Presentation
We present a case in which both pulmonary adenocarcinoma and Bazex syndrome were present. [archbronconeumol.org]
We present evidence to justify costs of therapy for insurance purposes. [ncbi.nlm.nih.gov]
Entire Body System
- Short Stature
[…] bowel syndrome short QT syndrome + Short Stature, Onychodysplasia, Facial Dysmorphism, and Hypotrichosis Short Stature-Obesity Syndrome SHORT syndrome shoulder impingement syndrome Shprintzen Omphalocele Syndrome SHUKLA-VERNON SYNDROME sick building [rgd.mcw.edu]
Presence of ichthyotic lesions, cicatricial alopecia, cataracts, and skeletal abnormalities (short stature, asymmetric shortening of the limbs, epiphyseal stippling) help in differentiating it from Bazex-Dupré-Christol syndrome.[8] Table 1: Differential [ijdvl.com]
- Surgical Procedure
“Tumor spillage,” direct contamination of wounds with tumor cells during a laparoscopy or surgical procedure, was once a problem in nearly 20% of cases but is now uncommon; laparoscopic port site metastasis rates and laparotomy wound metastasis rates [ijmpo.org]
Cardiovascular
- Cyanosis
The Rombo syndrome: a familial disorder with vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis. Acta Derm Venereol. 1981;61:497-503. Inoue Y, Ono T, Kayashima K et al. [jpad.com.pk]
[…] are : - Gorlin syndrome, an autosomal dominant disease associating multiple basal cell carcinomas, jaw keratocysts, dyskeratotic palmar/plantar pits and skeletal malformations; - Rombo syndrome, a probably autosomal dominant disease that presents with cyanosis [atlasgeneticsoncology.org]
Skin
- Ulcer
Ulcerations and crusts affecting vermillion and mucosa of upper and lower lips. b Limited mouth opening and widespread ulcerations involving lips, buccal mucosa and tongue Scales on the dorsal surfaces of the metacarpophalangeal and proximal interphalangeal [ncbi.nlm.nih.gov]
Dermoscopy of the ulcerated plaque over right cheek showed blue-gray ovoid nests, blue-gray globules, multiple telangiectasias and erosions [Figure - 12]. [ijdvl.com]
A complete physical examination revealed the presence of blood in the stool, and sigmoidoscopy showed an ulcerative growth at the rectosigmoid junction. The histopathology showed adenocarcinoma Actions (login required) View Item [eprints.manipal.edu]
Sigmoidoscopy showed an ulcerative growth at the rectosigmoid junction. A biopsy of that lesion revealed well-differentiated adenocarcinoma. [escholarship.org]
- Papule
There was a single 0.5 cm × 0.5 cm hyperpigmented papule below her left eye, [Figure - 7] which had appeared 5 years back. [ijdvl.com]
Multiple discrete, white, superficial, firm papules were present over scalp, face, and ears [Figure 1]b. Few scattered papules were seen over thighs, legs, and hands [Figure 2]. [ijtrichology.com]
A few violaceous shiny papules with a lichenoid aspect were noticed on the dorsal part of the hands. The patient was cachectic and depressed. [emjreviews.com]
Basit bir papuler BCC lezyonu icin basvuran ve dikkatli bir inspeksiyon ve arastirma sonucu BDCS tanisi alan hastamizi sunuyoruz. [go.gale.com]
- Alopecia
Rare (currently about 20 families have been described worldwide) X-linked chromosomal dominant syndrome with generalized hypotrichosis since birth, diffuse alopecia mainly of the parietal and temporal region, atrophodermia vermiculata with emphasis on [altmeyers.org]
Nail dystrophy, palmoplantar keratoderma, and alopecia are common. In most patients, manifestations of Bazex syndrome precede the diagnosis of malignancy or the malignancy is diagnosed concurrently. [medicowesome.com]
Acrokeratosis paraneoplastica ( Bazex syndrome ) associated with primary cutaneous squamous cell carcinoma of the lower leg, vitiligo and alopecia areata [6]. [wikigenes.org]
548–550.Google Scholar Hara, M., Hunayama, M., Aiba, S., Suetake, T., Watanabe, M., Tanaka, M., Tagami, H. (1995) Acrokeratosis paraneoplastica (Bazex syndrome) associated with primary cutaneous squamous cell carcinoma of the lower leg, vitiligo and alopecia [cambridge.org]
[…] syndrome (SV) include: starfish-shaped palmoplantar hyperkeratosis, constricting bands on the digits of the hands or feet and starfish-shaped hyperkeratosic lesions, salmon-colored or articular cushion on the dorsum of the hands. 1, 3 Nail dystrophy, alopecia [scielo.br]
- Sweating
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]
Symptoms * Scaly skin rash * Reduced sweating * Reduced body hair * Sparse scalp hair * Light-colored hair Diagnosis azex-Dupre-Christol syndrome signs may often refer to those signs that are only noticable by a doctor: * Scaly skin rash * Reduced sweating [checkorphan.org]
He was also discovered to have an enlarged mass on the left side of his neck and weight loss of 4 kg without other B symptoms (i.e., fever, night sweats, and weight loss). [nejm.org]
- Sparse Hair
Bazex-Dupre-Christol syndrome: a possible diagnosis for basal cell carcinomas, coarse sparse hair, and milia. Am J Med Genet. 1993;45:786. [jpad.com.pk]
[…] dominant inheritance. 57 Rapelanoro R...Lacombe D 7747764 1994 9 Basal cell carcinomas, coarse sparse hair, and milia. 57 Oley CA...Chenevix-Trench G 1642265 1992 10 Follicular atrophoderma with multiple basal cell carcinomas (Bazex). 57 Gould DJ...Barker [malacards.org]
(REVIEW) PMID 16272260 Basal cell carcinomas, coarse sparse hair, and milia. Oley CA, Sharpe H, Chenevix-Trench G. Am J Med Genet. 1992 Jul 15;43(5):799-804. [atlasgeneticsoncology.org]
The scalp hair was coarse and sparse. The patient had camouflaged the sparseness of the eyebrows with a black pencil [Figure - 7]. [ijdvl.com]
Face, Head & Neck
- Long Thin Nose
The horizontal bars indicate patients who were seen by the authors Figure 2: Multiple well-defined hyperpigmented papules, ice pick scars over face along with long thin nose with prominent columella in the index case. [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]
The cutaneous condition is refractory to topical treatment, which may be attempted with coal tar, salicylic acid, vitamin D analogues, corticoids and UVB radiation. [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]
Prognosis Prognosis is related to the underlying neoplasm. [orpha.net]
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 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
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]
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]
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]