We are presenting a case of acantholytic palmoplantar keratoderma (Vörner type) which various authors consider to be an epidermolytic hyperkeratosis in a local form. It is a genodermatosis that is transmitted by an autosomal dominant mode. This genetic anomaly concerns a gene which is responsible for K9 Keratin, that is specifically related to the palmoplantar areas. The subject described is a young adult twenty-eight years old with a negative family anamnesis for genodermatoses. This pathology was present at birthas an erythema which after a few weeks was replaced by a palmoplantar keratosis without any associated symptoms. The actual aspect is that of a diffused palmoplantar keratosis with a wrinkled surface and a yellow-brownish colour. It is not spread out the back of hands and feet and it is delimited on its edge by a small erythematous border. This keratodermia is so high a degree to cause difficulty to walk and in the capacity to hold with the hands. Moreover, there is an accentuated hyperhidrosis and bromidrosis with a stinking smell which can be sensed even at a long distance. From a histological point of view, as well as a remarkable orthohyperkeratosis, there are also acanthosis, elongation of the dermal papillae, perinuclear vacuolization and a granular degeneration with acanthokeratolysis of keratinocites in the medium and high portion of the malpighian layer. The Vörner palmoplantar keratoderma is at present classified among hereditary forms spread without any associated symptoms and it is considered to be a local form of congenital ichthyosiform erythroderma. It can be taken into consideration the differential diagnosis with the Thost-Unna type and with the Meleda type. This clinical case is considered to be interesting enough to be. studied deeply both for the level of functional problems and for the notable state of hyperhidrosis and bromidrosis, functional problems which are not always present in the Vörner disease. Besides the absence of palmoplantar keratodermas, between ancestors and sisters (and their descendants), enable us to classify this case among the sporadic cases due to spontaneous mutation, which in any case are very few.

Acantholytic palmo plantar keratoderma (Vörner type)

CALTABIANO, ROSARIO
1998

Abstract

We are presenting a case of acantholytic palmoplantar keratoderma (Vörner type) which various authors consider to be an epidermolytic hyperkeratosis in a local form. It is a genodermatosis that is transmitted by an autosomal dominant mode. This genetic anomaly concerns a gene which is responsible for K9 Keratin, that is specifically related to the palmoplantar areas. The subject described is a young adult twenty-eight years old with a negative family anamnesis for genodermatoses. This pathology was present at birthas an erythema which after a few weeks was replaced by a palmoplantar keratosis without any associated symptoms. The actual aspect is that of a diffused palmoplantar keratosis with a wrinkled surface and a yellow-brownish colour. It is not spread out the back of hands and feet and it is delimited on its edge by a small erythematous border. This keratodermia is so high a degree to cause difficulty to walk and in the capacity to hold with the hands. Moreover, there is an accentuated hyperhidrosis and bromidrosis with a stinking smell which can be sensed even at a long distance. From a histological point of view, as well as a remarkable orthohyperkeratosis, there are also acanthosis, elongation of the dermal papillae, perinuclear vacuolization and a granular degeneration with acanthokeratolysis of keratinocites in the medium and high portion of the malpighian layer. The Vörner palmoplantar keratoderma is at present classified among hereditary forms spread without any associated symptoms and it is considered to be a local form of congenital ichthyosiform erythroderma. It can be taken into consideration the differential diagnosis with the Thost-Unna type and with the Meleda type. This clinical case is considered to be interesting enough to be. studied deeply both for the level of functional problems and for the notable state of hyperhidrosis and bromidrosis, functional problems which are not always present in the Vörner disease. Besides the absence of palmoplantar keratodermas, between ancestors and sisters (and their descendants), enable us to classify this case among the sporadic cases due to spontaneous mutation, which in any case are very few.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/20.500.11769/15789
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