Bazex-Dupré-Christol syndrome: First report in an Indian family

Chauhan, Payal; Meena, Durga Shankar; Dhanta, Aditi; Kansal, Naveen Kumar; Durgapal, Prashant

doi:10.4103/ijdvl.ijdvl_599_17

Cited by 1 publication

(2 citation statements)

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“…Thus it may not be possible to diagnose these syndromes before the patient actually presents with CAT. Familial cases, such as Birt-Hogg-Dubé syndrome, Cowden syndrome, Bazex-Dupre-Christol syndrome, Muir-Torre Syndrome, multiple familial trichoepithelioma, can be given as examples to such lesions (Table 2) [19,21,24,[26][27][28][29][30][31][32][33][34][35][36][37]. Although very [8,10].…”

Section: Discussionmentioning

confidence: 99%

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Areclinicianssuccessful in diagnosingcutaneousadnexaltumors? aretrospective, clinicopathologicalstudy

Kayıran¹,

Karadağ²,

Küçük³

et al. 2020

Turk J Med Sci

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Background/aim: Cutaneous adnexal tumors (CAT) are rare tumors originating from the adnexal epithelial parts of the skin. Due to its clinical and histopathological characteristics comparable with other diseases, clinicians and pathologists experience difficulties in its diagnosis.We aimed to reveal the clinical and histopathological characteristics of the retrospectively screened cases and to compare the prediagnoses and histopathological diagnoses of clinicians. Materials and methods:The data of the last 5 years were scanned and patients with histopathological diagnosis of CAT were included in the study.Results: A total of 65 patients, including 39 female and 26 male patients aged between 8 and 88, were included in the study. The female to male ratio was 1.5, and the mean age of the patients was 46.15 ± 21.8 years. The benign tumor rate was 95.4%, whereas the malignant tumor rate was 4.6%. 38.5% of the tumors were presenting sebaceous, 35.4% of them were presenting follicular, and 18.5% of them were presenting eccrine differentiation. It was most commonly seen in the head-neck region with a rate of 66.1%. When clinical and histopathological prediagnoses were compared, prediagnoses and histopathological diagnoses were compatible in 45% of the cases. Most frequently, it was the basal cell carcinoma, epidermal cyst, and sebaceous hyperplasia identified in preliminary diagnoses. Conclusion:Cutaneous adnexal tumors are very important, as they can accompany different syndromes and may be malignant. Due to difficulties in its clinical diagnosis, histopathological examination must be performed from suspicious lesions for definitive diagnosis.

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Section: Discussionmentioning

confidence: 99%

“…Syndromes associated with cutaneous adnexal tumors[19,21,24,[26][27][28][29][30][31][32][33][34][35][36][37]. (AD: Autosomal dominant; AR: Autosomal recessive; XR: X-linked recessive) CAT also have malignant forms.…”

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confidence: 99%