Hanhart syndrome

Classification according to ICD-10
Q82.8	Keratodermia palmoplantaris papulosa
E34.3	Dwarfism
Q87.2	Congenital malformation syndromes with predominant involvement of the extremities
G11.4	Hereditary spastic paraplegia
ICD-10 online (WHO version 2019)

The term Hanhart syndrome is not clearly defined in the specialist literature.

The naming refers to the first author of the first descriptions by the Swiss internist and human geneticist Ernst Hanhart (1891–1973).

It describes the following syndromes:

Keratoderma with lipomas
Heredodegenerative dwarfism
Oro-acral malformation complex
Familial spastic paraplegia

The syndromes are not to be confused with the Richner-Hanhart syndrome .

Keratoderma with lipomas

Synonyms are: Keratosis plamo-plantaris with systematized lipomas ; Latin Keratodermia palmo-plantaris papulosa Hanhart

The name refers to a description from 1947.

Heredodegenerative dwarfism

Synonyms are: Hanhart Dwarfism ; English Hanhart's syndrome I

The name refers to a description from 1925.

Oro-acral malformation complex

Synonyms are: aglossia-adactyly syndrome; Hanhart Syndrome; Jussieu Syndrome; Hypoglossia-hypodactyly syndrome English Hanhart's syndrome II

The name refers to a description from 1950.

Familial spastic paraplegia

Synonyms are: Hanhart syndrome IV; English Hanhart's disease

It is an autosomal dominant inherited familial spastic paraplegia with intellectual disability.

The name refers to a description from 1936.

Individual evidence

↑ ^a ^b Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
^ E. Hanhart: New special forms of keratosis palmo-plantaris, u. a. a regular-dominant one with systematized lipomas, further 2 single-recessive ones with dementia and z. Sometimes with corneal changes in the eye (ectodermal syndrome). In: Dermatologica . Vol. 94, 1947, pp. 286-308.
↑ E. Hanhart: About heredodegenerative dwarfism with dystrophia adiposo-genitalis. Based on studies of three clans of proportioned dwarfs. In: Archive of the Julius Klaus Foundation for Heredity Research, Social Anthropology and Racial Hygiene . Zurich, Vol. 1, 1925, p. 181.
^ Who named it Hanhart's syndrome I.
^ Who named it Hanhart's syndrome II
↑ Hypoglossia-Hypodactyly Syndrome. In: Orphanet (Rare Disease Database).
↑ E. Hanhart: About the combination of peromelia with micrognathia, a new syndrome in humans, corresponding to the acroteriasis congenita by Wriedt and Mohr in cattle. In: Archive of the Julius Klaus Foundation for Heredity Research, Social Anthropology and Racial Hygiene . Vol. 25, 1950, pp. 531-543.
↑ who named it Hanhart's disease
↑ E. Hanhart: A clan with a single recessive Diplegia spastica infantilis (Little-scher) from a Swiss inbred area. In: Erbarzt , 1936.

[Leiber-1] Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .

[2] E. Hanhart: New special forms of keratosis palmo-plantaris, u. a. a regular-dominant one with systematized lipomas, further 2 single-recessive ones with dementia and z. Sometimes with corneal changes in the eye (ectodermal syndrome). In: Dermatologica . Vol. 94, 1947, pp. 286-308.

[3] E. Hanhart: About heredodegenerative dwarfism with dystrophia adiposo-genitalis. Based on studies of three clans of proportioned dwarfs. In: Archive of the Julius Klaus Foundation for Heredity Research, Social Anthropology and Racial Hygiene . Zurich, Vol. 1, 1925, p. 181.

[4] Who named it Hanhart's syndrome I.

[5] Who named it Hanhart's syndrome II

[6] Hypoglossia-Hypodactyly Syndrome. In: Orphanet (Rare Disease Database).

[7] E. Hanhart: About the combination of peromelia with micrognathia, a new syndrome in humans, corresponding to the acroteriasis congenita by Wriedt and Mohr in cattle. In: Archive of the Julius Klaus Foundation for Heredity Research, Social Anthropology and Racial Hygiene . Vol. 25, 1950, pp. 531-543.

[8] who named it Hanhart's disease

[9] E. Hanhart: A clan with a single recessive Diplegia spastica infantilis (Little-scher) from a Swiss inbred area. In: Erbarzt , 1936.