Febrile ulceronecrotic Mucha-Habermann disease - a case and treatment review.

Febrile ulceronecrotic Mucha-Habermann disease is a rare and severe variant of pityriasis lichenoides, characterized by sudden onset of generalized ulceronecrotic papules that rapidly coalesce into ulcers associated with high fever. Systemic manifestations such as intravascular disseminated coagulation and pulmonary, cardiac, gastrointestinal, and central nervous system involvement are common. Treatment is based on oral corticosteroids, immunosuppressive drugs such as methotrexate, and general supportive treatment. The present case describes a stepwise approach to a patient with Mucha-Habermann disease with insufficient response to methotrexate.

Primary Central Nervous System Lymphoma Complicated by Cerebral Venous Sinus Thrombosis: A Case Report.

Central nervous system (CNS) lymphoma is a rare and aggressive primary neoplasm that comprises a small proportion of brain tumours and non-Hodgkin lymphomas. We present a case report of a 64-year-old woman with CNS lymphoma, who exhibited cognitive changes, weight loss and neurological symptoms. Imaging scans revealed multiple lesions in the brain and thrombosis in the venous sinuses. A diagnosis of diffuse large B-cell lymphoma of the CNS was confirmed through histological examination. The patient underwent treatment with corticosteroids and chemotherapy, but experienced clinical deterioration with thrombocytopenia and disease progression. Despite efforts to manage complications and provide targeted therapy, the patient passed away. Primary CNS lymphoma typically responds well to chemotherapy, and prognostic factors such as age and functional status play a significant role in patient outcomes. However, complications such as thromboembolism pose challenges during treatment due to the hypercoagulable state induced by chemotherapy agents. The pathophysiology of thromboembolic events in the context of malignancy remains uncertain but may involve direct tumour compression, vascular invasion and alterations in coagulation factors. The diagnostic process for CNS lymphoma can be complex, and the information obtained from cerebrospinal fluid analysis, including flow cytometry, may be limited in cases with low cell counts. Ongoing research exploring genetic tests and biomarkers shows promise for improving diagnostic accuracy in such cases. This case underscores the need for comprehensive management strategies that address both the neoplasm and its associated complications, to optimise patient outcomes. LEARNING POINTS: Primary CNS lymphoma is a rare primary neoplasm, being even rarer in immunocompetent patients.In 25% of all cases of CNS lymphoma, it is complicated by cerebral venous thromboembolism.Leptomeningeal spread can occur with or without MRI evidence and is diagnosed with cerebrospinal fluid (CSF) flow cytometry. Age and functional status are important prognostic factors.

Chorea as a Manifestation of Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease with multisystemic manifestations, including central nervous system involvement. Chorea is a hyperkinetic movement disorder, characterized by involuntary, dance-like and poorly coordinated movements. Acute-onset chorea is a rare neuropsychiatric inaugural manifestation of SLE. This presentation is frequently associated with positive antiphospholipid antibodies, and it usually improves with immunosuppressive treatment. We report the case of a 20-year-old female, who presented with acute onset left hemichorea and fever. Analysis showed active urine sediment. A detailed anamnesis and evaluation revealed several clinical manifestations suggestive of SLE with multiorgan involvement: neurological, renal, cardiac, hematological, joint and mucocutaneous. This case emphasizes the importance of keeping a high clinical awareness for rarer presentations of common autoimmune disorders, such as SLE, which can be severe and should be promptly treated. Furthermore, the relevance of SLE in the differential diagnosis of acute-onset movement disorders in young patients is highlighted in this report.