Langerhans cell histiocytosis (LCH) is a rare disordershowing a wide spectrum of clinical manifestationsranging from single cutaneous lesions to multifocalsystemic disease [1,2]. The typical infiltration consistsof pathological monoclonal Langerhans cells togetherwith lymphocytes, eosinophilic granulocytes and non-dendritic histiocytes [3]. Langerhans cells are antigen-presenting cells with features of dendritic cells, stainingpositive for CD1a [4]. One of their original functions iscutaneous immunosurveillance. LCH is rare and theincidence in adults is 1–2 cases per million, with a meanage of 35 14 years, and is slightly more prevalent inmales [5,6]. Histological diagnosis is made if lightmicroscopic morphological characteristics of the dis-ease are found with positive staining for CD1a antigen[4,7]. In addition, Birbeck granules in the lesional cellthat are detected with electron microscopy are alsosuggestive of the disease [4,7]. Typical manifestationsof LCH in adults are central diabetes insipidus, bone,pulmonary, skin disease and dental involvement.We report a case of a patient with progressive renalinsufficiency due to multi-systemic manifestation ofLangerhans cell histiocytosis including involvement ofthe kidneys. Treatment of LCH with chemotherapyresulted in stabilization of renal function.CaseA 46-year-old male patient was admitted to ournephrology clinic in May 2006, with a 1-year historyof impaired renal function with moderate proteinuriaand glomerular erythrocyturia. In 1991, the patientwas successfully treated for fibrous alveolitis withprednisolone. Two years later, the patient developedcentral diabetes insipidus. In 1999, Histiocytosis X(Langerhans cell histiocytosis, LCH) was diagnosed ina skin biopsy obtained from an axillary erythema(histological findings: cellular infiltration of the epi-dermis with histiocytes, co-expression of S100 andCD1a; negativity for lymphocytic markers and KL-1;positive PG-M1; epitheliotropism; highly-positive toMiB1). In the follow-up, he also developed bilaterallyrelapsing therapy-resistant external otitis and swollencervical and submandibular lymph nodes. CT thoraxrevealed disseminated micronodular pulmonal opaci-ties. Skeletal scintigraphy showed a suspicious focus inthe proximal left tibia, and foci in the left mandible andleft mastoid could be localized. Thin-layer magneticresonance tomography of the sella turcica providedevidence for granuloma, with contrast enhancement inthe area of the hypophyseal stalk. An intravenousprednisolone therapy with 100mg was performed andthe cutaneous focus showed a rapid diminution.Therefore, methylprednisolone (60mg/day for 4weeks) and vinblastine (7mg/m
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