Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign proliferative disorder of histiocytes with an unknown etiology. It is a rare disease characterized by overproduction and accumulation of histiocytes within lymph node sinuses and many other extranodal sites, including skin, oral and nasal cavities, respiratory tract, eyelid, and periorbital area. In this case, a 44-year-old woman with diagnosis of Rosai-Dorfman disease, with xanthelasma-like cutaneous lesions on facial area, extending to her neck and acneiform papules on her back, cervical lymph node involvement, and concomitant presence of diabetes insipidus was presented. Histopathological examination of the lesions demonstrated diffuse lymphocyte, plasmocyte, eosinophil, and sparse neutrophil infiltration, together with histiocytes showing phagocytosed inflammatory cells (emperipolesis). Histiocytes demonstrated immunoreactivity with the antibodies for CD68 and S100, whereas they were negative for CD1a and Langerin. Laboratory tests were normal, except mild immunoglobulin G hypergammaglobulinemia. Systemic methylprednisolone therapy was effective for cutaneous lesions.
Background/aim: Alopecia areata is an inflammatory disease with a genetic and autoimmune basis. We aimed to study the efficacy, and safety of an immunomodulatory therapeutic agent, diphenylcyclopropenone, while manifesting the association with histopathological features, prognostic factors and side effects. Materials and Methods: In this retrospective study, 98 patients (60 men, 38 women) with alopecia who were referred to the "Hair Disease Polyclinic" at Department of Dermatology between 2011 and 2015 were included. Together with medical histories and dermatological examinations, all patients were taken a skin biopsy for histopathological examination prior to the therapy. Therapeutic success was evaluated on the basis of hair re-growth percentage. Results: Regarding the overall treatment success, 33 (34%) patients had complete response, 16 (16%) patients had partial response (between 50-99%), 27 (28%) patients had minimal response (between 1-49%), and 22 (22%) patients were non-responders. Both genders were equally represented in the outcome. Conclusions: There was a significant relation between the severity of alopecia and treatment outcome. (p= 0.038) Patients with alopecia areata had significantly better response when compared to alopecia totalis and universalis. There was no statistically significant relation with other parameters such as disease duration, age, gender, atopy history, age of onset, and histopathological features.
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