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Bazex Syndrome
Bazex-Dupré-Christol syndrome

Images

WIKIDATA, Public Domain
WIKIDATA, Public Domain

Presentation

We present a case in which both pulmonary adenocarcinoma and Bazex syndrome were present. [unboundmedicine.com]

We present evidence to justify costs of therapy for insurance purposes. [ncbi.nlm.nih.gov]

Multiple fungating hard, dried and blackened skin lesions were present over both hands and feet along with clubbing in all fingernails lesions were not present over face and ear. [cureus.com]

Entire Body System

  • Hodgkin Lymphoma

    Other rare associations include adenocarcinoma of the stomach, esophagus, or uterus, anaplastic small-cell carcinoma of the lung, Hodgkin's lymphoma [3], transitional-cell carcinoma of the bladder [4], adenocarcinoma of the colon [5], and squamous cell [escholarship.org]

    It is a sign of diseases such as Hodgkin’s lymphoma, leukemias and solid tumors in the bladder, breasts or lungs. Treatment involves treating the underlying disease that is causing the skin condition. [news-medical.net]

    […] manifestation of malignancy - Paraneoplastic pemphigus Paraneoplastic pemphigus: apperance - Painful ulcers/bullae of the mouth, lips, esophagus, skin Paraneoplastic pemphigus: associations - Autoimmune disorder - lymphoreticular malignancy (least common) - Non-Hodgkins [studyblue.com]

    Clubbing is seen in bronchogenic carcinoma and mesotheliomas. [57] Scleromyxedema is seen in multiple myeloma, Waldenstrom's macroglobulinemia, Hodgkin's or non-Hodgkins lymphoma, or leukemias. [ijmpo.org]

Respiratoric

  • Pneumonia

    Although exacerbation of the eruptions or thymomas was not observed, the patient developed bacterial pneumonia. Despite the administration of broad-spectrum antibiotic therapy, the patient’s physical state deteriorated and he died. [journal.jrs.or.jp]

    Infection: URI-strep throat and pneumonia 3. Drugs: G-CSF 4. [studyblue.com]

    The medical group was warned about the possibility of Bazex syndrome and before they had the chance to perform a tumour screening in the patient, he was hospitalized in an emergency character with crisis of persistent and acute cough suggestive of pneumonia [ncbi.nlm.nih.gov]

Skin

  • Skin Lesion

    Occasionally, the reappearance of skin lesions has signaled a recurrence of the tumor. [ncbi.nlm.nih.gov]

    This diagnosis was confirmed when the skin lesions resolved after three weeks of radiation therapy to the mass.1 There was no recurrence of skin lesions at six-month follow-up. [cmaj.ca]

    […] the diagnosis of the neoplasm in only 16 (15%) of 109; in the remainder, the onset of the skin lesions and the diagnosis of the tumor occurred simultaneously. [pubmed.ncbi.nlm.nih.gov]

  • Eruptions

    Bazex syndrome, a rare paraneoplastic syndrome characterized by psoriasiform eruptions, palmoplantar keratosis, and symmetric onychodystrophy, is most prevalent with squamous cell carcinomas of the upper aerodigestive tract.Here, we reported an uncommon [ncbi.nlm.nih.gov]

    In the last stage, the eruption may extend to the limbs, trunk, and scalp.1 Histopathological findings are nonspecific. [emjreviews.com]

    Katsura Hospital ABSTRACT A 62-year-old male presented with palm and footpad eruptions in February 20XX. [journal.jrs.or.jp]

  • Hyperkeratosis

    The patient's skin disease was more remarkable for its hyperpigmentation than its hyperkeratosis. [ncbi.nlm.nih.gov]

    N Immuno-suppression Y hyperkeratosis palmaris et plantaris Member Rated 0 Patient case no. 338 Date added 10 February 2003 Patient details Age Elderly patient Localisation Lower limbs / feet / soles Description Primary Lesions Hyperkeratosis Squames [dermquest.com]

    The biopsy from the ear revealed hyperkeratosis and interface dermatitis with mixed perivascular inflammatory infiltrate [Figure - 5]. [ijdvl.com]

    […] initially presenting with palmoplantar hyperkeratosis. [bmccancer.biomedcentral.com]

    Examination of the nails showed thickening and brownish discoloration of nail plates with subungual hyperkeratosis involving a few nails of the hands and feet. [escholarship.org]

  • Keratosis

    Bazex syndrome, a rare paraneoplastic syndrome characterized by psoriasiform eruptions, palmoplantar keratosis, and symmetric onychodystrophy, is most prevalent with squamous cell carcinomas of the upper aerodigestive tract.Here, we reported an uncommon [ncbi.nlm.nih.gov]

    Very rarely signs of atopic diathesis, keratosis pilaris, ichthyosis, arachnodactyly, osteochondritis, hearing loss and learning problems are found. [altmeyers.org]

    10.1097/MD.0000000000002415, PMID: 26765420 Issn Print: Model.IssnPrint Publication Date: 2016/01/01 Checking for direct PDF access through Ovid Abstract Bazex syndrome, a rare paraneoplastic syndrome characterized by psoriasiform eruptions, palmoplantar keratosis [doi.org]

    Publisher Full Text Publisher Full Text Authors +Show Affiliations, Unidad de Neumología, Hospital Virgen de la Luz, Cuenca, España. [email protected], , Source MeSH Acrodermatitis Adenocarcinoma Aged Fatal Outcome Foot Dermatoses Hand Dermatoses Humans Keratosis [unboundmedicine.com]

    keratosis and melanocytic nevi. [ijdvl.com]

  • Erythema

    Abstract Bazex syndrome, or paraneoplastic acrokeratosis, is a rare psoriasis-like paraneoplastic skin disease, characterized by erythema and scaling, which is accompanied by hyperkeratotic lesions. [unboundmedicine.com]

    A 65-year-old white man presented with all the clinical features of acrokeratosis paraneoplastica of Bazex, characterized by violaceous erythema and scaling of the nose, aural helices, fingers, and toes, with keratoderma and severe nail dystrophy. [ncbi.nlm.nih.gov]

    Histological and immunohistochemical findings during the first visit. a, b Skin biopsy of hyperkeratotic erythema on the left palm. [karger.com]

    Abstract A 63-year-old man had acrokeratosis paraneoplastica (Bazex' syndrome) characterized by violaceous erythema and scaling of the fingers, toes, nose, and aural helices. Severe nail dystrophy was present. [safeaccess.unboundmedicine.com]

Psychiatrical

  • Suggestibility

    Want to suggest a symptom? Please send suggestions to RareGuru! [rareguru.com]

    Previous reports suggested an X-linked mode of inheritance. We therefore performed linkage analysis with microsatellite markers of the X chromosome in three families. [ncbi.nlm.nih.gov]

    None of them has any skeletal feature suggestive of Gorlin's syndrome. The clinical features, skin histology, and scanning electron microscopic (SEM) examination of the hair are described and illustrated. [unboundmedicine.com]

    Laboratory Findings Any clinical sign that could suggest Bazex syndrome should automatically guide a cutaneous biopsy. [wikidoc.org]

Workup

Other Pathologies

  • Lymphocytic Infiltrate

    Histological findings are not specific and include psoriasiform epidermal hyperplasia, hyperkeratosis with parakeratosis, and perivascular lymphocytic infiltrates in the dermis. [orpha.net]

    Hematoxylin and eosin staining revealed parakeratosis, acanthosis, elongated rete ridges, and perivascular lymphocyte infiltration of the upper dermis. The granular layer did not disappear and exocytosis of lymphocytes was found. [karger.com]

    Biopsy of skin changes revealed hyperkeratosis with parakeratosis and marked acanthosis and papillomatosis, sparse lymphocytic infiltrate and papillary edema [Figure 5]. [ijdvl.com]

Treatment

They should not receive treatment with irradiation as this is liable to provoke the development of more tumours. Some patients with Bazex-Dupré-Christol may require long-term treatment with oral retinoids such as isotretinoin or acitretin. [dermnetnz.org]

Systemic treatment also presents inconsistent results in studies with etretinate, corticoids and antibiotics.77. Abrou AE, Hope J, Jackson-Richards D, Lim HW, Ozog DM. Acrodermatitis paraneoplastica responding to topical PUVA treatment. [scielo.br]

Our patient's clinical skin findings improved significantly following treatment and partial remission of her underlying malignancy. [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]

Prognosis Prognosis is related to the underlying neoplasm. The documents contained in this web site are presented for information purposes only. [orpha.net]

[…] 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]

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]

Etiology The pathogenesis of Bazex syndrome remains unknown. It may be caused by the production of epidermal growth factor by tumor cells or by cross-reactivity between epidermal and tumor antigens. [orpha.net]

Etiology The gene has been mapped to the long arm of the X chromosome, within the Xq24-q27.1 region. UBE2A (Xq24), encoding a protein involved in repair of UV-damaged DNA, has been proposed as a candidate gene. [rarediseases.info.nih.gov]

Epidemiology

Summary Epidemiology To date, approximately 145 cases have been reported in the literature. The syndrome most commonly affects males over 40 years of age (mean age of onset: 61 years), and only 12 female patients have been reported so far. [orpha.net]

Epidemiology So far, 143 cases have been reported, mostly from France and Belgium. Clinical description The disease manifests during the neonatal period or during infancy. [rarediseases.info.nih.gov]

The BJD has distinct sections for clinical trials, guidelines, translational research, epidemiology and qualitative and outcomes research. [onlinelibrary.wiley.com]

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]

Bazex syndrome is frequently associated with squamous cell carcinomas of the upper aerodigestive tract.1,2 Although its pathophysiology is unknown, an association with bladder, breast and gynecological cancers has also been reported.1,2 This paraneoplastic [cmaj.ca]

Pathophysiology The pathophysiology of acrokeratosis paraneoplastica is not understood. [emedicine.medscape.com]

Prevention

[…] sunscreens is paramount, and goes a long way in prevention of malignancy. [ijdvl.com]

We report that BDCS is a novel ciliopathy and the first case of a skin cancer ciliopathy, where ARP-T1 plays a critical role to prevent pathogenesis. [biorxiv.org]

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]

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]

[…] minus' sign Virchow -triad Use Parentheses water AND (cup OR glass) Add an asterisk (*) at end of a word to include word stems Neuro* will search for Neurology, Neuroscientist, Neurological, and so on Use quotes to search for an exact phrase "primary prevention [read.qxmd.com]

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