Xanthoma disseminatum (XD) is one of cutaneous nonhistiocytosis X, and characterized by multiple, widely distributed red, brown, yellowish papules and nodules that tend to coalesce. We present a case of XD with characteristic clinical findings and treated with the combination therapy of COz laser vaporization and high dose steroid.
Histiocytosis, Non-Langerhans-Cell* ; Laser Therapy ; Lasers, Gas* ; Volatilization* ; Xanthomatosis*

Histiocytosis, Langerhans-Cell ; diagnosis ; therapy ; Humans ; Practice Guidelines as Topic

Adult ; Cladribine/therapeutic use* ; Histiocytosis, Langerhans-Cell/drug therapy* ; Humans

Langerhans cell histiocytosis (LCH) is a group of unexplainable abnormal proliferation and aggregation of Langerhans cell. LCH can be classified into four clinical variants: Letterer-Siwe disease, Hand-Schüller-Christian disease, eosinophilic granuloma, and congenital self-healing LCH. LCH is most prevalent in children. Lesions can be localized in a single system or multiple organs, and clinical manifestations vary depending on the affected organs. The skin and mucocutaneous tissues are the starting point of the affected tissue. This study presents a LCH case characterized by transient self-healing. This case can further provide references for the clinical diagnosis and treatment of LCH.
Child ; Humans ; Histiocytosis, Langerhans-Cell/therapy* ; Diagnosis, Differential

Child ; Endoscopy ; Histiocytosis, Langerhans-Cell/therapy* ; Humans ; Mandible ; Tongue

By using the method of clonal analysis the evidence to prove that Hemophagocytic syndrome (HPS) is reactive or malignant was investigated to probe into the pathogenesis of HPS and its relations with clinical prognosis. The macrophages abnormally proliferated in bone marrow were isolated. Electrophoresis analysis was made after DNA extraction, enzyme restriction of human ardrogen receptor (HUMARA) genetic locus, and PCR amplification. In the 9 specimens, clonal proliferation was found in 2 cases and nonclonal proliferation in 7. Among the 7 cases of nonclonal proliferation, 3 were voluntarily discharged without clinical outcome, 2 cases fully recovered after 2-3 week treatment of large dose gamma globulin intravenous drip and hormone therapy, 1 case died at the 43th day after the hormone and anti-infection therapy, and one case was found to have granular leukoblast in peripheral blood after 3 weeks and diagnosed as having M2a after bone puncture. For the two patients with clonal proliferation, one obtained remission after chemotherapy and the other was died after 32 days without chemotherapy. It was concluded that there do exist clonal or malignant proliferation in HPS, so not every case is reactive.
Clone Cells ; Histiocytosis, Non-Langerhans-Cell/*blood ; Histiocytosis, Non-Langerhans-Cell/therapy ; Macrophages/*pathology ; gamma-Globulins/therapeutic use

Langerhans cell histiocytosis is known as one of the diseases related to excessive proliferation of normal monocytes and has the variety of clinical courses and treatment. Especially, in cases with the spine, it shows a feature of single or multiple osteolysis. According to the location, disease progression and concomitant symptom, variety of treatments (observation, radiotherapy, chemotherapy, surgery, etc.) have been attempted, however, appropriate treatment has not been established yet. The authors introduce the case of single system Langerhans cell histiocytosis which involves cervical and lumbar vertebrae simultaneously with bone marrow destruction and pathologic fracture.
Bone Marrow ; Disease Progression ; Drug Therapy ; Fractures, Spontaneous* ; Histiocytosis* ; Histiocytosis, Langerhans-Cell ; Lumbar Vertebrae ; Monocytes ; Osteolysis ; Radiotherapy ; Spine

Histiocytosis is a relatively rare disorder of the reticuloendothelial system involving the proliferation of histicoytes, granulation tissue, and inflammatory cells in many different organ systems1). Thus, the three manifestations of the same basic pathologic process:Eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease have been classified as localized, chronic disseminated and acute disseminated histiocytosis-X. They were therefore included under the term histiocytosis-X and this concept has been generally accepted. The authors have experienced one case of histiocytosis-X, a rare disease. A 11 month-old femal patient presented with gradually enlarged palpable mass on the occipital area. The occipital skull was defected in a punched out fashion. The mass was completely removed. The pathologic findings revealed Histiocytosis-X and the patient was given chemotherapy.
Drug Therapy ; Eosinophilic Granuloma ; Granulation Tissue ; Granuloma ; Histiocytosis ; Histiocytosis, Langerhans-Cell* ; Humans ; Infant ; Mononuclear Phagocyte System ; Rare Diseases ; Scalp* ; Skull

Child, Preschool ; Female ; Histiocytosis, Non-Langerhans-Cell ; diagnosis ; etiology ; therapy ; Humans

Hand-Schuller-Christian disease, eosinophilic granuloma and Letterer-Siwe disease are classified as histiocytosis-x, on the basis of the underlying proliferation of histiocytes in all three conditions and of their indefinite etiology. The authors experienced a case of Hand-Schuller-Christian disease with exophthalmos. A 17 months old boy was admitted to our Dept. of Ophthalmology because of left exophthalmos since 2 months ago. A biopsy demonstrated that the lesion was Hand-Schuller-Christian disease. The patient was treated with chemotherapy and low dose radiation therapy. We report our experience with this patient along with the review of other papers in this report.
Biopsy ; Drug Therapy ; Eosinophilic Granuloma ; Exophthalmos* ; Histiocytes ; Histiocytosis, Langerhans-Cell ; Humans ; Infant ; Male ; Ophthalmology