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Unna-Thost syndrome
Also known as:
Brauer's syndrome
Brünauer's syndrome
Fuhs' syndrome
Brünauer-Fuhs-Siemens syndrome
Keratosis palmoplantaris striata Brünauer-Fuhs
Greither's keratosis, type Brünauer-Fuhs
Associated persons:
August Brauer
Stefan Robert Brünauer
Herbert Fuhs
Aloys Greither
Hermann Werner Siemens
Herrmann Arthur Thost
Paul Gerson Unna
Description:
A rare hereditary skin disease with variable involvement of the palms and soles. Typically, the lesions are thick, horny, hard, yellowish plaques with waxy smooth surfaces, although in some cases they may be pitted and verrucous, surrounded by erythematous halos. Occasionally associated corneal opacities, pilitorti, sensorineural hearing loss, hypohidrosis, dental abnormalities. Inheritance is autosomal dominant. According to one author, it also occurs in an acquired form preceding hyperhidrosis
We thank Andre Trombeta for information submitted.
Bibliography:
- A. Thost:
Über erbliche Ichtyosis palmaris et plantaris cornea. Dissertation, Heidelberg, 1880.
- P. G. Unna:
Über das Keratoma palmare et plantare hereditarium.
Vierteljahrsschrift für Dermatologie und Syphilis, Wien, 1883, 15: 231.
- A. Brauer:
Über eine besondere Form des hereditären Keratoms (Keratoma dissipatum hereditarium palmare et plantare).
Archiv für Dermatologie und Syphilis, Berlin, 1912, 114: 211-236.
- S. R. Brünauer:
Zur Vererbung des Keratoms hereditarium palmare et plantare.
Acta dermato-venereologica, Stockholm, 1923-1924, 4: 489-503.
- A. Greither:
Keratosis extremitatum hereditaria progrediens mit dominanten Erbgang.
Der Hautarzt, Berlin, 1952, 3: 198-203.
- H. W. Siemens and Winkler:
Über regelmässige Dominanz und andere Vererbungsmodi bei Keratosis plantaris et palmaris (Verhornung der Hand- und Fussflächen). Sitzungsberichte der Gesellschaft für Morphologie und Physiologie in München, 1924 (1925), 36: 45-47.
- S. R. Brünauer:
Zur Symptomatologie und Histologie der kongenitalen Dyskeratosen.
Dermatologische Zeitschrift, Basel, 1925, 42: 6-26.
- H. Fuhs:
Keratoma palmare et plantare. Zentralblatt für Haut- und Geschlechtskrankheiten sowie deren Grenzgebiete, Berlin, 1933, 46: 292.
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