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Bazex Syndrome

BDCS


Presentation

  • We present evidence to justify costs of therapy for insurance purposes.[ncbi.nlm.nih.gov]
  • Further, the patient's presentation is unique as she was discovered to demonstrate laboratory findings consistent with coexistent porphyria cutanea tarda and relative zinc deficiency.[ncbi.nlm.nih.gov]
  • About 160 cases have been presented so far. We presented a patient with a three-month history of violaceous erythema, edema, erosions and scaling on the acral regions, elbows and knees and severe nail dystrophy.[ncbi.nlm.nih.gov]
  • We present the case of a patient with a newly diagnosed pyriform sinus tumour and associated acrokeratosis paraneoplastica. To the best of our knowledge, this is the first reported case in the local literature.[ncbi.nlm.nih.gov]
  • We present a case of Bazex syndrome and provide a review of the literature.[ncbi.nlm.nih.gov]
Cervical Lymphadenopathy
  • This article describes Bazex syndrome in a 56-year-old woman who presented with bilateral cervical lymphadenopathy and a mass at the base of her tongue.[ncbi.nlm.nih.gov]
  • Acrokeratosis paraneoplastica Bazex (APB) is a very rare disease in the group of obligate paraneoplastic dermatoses, associated mostly with squamous cell carcinoma of the upper aerodigestive tract and metastatic cervical lymphadenopathy.[ncbi.nlm.nih.gov]
  • Six weeks later, the patient developed bilateral cervical lymphadenopathy.[mdedge.com]
Lymphadenopathy
  • This article describes Bazex syndrome in a 56-year-old woman who presented with bilateral cervical lymphadenopathy and a mass at the base of her tongue.[ncbi.nlm.nih.gov]
  • Acrokeratosis paraneoplastica Bazex (APB) is a very rare disease in the group of obligate paraneoplastic dermatoses, associated mostly with squamous cell carcinoma of the upper aerodigestive tract and metastatic cervical lymphadenopathy.[ncbi.nlm.nih.gov]
  • Six weeks later, the patient developed bilateral cervical lymphadenopathy.[mdedge.com]
Severe Pain
  • She suffered severe pain in the fingers and toes and experienced some symptomatic relief with psoralen ultraviolet A therapy.[ncbi.nlm.nih.gov]
  • Since then, the ulcers quickly progressed causing severe pain and limited oral overall function, thus, the radiotherapy protocol had to be discontinued in the 11th day due to the severity of these lesions.[ncbi.nlm.nih.gov]
Pathologist
  • Cary Chisholm, MD Dermatopathology Fellow, Department of Dermatology, University of Texas Southwestern Medical Center Cary Chisholm, MD is a member of the following medical societies: College of American Pathologists, Texas Medical Association, and United[emedicine.medscape.com]
  • Cary Chisholm, MD Dermatopathology Fellow, Department of Dermatology, University of Texas Southwestern Medical Center Cary Chisholm, MD is a member of the following medical societies: College of American Pathologists , Texas Medical Association , and[emedicine.medscape.com]
Shivering
  • Case presentation A 60-year-old woman presented with acute upper abdominal pain, fever, nausea and shivering as well as elevated liver and pancreatic enzymes.[bmccancer.biomedcentral.com]
Respiratory Insufficiency
  • Insufficiency Syndrome Pub Type(s) Case Reports English Abstract Journal Article Language spa PubMed ID 17257564 TY - JOUR T1 - [Pulmonary adenocarcinoma and Bazex syndrome (paraneoplastic acrokeratosis)].[unboundmedicine.com]
Acute Cough
Hematochezia
  • Colonoscopy was performed because of hematochezia, and it revealed an early colon cancer. After the resection of the cancer, the skin lesions began to fall off dramatically.[ncbi.nlm.nih.gov]
Upper Abdominal Pain
  • Case presentation A 60-year-old woman presented with acute upper abdominal pain, fever, nausea and shivering as well as elevated liver and pancreatic enzymes.[bmccancer.biomedcentral.com]
Abdominal Pain
  • Case presentation A 60-year-old woman presented with acute upper abdominal pain, fever, nausea and shivering as well as elevated liver and pancreatic enzymes.[bmccancer.biomedcentral.com]
Anhidrosis
  • Anhidrosis (inability to sweat) of the face and head may also occur. In adulthood, around 20-30 years old, basal cell carcinomas begin to appear on the face. These are similar to the tumours of Gorlin syndrome.[dermnetnz.org]
  • Anhidrosis (inability to sweat) of the face and head may also occur. In adulthood, around 20-30 years old, basal cell carcinomas begin to appear on the face. These are similar to the tumours of Gorlin syndrome .[dermnetnz.org]
Skin Bullae
  • Less frequently, skin bullae and vesicles as well as mucosa alterations can occur concomitantly to the more typical cutaneous lesions [ 1 , 2 ].[ncbi.nlm.nih.gov]
Incontinence
  • […] always of epidermal and never of mesodermal origin, possible histopathological changes including the presence of necrotic foci, scattered vacuolar degeneration of basal cells, cytoplasmic eosinophilia and vacuolization of keratinocytes and pigmentary incontinence[bmccancer.biomedcentral.com]
Genital Lesions
  • In summary, as rarely reported before, the authors have presented a case where oral recalcitrant ulcers, skin and genital lesions led to the diagnosis of Bazex syndrome, most probably, as a consequence of a distant metastasis from a tongue SCC.[ncbi.nlm.nih.gov]

Workup

Mediastinal Mass
  • Since the family refused permission for necropsy, it was not possible to collect tissue samples from the mediastinal tumour and consequently to achieve the final diagnosis of the mediastinal mass that was highly suggestive of malignant tumour.[ncbi.nlm.nih.gov]

Treatment

  • Treatment modalities including topical and systemic corticosteroids, salicylic acid, topical vitamin D analogues, etretinate and PUVA therapy are often ineffective.[ncbi.nlm.nih.gov]
  • Our patient's clinical skin findings improved significantly following treatment and partial remission of her underlying malignancy.[ncbi.nlm.nih.gov]
  • […] language.RESULTSWe present a case of acrokeratosis paraneoplastica in a patient with incurable stage IV squamous cell carcinoma of the hypopharynx that significantly improved on acitretin.CONCLUSIONAlthough acrokeratosis paraneoplastica most often is cured by treatment[ncbi.nlm.nih.gov]
  • We report a typical case with a squamous cell carcinoma of the tongue and classical cutaneous symptoms disappearing with the treatment of the tumour.[ncbi.nlm.nih.gov]
  • Imaging analyses pulmonary adenocarcinoma with both local metastatic nodules and osseous metastasis.Symptomatic treatment with topical corticosteroids and oral retinoids showed no improvement.[ncbi.nlm.nih.gov]

Prognosis

  • An early diagnosis may improve the patient's prognosis substantially. 2017 European Academy of Dermatology and Venereology.[ncbi.nlm.nih.gov]
  • […] made on the basis of radiological (computerized) images, blood tests, and tissue biopsies The main treatment involves attempting to resolve the underlying cancer, and the use of topical corticosteroid creams to help control and treat skin lesions The prognosis[dovemed.com]
  • In most reported cases of acrokeratosis paraneoplastica, cervical lymph node metastases are present at the time of diagnosis, usually indicating a poor prognosis. [4] Morbidity and mortality in acrokeratosis paraneoplastica are related directly to the[emedicine.medscape.com]
  • Bazex syndrome). ( 29036561 ) Dabas G....Radotra B.D. 2018 4 Acrokeratosis Neoplastica (Bazex Syndrome) ( 29083711 ) Pulickal J.K....Kaliyadan F. 2017 5 Acrokeratosis paraneoplastica (Bazex syndrome) - a systematic review on risk factors, diagnosis, prognosis[malacards.org]

Etiology

  • Moreover, etiological therapy against underlying malignancy is essential for treating paraneoplastic Bazex syndrome.[ncbi.nlm.nih.gov]
  • Etiology The etiology is unknown. Proposed mechanisms include reaction to secreted epidermal growth factors, tumor specific antibodies that react to the skin basement membrane, or zinc deficiency.[dermatologyadvisor.com]
  • Acrokeratosis paraneoplastica (Bazex syndrome) presenting in a patient with metastatic breast carcinoma: possible etiologic role of zinc. J Cutan Med Surg. 2007 Mar-Apr. 11(2):78-83. [Medline].[emedicine.medscape.com]
  • Acrokeratosis paraneoplastica (Bazex syndrome) presenting in a patient with metastatic breast carcinoma: possible etiologic role of zinc. J Cutan Med Surg . 2007 Mar-Apr. 11(2):78-83. [Medline] .[emedicine.medscape.com]

Epidemiology

  • Proposed mechanisms include cross-reactivity between skin and tumor antigens, the action of tumor-produced growth factors, and even zinc deficiency. [1, 2] Epidemiology Frequency Approximately 140 cases of acrokeratosis paraneoplastica have been reported[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • […] dermatofibroma Member Rated 0 Patient case no. 338 Date added 01 January 1990 Patient details Age --Undetermined-- Localisation --Undetermined-- Primary Lesions Papule / hyperpigmented Pathophysiology tumors and proliferative diseases / connective tissue[dermquest.com]
  • Pathophysiology Pathophysiology consists of keratinocyte proliferation with resultant parakeratosis and acanthosis.[dermatologyadvisor.com]
  • Pathophysiology The pathophysiology of acrokeratosis paraneoplastica is not understood.[emedicine.medscape.com]
  • This mutation presents a palmoplantar keratoderma, generalized thin desquamation on the extensor surfaces of the finger joints, and constricting bands around the digits (pseudo-ainhum). 1, 3, 4 Abnormal structure of the stratum corneum is an important pathophysiological[scielo.br]

Prevention

  • Neoplastica and by the response of the affected individual to treatment Please find comprehensive information on Acrokeratosis Neoplastica regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention[dovemed.com]
  • Sun protection is vital to reduce the number of skin cancers developing but even complete protection will not prevent all basal cell carcinomas in patients with Bazex-Dupre-Christol syndrome. See smartphone apps to check your skin.[dermnetnz.org]
  • Sun protection is vital to reduce the number of skin cancers developing but even complete protection will not prevent all basal cell carcinomas in patients with Bazex-Dupre-Christol syndrome .[dermnetnz.org]
  • Sun protection is essential to reduce the number of skin cancers developing but even total protection will not prevent occurrence of basal cell carcinomas in these patients.[xpertdox.com]
  • And because BZX has not demonstrated that its proposed rule change is designed to prevent fraudulent and manipulative acts and practices, the Commission must disapprove the proposal. 1.[federalregister.gov]

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