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European Journal of Dermatology

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Mammo-renal and acro-mammo-renal syndromes. A nosologic approach, synopsis and update Volume 7, issue 4, June 1997

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Service of Dermatology, San Raffaele-Resnati Hospital, Viale Pisa, 18, I-20146 Milan, Italy.

Mammo-renal syndromes cover a heterogeneous spectrum of complex congenital anomalies – regarding formation, number and location – concomitantly affecting the breasts/nipples and the kidneys/urinary collecting system. A nosologic classification of these entities should include the following subsets: (1) “pure mammo-renal syndromes”, which occur as sporadic conditions, characterized by the involvement of mammary tissue and kidney alone; (2) mammo-renal syndromes associated with chromosomal disorders; (3) mammo-renal syndromes associated with dysmorphic genetic syndromes; (4) acro-mammo-renal syndromes, characterized by an additional coexistence of acral (limb) defects. This classificative approach would be a “starting point” for the correct arrangement and in-depth investigation of these embryogenetic disorders which are poorly considered in dermatological literature. Therefore, the evidence of inborn anomalies pertaining to the breast/nipple, or pectoralis major muscle, or an acral location of Becker’s naevus with ipsilateral polythelia, should always alert the physician who should then perform a renal ultrasound examination to quickly rule out the occurrence of hidden, concomitant nephrouropathies. Although the exact embryogenetic mechanisms are far from clear, mammo-renal and acro-mammo-renal syndromes represent distinct polytopic congenital malformations which reflect disturbances in those specific developmental fields involved (nipple/kidney/skin/limb buds).