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Xanthoma Disseminatum

Montgomery syndrome


Presentation

  • The gross and microscopic pathology are presented with immunohistochemical, ultrastructural and biochemical details.[ncbi.nlm.nih.gov]
Weight Gain
  • A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas.[ncbi.nlm.nih.gov]
  • Results: A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas.[journals.aace.com]
  • Case: A 16-year-old female patient admitted to our hospital with the complaints of amenorrhoea, weight gain, polidipsia, poliuria, yellow-brownish papular lesions on the cervical, periorbital, axillary and genital regions.[endocrine-abstracts.org]
Galactorrhea
  • A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas.[ncbi.nlm.nih.gov]
  • Results: A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas.[journals.aace.com]
Inguinal Lesions
  • The underarm lesions and inguinal lesions had improved significantly. He had no new lesions develop during follow-up. After 6 months of follow-up, the lesions continued to flatten, with involution of the XD lesions and no evidence of new lesions.[jamanetwork.com]
Vietnamese
  • Vietnamese - English Vietnamese - Vietnamese Vietnamese - French French - Vietnamese Computing English - English Chinese - Vietnamese Search in: Word Vietnamese keyboard: Off Virtual keyboard: Show English - English (Wordnet) dictionary xanthoma disseminatum[vdict.com]
Limited Mobility
  • Laryngoscopy was done and revealed small, yellow-colored nodules on laryngeal mucosa and on both vocal cords which showed limited mobility.[jpgmonline.com]
Cough
  • A 10-year-old boy was admitted to the University Hospital complaining of exhaustion, night sweats, coughing and dyspnea. Physical examination, laboratory findings and histopathological examination led to the diagnosis of Xanthoma disseminatum.[ncbi.nlm.nih.gov]
  • せき/ 【咳喘息】*cough variant asthma (略 CVA) (顎) 【顎関節機能障害】【顎関節機能異常】*temporomandibular dysfunction 【顎口蓋裂】*gnathopalatoschisis 【顎口虫症】*gnathostomiasis (含) 【含気性腹膜炎】*pneumoperitonitis 【含歯性嚢胞】 *dentigerous cyst (癌) 【癌】【癌腫】*carcinoma *cancer 【癌腫症】【癌多発症】*carcinomatosis[medo.jp]
Sore Throat
  • He had a sore throat and severe hoarseness of his voice. There was also infiltration of the pituitary stalk, adenoids, and soft palate. The patient's lipid profile results showed a high triglyceride level.[jamanetwork.com]
Clubbed Finger
  • 苔)* 【苔癬】*lichen 【苔癬様異形成】*lichenoid dysplasia 【苔癬様角化症】*lichenoid keratosis 【苔癬様湿疹】*lichenoid eczema 【苔癬様皮膚症】【苔癬様皮膚病】*lichenoid dermatosis (退)* 【退行形成】【退形成】*retroplasia *anaplasia 【退縮眼振】*retractory nystagmus *nystagmus of retraction (太) 【太鼓バチ状指】【太鼓バチ指】*clubbed[medo.jp]
Night Sweats
  • A 10-year-old boy was admitted to the University Hospital complaining of exhaustion, night sweats, coughing and dyspnea. Physical examination, laboratory findings and histopathological examination led to the diagnosis of Xanthoma disseminatum.[ncbi.nlm.nih.gov]
  • Adverse effects of 2-CdA were limited to joint pain and night sweats. After 5 treatment cycles, the patient had no new lesions and had achieved additional improvement in established lesions, which were more flat and less erythematous.[jamanetwork.com]
Facial Skin Lesion
  • Treatment was initiated with cabergoline, nasally administered desmopressin, radio-frequency ablation of facial skin lesions, and surgical excision of other accessible lesions.[ncbi.nlm.nih.gov]
Shoulder Pain
  • pain *omalgia *omodynia 【剣状突起炎】*xiphoiditis 【剣状突起結合体】*xiphopagus 【顕性遠視】*manifest hyperopia (略 Hm) (コ) 【コイロサイトーシス】*koilocytosis ( 空胞細胞症) 【コクシジウム症】*coccidiosis 【コラーゲン炎】*collagenitis 【コルサコフ症候群】*Korsakoff syndrome 【コレラ】*cholera 【コレステロール塞栓症】*cholesterol embolism[medo.jp]
Blurred Vision
  • Skin lesions were accompanied by blurred vision and hoarseness of voice. Examination revealed xanthomatous infiltration of cornea, oral, pharyngeal, and laryngeal mucosae.[ncbi.nlm.nih.gov]
  • Patients present with characteristic mucocutaneous lesions, diabetes insipidus, dysphagia, dyspnea, hoarseness of voice, and blurred vision.[orpha.net]
Diplopia
  • The patient suffered from diplopia and had a severe sensorimotor polyneuropathy causing progressive paresis of the limbs.[ncbi.nlm.nih.gov]
  • […] subluxation 【不全対麻痺】*paraparesis 【不全麻痺】*paresis 【不耐性】*intolerance 【不定疼痛】*atypical pain 【不眠症】*insomnia *agrypnia (浮) 【浮腫】*edema ( 水腫) (婦) 【婦人病】*gynecopathy *gynopathy *female complaint (付) 【付属器炎】*adnexitis 子宮付属器炎 (風) 【風疹】*rubella *German measles (複) 【複視】*diplopia[medo.jp]
Corneal Infiltrate
  • Ocular examination revealed conjunctival and left corneal infiltration by yellow minute papules with normal visual field. Abdomino-pelvic ultrasonography, chest X-ray, upper and lower gastrointestinal endoscopy revealed no abnormality.[jpgmonline.com]
Amenorrhea
  • A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas.[ncbi.nlm.nih.gov]
  • Additional findings were polyuria, polydipsia, and amenorrhea. Skin biopsy and electron microscopy results confirmed the diagnosis of XD. The patient was treated with fenofibrate, simvastatin, desmopressin, and sex-hormone replacement therapy.[karger.figshare.com]
  • Results: A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas.[journals.aace.com]
Secondary Amenorrhea
  • A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas.[ncbi.nlm.nih.gov]
  • Results: A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas.[journals.aace.com]
Paresis
  • The patient suffered from diplopia and had a severe sensorimotor polyneuropathy causing progressive paresis of the limbs.[ncbi.nlm.nih.gov]
  • RA) 【不可避流産】*inevitable abortion 【不完全ブロック】*partial block 【不整脈】*cardiac arrhythmia/*arrhythmia 【不全感染】【不発感染】*abortive infection ( 頓挫感染) 【不全症】*insufficiency 【不全整復】*malreduction 【不全脱臼】*incomplete dislocation *[ 亜脱臼] subluxation 【不全対麻痺】*paraparesis 【不全麻痺】*paresis[medo.jp]

Workup

  • Progress logically from the histologic pattern, through the appropriate workup, around the pitfalls, to the best diagnosis.[books.google.it]
  • A thorough workup is essential to identify the underlying condition in order to initiate early treatment and to prevent later complications.[austinpublishinggroup.com]
  • The macroscopic lesions arise from phagocytosis in the dermis of plasma lipoproteins that leak from capillaries. 1 Laboratory workup is mandatory to exclude diabetes, nephrotic syndrome, or hypothyroidism.[hkmj.org]
Atelectasis
  • Chest x-rays showed hyperaeration and segmental atelectasis. High-resolution CT of the thorax revealed diffuse thickening of the whole tracheal and bronchial wall, and bronchiectasis in the lower lobes.[ncbi.nlm.nih.gov]
  • Chest x‐rays showed hyperaeration and segmental atelectasis. High‐resolution CT of the thorax revealed diffuse thickening of the whole tracheal and bronchial wall, and bronchiectasis in the lower lobes.[doi.org]
  • 【象牙質腫】*dentinoma (増) 【増殖型網膜症】*proliferating retinopathy 【増殖性糸球体腎炎】*proliferative glomerulonephritis 【増殖性心内膜炎】*vegetative endocarditis (続) 【続発性胆汁性肝硬変】*secondary biliary cirrhosis (略 PBC) (粟) 【粟粒結核】*miliary tuberculosis 【粟粒疹】*miliaria 【粟粒性無気肺】*miliary atelectasis[medo.jp]
Hyperprolactinemia
  • Laboratory tests showed hyperprolactinemia, normal electrolytes, and a normal lipid profile.[ncbi.nlm.nih.gov]

Treatment

  • We studied 15 articles on treatment of xanthoma disseminatum. Local treatment with cryotherapy, radiotherapy, surgery, and carbon dioxide lasers have been attempted with various results.[ncbi.nlm.nih.gov]

Prognosis

  • CNS involvement carries a poor prognosis in this otherwise benign condition.[ncbi.nlm.nih.gov]
  • What is the Prognosis of Xanthoma Disseminatum? (Outcomes/Resolutions) The prognosis of Xanthoma Disseminatum (XD) depends on the type of condition and the symptoms that manifest.[dovemed.com]
  • Prognosis often resistant to treatment Localization mucocutaneous ocular laryngeal pituitary central nervous system liver ( 16354260 ) See also systemic histiocytoses non-Langerhans cell histiocytoses dermal dendrocytes dendrocytic tumors (dendrocytic[humpath.com]
  • Even with this escalation of treatment, progressive cases of XD typically have a poor prognosis.[dermatologyadvisor.com]

Etiology

  • Xanthoma disseminatum is a rare, benign, non-Langerhans' cell histiocytic disorder of unknown etiology.[ncbi.nlm.nih.gov]
  • Xanthoma Disseminatum: Case Report Abstract Xanthoma disseminatum is a rare, benign, non-Langerhans cell histiocytic disorder of unknown etiology.[adc.mef.hr]
  • Tumoral pathology xanthoma disseminatum Xanthoma disseminatum is a rare, usually self- healing dermatologic disease of unknown etiology . Involvement of other organs and tissues including bone marrow, bone, and brain may be seen rarely in children.[humpath.com]

Epidemiology

  • XD could be differentiated from BCH on the basis of epidemiological, clinical and histopathological findings. One hypothesis could link such circumscribed localization to some regional (dis-)immune factors (10).[medicaljournals.se]
  • Definition / general Localized mass forming collection of lipid bearing histiocytes Terminology Also called xanthomatous polyp, xanthogranulomatous inflammation, xanthelasma Epidemiology Very rare in colon - stomach is most common gastrointestinal site[pathologyoutlines.com]
  • The pathogenesis of NLCH is not clear 1 ; however, there is some information about the epidemiology and clinical findings. First, XD usually starts before the age of 25 years in about 60% of patients 1 . Second, it is more common in males 1 .[synapse.koreamed.org]
Sex distribution
Age distribution

Pathophysiology

  • Etiology Pathophysiology The etiology and pathogenesis of XD are unknown. Some authors have speculated that the association with paraproteinemia suggests an immunologic basis for this disease.[dermatologyadvisor.com]
  • Department of Molecular Medicine, IRCCS San Matteo Foundation, University of Pavia, Pavia, 2 Unit of Dermatology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Pace 9, Milan, 3 Department of Oncology and Hematology, and 4 Department of Pathophysiology[medicaljournals.se]
  • Indeed, the different subtypes of NLH share largely common immune, histopathological and probably pathophysiological features, and their differential diagnosis is frequently based on some specific organ involvements.[karger.com]

Prevention

  • Xanthomas associated with hyperlipidemia require treatment of the lipid disorder to reduce the xanthomas and prevent possible risks (such as atherosclerosis) (2).[empowher.com]
  • Although many conservative treatments are unsatisfactory, active intervention may still be warranted for the noninvoluting or disfiguring type for cosmetic reasons or to prevent permanent functional impairments. [1] :: Case Report A 10-year-old male child[jpgmonline.com]
  • A thorough workup is essential to identify the underlying condition in order to initiate early treatment and to prevent later complications.[austinpublishinggroup.com]
  • Treatment of eruptive xanthoma involves dietary restriction, exercise, and drug therapy to control the hyperlipidemia and the diabetes. 2 Early recognition and proper control of hypertriglyceridemia can prevent sequelae such as acute pancreatitis. 3[mdedge.com]

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