U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Keratosis follicularis spinulosa decalvans, X-linked(KFSDX)

MedGen UID:
854384
Concept ID:
C3887525
Congenital Abnormality; Disease or Syndrome
Synonym: KFSDX
 
Gene (location): MBTPS2 (Xp22.12)
 
Monarch Initiative: MONDO:0010637
OMIM®: 308800

Definition

Keratosis follicularis spinulosa decalvans is an uncommon genodermatosis chiefly characterized by widespread keratosis pilaris, progressive cicatricial alopecia of the scalp, eyebrows, and eyelashes, and an excess of affected males. Photophobia, blepharitis/conjunctivitis, and corneal dystrophy are characteristic ancillary findings. It is most often inherited as an X-linked trait (summary by Castori et al., 2009). Autosomal dominant inheritance has also been reported (KFSD; 612843). The term 'cum ophiasi' means 'with ophiasis,' i.e., baldness in 1 or more winding streaks about the head, which comes from the Greek for snake. Decalvans refers to the loss of hair. [from OMIM]

Clinical features

From HPO
Palmoplantar keratoderma
MedGen UID:
1635750
Concept ID:
C4551675
Disease or Syndrome
Abnormal thickening of the skin of the palms of the hands and the soles of the feet.
Photophobia
MedGen UID:
43220
Concept ID:
C0085636
Sign or Symptom
Excessive sensitivity to light with the sensation of discomfort or pain in the eyes due to exposure to bright light.
Blepharitis
MedGen UID:
598
Concept ID:
C0005741
Disease or Syndrome
Inflammation of the eyelids.
Conjunctivitis
MedGen UID:
1093
Concept ID:
C0009763
Disease or Syndrome
Inflammation of the conjunctiva.
Folliculitis
MedGen UID:
4752
Concept ID:
C0016436
Disease or Syndrome
Inflammatory cells within the wall and ostia of the hair follicle, creating a follicular-based pustule.
Keratitis
MedGen UID:
44013
Concept ID:
C0022568
Disease or Syndrome
Inflammation of the cornea.
Ectropion
MedGen UID:
4448
Concept ID:
C0013592
Disease or Syndrome
An outward turning (eversion) or rotation of the eyelid margin.
Sparse eyebrow
MedGen UID:
371332
Concept ID:
C1832446
Finding
Decreased density/number of eyebrow hairs.
Scarring alopecia of scalp
MedGen UID:
812631
Concept ID:
C3806301
Finding
Dry skin
MedGen UID:
56250
Concept ID:
C0151908
Sign or Symptom
Skin characterized by the lack of natural or normal moisture.
Nail dystrophy
MedGen UID:
66368
Concept ID:
C0221260
Disease or Syndrome
Onychodystrophy (nail dystrophy) refers to nail changes apart from changes of the color (nail dyschromia) and involves partial or complete disruption of the various keratinous layers of the nail plate.
Facial erythema
MedGen UID:
65986
Concept ID:
C0239488
Finding
Redness of the skin of the face, caused by hyperemia of the capillaries in the lower layers 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.
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.
Sparse eyelashes
MedGen UID:
375151
Concept ID:
C1843300
Finding
Decreased density/number of eyelashes.
Perifollicular fibrosis
MedGen UID:
440567
Concept ID:
C2748531
Finding
Presence of excess fibrous connective tissue surrounding hair follicules.
Dystrophic fingernails
MedGen UID:
764340
Concept ID:
C3551426
Finding
The presence of misshapen or partially destroyed nail plates, often with accumulation of soft, yellow keratin between the dystrophic nail plate and nail bed, resulting in elevation of the nail plate.
Corneal dystrophy
MedGen UID:
3619
Concept ID:
C0010036
Disease or Syndrome
The term corneal dystrophy embraces a heterogenous group of bilateral genetically determined non-inflammatory corneal diseases that are restricted to the cornea.

Professional guidelines

PubMed

Bornholdt D, Atkinson TP, Bouadjar B, Catteau B, Cox H, De Silva D, Fischer J, Gunasekera CN, Hadj-Rabia S, Happle R, Holder-Espinasse M, Kaminski E, König A, Mégarbané A, Mégarbané H, Neidel U, Oeffner F, Oji V, Theos A, Traupe H, Vahlquist A, van Bon BW, Virtanen M, Grzeschik KH
Hum Mutat 2013 Apr;34(4):587-94. Epub 2013 Mar 8 doi: 10.1002/humu.22275. PMID: 23316014

Recent clinical studies

Etiology

Lan X, Qiao R, Sun J, Song H, Gao M, Mo R, Song Z, Yang Y, Jiang Y
J Dermatol 2024 Feb;51(2):253-260. Epub 2023 Dec 12 doi: 10.1111/1346-8138.17026. PMID: 38087855

Diagnosis

Lan X, Qiao R, Sun J, Song H, Gao M, Mo R, Song Z, Yang Y, Jiang Y
J Dermatol 2024 Feb;51(2):253-260. Epub 2023 Dec 12 doi: 10.1111/1346-8138.17026. PMID: 38087855
Chen C, Xu C, Li H, Jia M, Tang S
Int J Dermatol 2019 Apr;58(4):493-496. Epub 2018 Jun 27 doi: 10.1111/ijd.14129. PMID: 29951998
Bolduc C, Sperling LC, Shapiro J
J Am Acad Dermatol 2016 Dec;75(6):1101-1117. doi: 10.1016/j.jaad.2015.01.056. PMID: 27846945
Zhang J, Wang Y, Cheng R, Ni C, Liang J, Li M, Yao Z
Clin Exp Dermatol 2016 Oct;41(7):757-60. doi: 10.1111/ced.12889. PMID: 27663151
Bornholdt D, Atkinson TP, Bouadjar B, Catteau B, Cox H, De Silva D, Fischer J, Gunasekera CN, Hadj-Rabia S, Happle R, Holder-Espinasse M, Kaminski E, König A, Mégarbané A, Mégarbané H, Neidel U, Oeffner F, Oji V, Theos A, Traupe H, Vahlquist A, van Bon BW, Virtanen M, Grzeschik KH
Hum Mutat 2013 Apr;34(4):587-94. Epub 2013 Mar 8 doi: 10.1002/humu.22275. PMID: 23316014

Clinical prediction guides

Lan X, Qiao R, Sun J, Song H, Gao M, Mo R, Song Z, Yang Y, Jiang Y
J Dermatol 2024 Feb;51(2):253-260. Epub 2023 Dec 12 doi: 10.1111/1346-8138.17026. PMID: 38087855
Lim PJ, Marcionelli G, Srikanthan P, Ndarugendamwo T, Pinner J, Rohrbach M, Giunta C
Front Endocrinol (Lausanne) 2023;14:1195704. Epub 2023 May 25 doi: 10.3389/fendo.2023.1195704. PMID: 37305034Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...