09-12-2021-2039 - Epidermal Nevi, Neoplasms, Cysts, Ichthyosis hystrix Erythrokeratodermia progressiva Burns Keratitis-ichthyosis-deafness syndrome senter syndrome Hystrix-like ichthyosis–deafness syndrome KID syndrome HID syndrome AIDS-associated keratoderma Papulosquamous hyperkeratotic cutaneous conditions Epidermolytic hyperkeratosis necrobiosis

https://en.wikipedia.org/wiki/Category:Epidermal_nevi,_neoplasms,_and_cysts

Edward Lambert, an Englishman who suffered from ichthyosis hystrix.

Ichthyosis hystrix is a group of rare skin disorders in the ichthyosis family of skin disorders characterized by massive hyperkeratosis with an appearance like spiny scales.^[1] This term is also used to refer to a type of epidermal nevi with extensive bilateral distribution.^[1][2]

The symptoms of ichthyosis hystrix Curth-Macklin are similar to epidermolytic hyperkeratosis(NPS-2 type) but there is no blistering and the hyperkeratosis is verrucous or spine-like. The hyperkeratosis is brown-grey in colour and is most obvious on the arms and legs. It is an autosomal dominant condition and can be caused by errors to the KRT1 gene.^[3][4] It is named after Helen Ollendorff Curth (1899-1982), a German-Jewish dermatologist, and Madge Thurlow Macklin (1893–1962),^[5] an American medical geneticist, and is one of the first syndromes named after two women.^[6]

Ichthyosis hystrix, Lambert type[edit]

Also known as ichthyosis hystrix gravior or porcupine man. This disease is characterised by spiny scales which cover the entire body except the face, genitals, palms and soles. The only known cases were in Edward Lambert (known as the porcupine man) who was exhibited in front of the Royal Society in London in 1731 and three generations of his descendants. No cases of this disease are now known though some experts believe that it may have been a type of epidermolytic hyperkeratosis. From the history of the Lambert family the disease appears to have been an autosomal dominant condition.^[1][7]

Hystrix-like ichthyosis with deafness syndrome[edit]

HID syndrome is also known as ichthyosis hystrix, Rheydt type after the German city of Rheydt near Düsseldorf where it was first discovered. Symptoms are bilateral hearing loss and spiky hyperkeratotic masses which cover the whole body though the palms and soles are less badly affected. It can be differentiated from KID syndrome which also has symptoms of deafness and ichthyosis by the different distribution of hyperkeratosis. It is an autosomal dominant condition caused by a mutation to the GJB2gene (the same gene affected by KID syndrome).^[8][9]

https://en.wikipedia.org/wiki/Ichthyosis_hystrix

Keratitis–ichthyosis–deafness syndrome (also known as "Ichthyosiform erythroderma, corneal involvement, and deafness," and "KID syndrome,") presents at birth/infancy and is characterized by progressive corneal opacification, either mild generalized hyperkeratosis or discrete erythematous plaques, and neurosensory deafness.^{[2]:483, 513[3]:565}

It is caused by a mutation in connexin 26.^[4]

Keratitis-ichthyosis-deafness syndrome
Other names	"Erythrokeratodermia progressiva Burns"[1]

https://en.wikipedia.org/wiki/Keratitis–ichthyosis–deafness_syndrome

Senter syndrome is a cutaneous condition characterized by similar skin changes and congenital hearing impairment to keratitis–ichthyosis–deafness syndrome, but is associated with glycogen storage leading to hepatomegaly, hepatic cirrhosis, growth failure and mental retardation.^[2]

https://en.wikipedia.org/wiki/Senter_syndrome

Hystrix-like ichthyosis–deafness syndrome (also known as "HID syndrome"^[1]) is a cutaneous condition characterized by a keratoderma.^[1]

https://en.wikipedia.org/wiki/Hystrix-like_ichthyosis–deafness_syndrome

Acquired[edit]

• Acquired keratodermas
  • AIDS-associated keratoderma
  • Arsenical keratoses
  • Calluses
  • Climacteric keratoderma
  • Clavi (Corns)
  • Eczema
  • Human papillomavirus
  • Keratoderma blenorrhagicum
  • Lichen planus
  • Norwegian scabies
  • Paraneoplastic keratoderma
  • Psoriasis
  • Reactive arthritis
  • Secondary syphilis
  • Tinea pedis
  • Sézary syndrome
  • Tuberculosis verrucosa cutis
  • Drug-induced keratoderma^[3]

See also[edit]

• Palmoplantar keratoderma
• Skin lesion
• List of cutaneous conditions
• List of conditions caused by problems with junctional proteins

Categories: 

• Papulosquamous hyperkeratotic cutaneous conditions
• Cutaneous condition stubs

https://en.wikipedia.org/wiki/Keratoderma

Cutaneous keratosis, ulcer, atrophy, and necrobiosis

hidevte

Congenital malformations and deformations of integument / skin disease

Genodermatosis

Congenital ichthyosis/

erythrokeratodermia

AD

Ichthyosis vulgaris

AR

Congenital ichthyosiform erythroderma: Epidermolytic hyperkeratosis Lamellar ichthyosis Harlequin-type ichthyosis

Netherton syndrome Zunich–Kaye syndrome Sjögren–Larsson syndrome

XR

X-linked ichthyosis

Ungrouped

Ichthyosis bullosa of Siemens Ichthyosis follicularis Ichthyosis prematurity syndrome Ichthyosis–sclerosing cholangitis syndrome Nonbullous congenital ichthyosiform erythroderma Ichthyosis linearis circumflexa Ichthyosis hystrix

EB

and related

EBS EBS-K EBS-WC EBS-DM EBS-OG EBS-MD EBS-MP

JEB JEB-H Mitis Generalized atrophic JEB-PA

DEB DDEB RDEB

related: Costello syndrome Kindler syndrome Laryngoonychocutaneous syndrome Skin fragility syndrome

Ectodermal dysplasia

Naegeli syndrome/Dermatopathia pigmentosa reticularis Hay–Wells syndrome Hypohidrotic ectodermal dysplasia Focal dermal hypoplasia Ellis–van Creveld syndrome Rapp–Hodgkin syndrome/Hay–Wells syndrome

Elastic/Connective

Ehlers–Danlos syndromes Cutis laxa (Gerodermia osteodysplastica) Popliteal pterygium syndrome Pseudoxanthoma elasticum Van der Woude syndrome

Hyperkeratosis/

keratinopathy

PPK

diffuse: Diffuse epidermolytic palmoplantar keratoderma Diffuse nonepidermolytic palmoplantar keratoderma Palmoplantar keratoderma of Sybert Meleda disease

syndromic connexin Bart–Pumphrey syndrome Clouston's hidrotic ectodermal dysplasia Vohwinkel syndrome Corneodermatoosseous syndrome plakoglobin Naxos syndrome Scleroatrophic syndrome of Huriez Olmsted syndrome Cathepsin C Papillon–Lefèvre syndrome Haim–Munk syndrome Camisa disease focal: Focal palmoplantar keratoderma with oral mucosal hyperkeratosis Focal palmoplantar and gingival keratosis Howel–Evans syndrome Pachyonychia congenita Pachyonychia congenita type I Pachyonychia congenita type II Striate palmoplantar keratoderma Tyrosinemia type II

punctate: Acrokeratoelastoidosis of Costa Focal acral hyperkeratosis Keratosis punctata palmaris et plantaris Keratosis punctata of the palmar creases Schöpf–Schulz–Passarge syndrome Porokeratosis plantaris discreta Spiny keratoderma

ungrouped: Palmoplantar keratoderma and spastic paraplegia desmoplakin Carvajal syndrome connexin Erythrokeratodermia variabilis HID/KID

Other

Meleda disease Keratosis pilaris ATP2A2 Darier's disease Dyskeratosis congenita Lelis syndrome

Dyskeratosis congenita Keratolytic winter erythema Keratosis follicularis spinulosa decalvans Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome Keratosis pilaris atrophicans faciei Keratosis pilaris

Other

cadherin EEM syndrome immune system Hereditary lymphedema Mastocytosis/Urticaria pigmentosa Hailey–Hailey

see also Template:Congenital malformations and deformations of skin appendages, Template:Phakomatoses, Template:Pigmentation disorders, Template:DNA replication and repair-deficiency disorder

Developmental

anomalies

Midline

Dermoid cyst Encephalocele Nasal glioma PHACE association Sinus pericranii

Nevus

Capillary hemangioma Port-wine stain Nevus flammeus nuchae

Other/ungrouped

Aplasia cutis congenita Amniotic band syndrome Branchial cyst Cavernous venous malformation

Accessory nail of the fifth toe Bronchogenic cyst Congenital cartilaginous rest of the neck Congenital hypertrophy of the lateral fold of the hallux Congenital lip pit Congenital malformations of the dermatoglyphs Congenital preauricular fistula Congenital smooth muscle hamartoma Cystic lymphatic malformation Median raphe cyst Melanotic neuroectodermal tumor of infancy Mongolian spot Nasolacrimal duct cyst Omphalomesenteric duct cyst Poland anomaly Rapidly involuting congenital hemangioma Rosenthal–Kloepfer syndrome Skin dimple Superficial lymphatic malformation Thyroglossal duct cyst Verrucous vascular malformation Birthmark

https://en.wikipedia.org/wiki/Keratoderma