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Congenital hypotrichosis with juvenile macular dystrophy(HJMD)

MedGen UID:
316921
Concept ID:
C1832162
Disease or Syndrome
Synonyms: HJMD; HYPOTRICHOSIS WITH CONE-ROD DYSTROPHY; Juvenile macular degeneration and hypotrichosis; Juvenile macular dystrophy and congenital hypotrichosis
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
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 individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Gene (location): CDH3 (16q22.1)
 
Monarch Initiative: MONDO:0011107
OMIM®: 601553
Orphanet: ORPHA1573

Definition

Congenital hypotrichosis with juvenile macular dystrophy (HJMD) is an autosomal recessive disorder characterized by hair loss followed by progressive macular degeneration and early blindness. Scalp hair is lost during the first months of life, with onset of retinal degeneration and vision loss a few years to 2 decades later (summary by Sprecher et al., 2001 and Indelman et al., 2002). [from OMIM]

Clinical features

From HPO
Abnormality of the hand
MedGen UID:
6715
Concept ID:
C0018564
Anatomical Abnormality
An abnormality affecting one or both hands.
Abnormal foot morphology
MedGen UID:
1762829
Concept ID:
C5399834
Anatomical Abnormality
An abnormality of the skeleton of foot.
Abnormal dental morphology
MedGen UID:
11849
Concept ID:
C0040427
Anatomical Abnormality
An abnormality of the morphology of the tooth.
Abnormality of the dentition
MedGen UID:
78084
Concept ID:
C0262444
Finding
Any abnormality of the teeth.
Sparse scalp hair
MedGen UID:
346499
Concept ID:
C1857042
Finding
Decreased number of hairs per unit area of skin of the scalp.
Epidermoid cysts
MedGen UID:
41829
Concept ID:
C0014511
Anatomical Abnormality
Nontender, round and firm, but slightly compressible, intradermal or subcutaneous cyst measuring 0.5-5 cm in diameter. Epidermal cysts are intradermal or subcutaneous tumors, grow slowly and occur on the face, neck, back and scrotum. They usually appear at or around puberty, and as a rule an affected individual has one solitary or a few cysts.
Pallor
MedGen UID:
10547
Concept ID:
C0030232
Finding
Abnormally pale skin.
Pili torti
MedGen UID:
82670
Concept ID:
C0263491
Finding
Pili (from Latin pilus, hair) torti (from Latin tortus, twisted) refers to short and brittle hairs that appear flattened and twisted when viewed through a microscope.
Phrynoderma
MedGen UID:
83101
Concept ID:
C0334013
Disease or Syndrome
A skin condition characterized by excessive development of keratin in hair follicles, resulting in rough, cone-shaped, elevated papules resulting from closure of hair follicles with a white plug of sebum.
Abnormal nail morphology
MedGen UID:
163115
Concept ID:
C0853087
Anatomical Abnormality
Abnormal structure or appearance of the nail.
Reduced terminal:vellus ratio
MedGen UID:
1706135
Concept ID:
C5139385
Finding
A terminal:vellus ratio under 4:1 is characteristic of androgenetic alopecia.
Absent sebaceous glands
MedGen UID:
1706541
Concept ID:
C5139558
Anatomical Abnormality
Absence of the sebaceous gland, the holocrine gland that secretes sebum into the hair follicles, or in hairless areas into ducts.
Sparse hair
MedGen UID:
1790211
Concept ID:
C5551005
Finding
Reduced density of hairs.
Macular degeneration
MedGen UID:
7434
Concept ID:
C0024437
Disease or Syndrome
A nonspecific term denoting degeneration of the retinal pigment epithelium and/or retinal photoreceptor cells of the macula lutea.
Reduced visual acuity
MedGen UID:
65889
Concept ID:
C0234632
Finding
Diminished clarity of vision.
Macular atrophy
MedGen UID:
140841
Concept ID:
C0423421
Finding
Well-demarcated area(s) of partial or complete depigmentation in the macula, reflecting atrophy of the retinal pigment epithelium with associated retinal photoreceptor loss.
Blindness
MedGen UID:
99138
Concept ID:
C0456909
Disease or Syndrome
Blindness is the condition of lacking visual perception defined as a profound reduction in visual perception. On the 6m visual acuity scale, blindness is defined as less than 3/60. On the 20ft visual acuity scale, blindness is defined as less than 20/400. On the decimal visual acuity scale, blindness is defined as less than 0.05. Blindness is typically characterized by a visual field of no greater than 10 degrees in radius around central fixation.
Macular dystrophy
MedGen UID:
196451
Concept ID:
C0730292
Disease or Syndrome
Macular dystrophy is a nonspecific term for premature retinal cell aging and cell death, generally confied to the macula in which no clear extrinsic cause is evident.
Macular hyperpigmentation
MedGen UID:
488933
Concept ID:
C0745109
Finding
Increased amount of pigmentation in the macula lutea.
Choriocapillaris atrophy
MedGen UID:
477389
Concept ID:
C3275758
Finding
Atrophy of the capillary lamina of choroid.
Subretinal deposits
MedGen UID:
1613321
Concept ID:
C4531024
Finding
Deposits accumulating between the outer retina and the retinal pigment epithelium.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital hypotrichosis with juvenile macular dystrophy
Follow this link to review classifications for Congenital hypotrichosis with juvenile macular dystrophy in Orphanet.

Recent clinical studies

Diagnosis

Hayashi T, Katagiri S, Kubota D, Mizobuchi K, Ishiuji Y, Asahina A, Kameya S, Nakano T
Mol Genet Genomic Med 2021 Jun;9(6):e1688. Epub 2021 Apr 9 doi: 10.1002/mgg3.1688. PMID: 33837674Free PMC Article
Nasser F, Kempf M, Kurtenbach A, Stöhr H, Weber BHF, Neuhaus C, Rating P, Zrenner E
Ophthalmic Res 2020;63(2):141-151. Epub 2020 Jan 10 doi: 10.1159/000504757. PMID: 31927556
Hull S, Arno G, Robson AG, Broadgate S, Plagnol V, McKibbin M, Halford S, Michaelides M, Holder GE, Moore AT, Khan KN, Webster AR
JAMA Ophthalmol 2016 Sep 1;134(9):992-1000. doi: 10.1001/jamaophthalmol.2016.2089. PMID: 27386845
Frainer RH, Abreu LB, Pinto GM, Carvalho AV, Meneghello LP
An Bras Dermatol 2013 Jan-Feb;88(1):135-7. doi: 10.1590/s0365-05962013000100023. PMID: 23539021Free PMC Article
Indelman M, Leibu R, Jammal A, Bergman R, Sprecher E
Br J Dermatol 2005 Sep;153(3):635-8. doi: 10.1111/j.1365-2133.2005.06734.x. PMID: 16120155

Prognosis

Saeidian AH, Vahidnezhad H, Youssefian L, Sotudeh S, Sargazi M, Zeinali S, Uitto J
Mol Genet Genomic Med 2019 Nov;7(11):e975. Epub 2019 Sep 27 doi: 10.1002/mgg3.975. PMID: 31560841Free PMC Article
Indelman M, Leibu R, Jammal A, Bergman R, Sprecher E
Br J Dermatol 2005 Sep;153(3):635-8. doi: 10.1111/j.1365-2133.2005.06734.x. PMID: 16120155

Clinical prediction guides

Nasser F, Kempf M, Kurtenbach A, Stöhr H, Weber BHF, Neuhaus C, Rating P, Zrenner E
Ophthalmic Res 2020;63(2):141-151. Epub 2020 Jan 10 doi: 10.1159/000504757. PMID: 31927556
Saeidian AH, Vahidnezhad H, Youssefian L, Sotudeh S, Sargazi M, Zeinali S, Uitto J
Mol Genet Genomic Med 2019 Nov;7(11):e975. Epub 2019 Sep 27 doi: 10.1002/mgg3.975. PMID: 31560841Free PMC Article
Hull S, Arno G, Robson AG, Broadgate S, Plagnol V, McKibbin M, Halford S, Michaelides M, Holder GE, Moore AT, Khan KN, Webster AR
JAMA Ophthalmol 2016 Sep 1;134(9):992-1000. doi: 10.1001/jamaophthalmol.2016.2089. PMID: 27386845
Van Marck V, Stove C, Van Den Bossche K, Stove V, Paredes J, Vander Haeghen Y, Bracke M
Cancer Res 2005 Oct 1;65(19):8774-83. doi: 10.1158/0008-5472.CAN-04-4414. PMID: 16204047
Indelman M, Leibu R, Jammal A, Bergman R, Sprecher E
Br J Dermatol 2005 Sep;153(3):635-8. doi: 10.1111/j.1365-2133.2005.06734.x. PMID: 16120155

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