases that were considered to be neuro-Sweet disease. They initially manifested with meningoencephalitis and no skin lesions, and rapidly improved with corticosteroid therapy. In both cases, patients complained of meningitic symptoms such as fever and headache, and HLA-B54 and -Cw1 turned out to be positive over the clinical course. Cerebrospinal fluid analysis showed increased levels of lymphocytes and protein. In case #1, fluid-attenuated inversion recovery (FLAIR), magnetic resonance imaging (MRI) and diffusion-weighted images (DWI) showed high-intensity signals in the right dorsal medulla oblongata, bilateral dorsal midbrain, and left thalamus. In case #2, FLAIR and DWI showed high-intensity signals in the bilateral cerebellar cortex and left caudate nucleus. Symptoms and MRI images were markedly improved in both cases after corticosteroid pulse therapy. According to published diagnostic criteria, these 2 cases were considered possible neuro-Sweet disease. These cases suggest that the combination of meningoencephalitis and HLA specificity is important to consider the possibility of neuro-Sweet disease, even without skin lesions.