U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Seborrhea-like dermatitis with psoriasiform elements

MedGen UID:
342832
Concept ID:
C1853258
Disease or Syndrome
Synonym: Seborrhea-Like Dermatitis with Psoriasiform Elements
SNOMED CT: Seborrhea-like dermatitis with psoriasiform elements (782910009)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Gene (location): ZNF750 (17q25.3)
 
Monarch Initiative: MONDO:0012446
OMIM®: 610227
Orphanet: ORPHA168606

Definition

A rare genetic epidermal disorder with characteristics of a chronic diffuse fine scaly erythematous rash on the face (predominantly the chin, nasolabial folds, eyebrows) around the earlobes and over the scalp, associated with hyperkeratosis over elbows, knees, palms, soles and metacarpophalangeal joints, in the absence of associated rheumatological or neurological disorders. Cold weather, emotional stress and strenuous physical activity may exacerbate symptoms. There is evidence the disease is caused by mutation in the ZNF750 gene. [from SNOMEDCT_US]

Clinical features

From HPO
Seborrheic dermatitis
MedGen UID:
19912
Concept ID:
C0036508
Disease or Syndrome
Seborrheic dermatitis is a form of eczema which is closely related to dandruff. It causes dry or greasy peeling of the scalp, eyebrows, and face, and sometimes trunk.
Epidermal acanthosis
MedGen UID:
65136
Concept ID:
C0221270
Finding
Diffuse hypertrophy or thickening of the stratum spinosum of the epidermis (prickle cell layer of the skin).
Keratosis pilaris
MedGen UID:
82664
Concept ID:
C0263383
Disease or Syndrome
An anomaly of the hair follicles of the skin that typically presents as small, rough, brown folliculocentric papules distributed over characteristic areas of the skin, particularly the outer-upper arms and thighs.
Hyperkeratosis
MedGen UID:
209030
Concept ID:
C0870082
Disease or Syndrome
Hyperkeratosis is thickening of the outer layer of the skin, the stratum corneum, which is composed of large, polyhedral, plate-like envelopes filled with keratin which are the dead cells that have migrated up from the stratum granulosum.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSeborrhea-like dermatitis with psoriasiform elements
Follow this link to review classifications for Seborrhea-like dermatitis with psoriasiform elements in Orphanet.

Recent clinical studies

Therapy

Imai Y, Ayithan N, Wu X, Yuan Y, Wang L, Hwang ST
J Immunol 2015 Jul 15;195(2):421-5. Epub 2015 Jun 5 doi: 10.4049/jimmunol.1500448. PMID: 26048148Free PMC Article

Clinical prediction guides

Imai Y, Ayithan N, Wu X, Yuan Y, Wang L, Hwang ST
J Immunol 2015 Jul 15;195(2):421-5. Epub 2015 Jun 5 doi: 10.4049/jimmunol.1500448. PMID: 26048148Free PMC Article

Supplemental Content

Recent activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...