Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a benign condition of the lymphatic tissue whose cause has not been fully elucidated. Although it has been thought that various viral infections are involved in the onset of the disease, its immunological characteristics expressed by the proliferation of cytotoxic CD8-positive T lymphocytes and histiocytic phagocytosis place it at the border of autoimmunity. Sharing immunological mechanisms, clinical manifestations, and epidemiological data (both are described in young women) with systemic lupus erythematosus (SLE), the association of the two pathologies represents a challenge for both clinicians and pathologists.
We present the case of a young woman with SLE with immunological, haematological and articular involvement, who associates a few months after diagnosis the significant swelling of the submandibular glands and latero-cervical lymphadenopathy. Histopathological and immunohistochemical examinations subsequently certify the diagnosis of histiocytic necrotizing lymphadenitis associated with the autoimmune disease.
Pneumosinus dilatans (PSD) is a rare disorder that causes aberrant paranasal sinus dilation. Pneumosinus dilatans is typically benign and asymptomatic, without any evidence of bone destruction or pathologic changes in the underlying mucosa. Localized discomfort, headaches, facial paraesthesia and deformities, sensory deficits, olfactory loss, and ocular abnormalities are common complaints. The diagnosis of PSD is confirmed by the radiological investigations. The surgical treatment’s objective is to return both form and function of the affected sinus to normal.
We briefly present our experience with pneumosinus dilatans and discuss the characteristics of this disease based on the specialised literature.
Rhinoscleroma is a chronic infectious condition found mainly in patients living in underdeveloped regions. It affects the respiratory tract (in 95% of the cases the nasal cavity is the first affected), but it can also involve the orbit, the middle ear, the lip, the gums and the cutaneous tissue. It is linked to infection with Klebsiella rhinoscleromatis. Females are far more frequently affected than males. Symptoms typically appear in the second and third decades of life. Diagnosis is confirmed based on the pathology result of the biopsy and also by microbiology exam, but it has a low-rate specificity. Treatment consists of prolonged antibiotic therapy and surgical intervention. Follow-up of the patient is necessary due to the high relapse rate of the disease.
We present a rare case of rhinoscleroma presented initially with cutaneous manifestations, which progressed rapidly causing invasion of the right frontal sinus by lysis of its anterior wall.
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