Follicular atrophoderma and basal cell carcinomas: the Bazex syndrome

Viksnins, P.

doi:10.1001/archderm.113.7.948

Cited by 24 publications

(20 citation statements)

References 1 publication

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“…In addition, they have been reported as part of Rambo's syndrome, which is a familial disorder with vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis (13). The Bazex syndrome is another familial disorder featuring follicular atrophoderma, basal cell carcinomas, and trichoepitheliomas (14,15).…”

Section: Discussionmentioning

confidence: 99%

Benign cutaneous adnexal tumors in childhood and young adults, excluding pilomatrixoma: review of 28 cases and literature

1991

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Benign adnexal tumors of the skin, excluding pilomatrixomas, were identified in 28 patients between the ages of 4 and 20 years with a mean age of 15; 23 women and 5 men. Most lesions were described as papules and they occurred on the face, scalp and neck in 75% of cases. Trichoepithelioma and syringoma accounted for 56% of all neoplasms. Only one patient gave a history of familial trichoepitheliomas. The remaining tumors were examples of syringocystadenoma papilliferum, eccrine acrospiroma, and eccrine and apocrine hidrocystomas. Excisional biopsy constituted the treatment in all cases; only one neoplasm, an eccrine acrospiroma, recurred locally.

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Section: Discussionmentioning

confidence: 99%

Benign cutaneous adnexal tumors in childhood and young adults, excluding pilomatrixoma: review of 28 cases and literature

1991

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“…16 Basal cell carcinomas may be seen in association with or occasionally arise within pre-existing lesions of TPM. 12,18,19 Cylindromas rarely undergo malignant degeneration. 2,20 Thus, tumours that differ from the appearance of those of TPM should be considered for histological evaluation.…”

Section: Discussionmentioning

confidence: 99%

“…Similarly, multiple trichoepitheliomas may be seen in two other rare syndromes: the Rombo syndrome (vermicular atrophoderma, milia, hypotrichosis, basal cell carcinomas, trichoepitheliomas and peripheral vasodilatation with cyanosis) and Basex syndrome (follicular atrophoderma, hypotrichosis, occasional trichoepitheliomas, basal cell carcinomas, and localized or generalized hypohidrosis). 19,24,25 TPM and cylindromatosis are both uncommon disorders with an autosomal dominant pattern of inheritance. Both may have decreased male penetrance and expressivity, and, as a result, be more common in women.…”

Section: Discussionmentioning

confidence: 99%

Brooke–Spiegler syndrome

Jc¹,

Wa̧sik²,

Szybejko-Machaj³

et al. 2001

Acad Dermatol Venereol

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The Brooke-Spiegler syndrome is an autosomal dominant one characterized by cylindromas, trichoepitheliomas and occasionally spiradenomas. Within a given family, some members may have cylindromas whereas others may have trichoepitheliomas or both. We describe the coexistence of trichoepithelioma papulosum multiplex (also known as epithelioma adenoides cysticum of Brooke) and cylindromas in a 30-year-old man, and discuss the relationship between these two autosomal dominant syndromes.

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“…Considering the unique manifestations of this patient, a diagnosis of multiple basocellular carcinoma syndrome has been hypothe-sized. Bazex's syndrome consists of multiple BCCs on the face, hipotrichosis, generalized hypohidrosis or anhidrosis of the head and face, and follicular atrophoderma of the hands, feet, and elbows (6)(7)(8). Rombo's syndrome is characterized by vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, BCCs, and peripheral vasodilatation with cyanosis (9, 10).…”

Section: Discusionmentioning

confidence: 99%