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Peeling skin syndrome 6(PSS6)

MedGen UID:
1648406
Concept ID:
C4748093
Disease or Syndrome
Synonym: PSS6
 
Gene (location): FLG2 (1q21.3)
 
Monarch Initiative: MONDO:0054852
OMIM®: 618084

Definition

Peeling skin syndrome-6 (PSS6) is characterized by generalized ichthyotic dry skin and bullous peeling lesions on the trunk and limbs at sites of minor trauma. There is residual hyperpigmentation in areas of healing, but no scarring. Skin symptoms are exacerbated by warmth and humidity; however, the disorder improves markedly with age (Bolling et al., 2018; Mohamad et al., 2018). For a discussion of genetic heterogeneity of peeling skin syndrome, see PSS1 (270300). [from OMIM]

Clinical features

From HPO
Atopic eczema
MedGen UID:
41502
Concept ID:
C0011615
Disease or Syndrome
Atopic dermatitis (ATOD), also known as eczema, is a common chronic pruritic inflammatory skin disease with a strong genetic component. Onset typically occurs during the first 2 years of life (review by Soderhall et al., 2007). Genetic Heterogeneity of Atopic Dermatitis Many inflammatory diseases, such as atopic eczema, are genetically complex, with multiple alleles at several loci thought to be involved in their pathogenesis. Several susceptibility loci for atopic dermatitis have been identified: ATOD1 on chromosome 3q21, ATOD2 (605803) on chromosome 1q21, ATOD3 (605804) on chromosome 20p, ATOD4 (605805) on chromosome 17q25.3, ATOD5 (603165) on chromosome 13q12-q14, ATOD6 (605845) on chromosome 5q31-q33, ATOD7 (613064) on chromosome 11q13.5, ATOD8 (613518) on chromosome 4q22.1, and ATOD9 (613519) on chromosome 3p24.
Parakeratosis
MedGen UID:
10572
Concept ID:
C0030436
Disease or Syndrome
Abnormal formation of the keratinocytes of the epidermis characterized by persistence of nuclei, incomplete formation of keratin, and moistness and swelling of the keratinocytes.
Pruritus
MedGen UID:
19534
Concept ID:
C0033774
Sign or Symptom
Pruritus is an itch or a sensation that makes a person want to scratch. This term refers to an abnormally increased disposition to experience pruritus.
Dry skin
MedGen UID:
56250
Concept ID:
C0151908
Sign or Symptom
Skin characterized by the lack of natural or normal moisture.
Scaling skin
MedGen UID:
472970
Concept ID:
C0237849
Finding
Refers to the loss of the outer layer of the epidermis in large, scale-like flakes.
Orthokeratosis
MedGen UID:
375169
Concept ID:
C1843359
Finding
Formation of an anuclear keratin layer

Recent clinical studies

Etiology

Srinivasaraghavan R, Krishnamurthy S, Chandar R, Mahadevan S, Chandrashekar L, Rajesh NG
Pediatr Dermatol 2015 Mar-Apr;32(2):244-7. Epub 2014 Sep 5 doi: 10.1111/pde.12374. PMID: 25196305
Cassidy AJ, van Steensel MA, Steijlen PM, van Geel M, van der Velden J, Morley SM, Terrinoni A, Melino G, Candi E, McLean WH
Am J Hum Genet 2005 Dec;77(6):909-17. Epub 2005 Oct 11 doi: 10.1086/497707. PMID: 16380904Free PMC Article

Diagnosis

Gordon H, Yap P, Hsiao KC, Watson M, Purvis D
Pediatr Dermatol 2022 Mar;39(2):268-272. Epub 2022 Feb 17 doi: 10.1111/pde.14939. PMID: 35178752Free PMC Article
Pradhan S, Sirka CS, Dash G
Skinmed 2021;19(4):305-307. Epub 2021 Aug 1 PMID: 34526207
Srinivasaraghavan R, Krishnamurthy S, Chandar R, Mahadevan S, Chandrashekar L, Rajesh NG
Pediatr Dermatol 2015 Mar-Apr;32(2):244-7. Epub 2014 Sep 5 doi: 10.1111/pde.12374. PMID: 25196305
Teye K, Hamada T, Krol RP, Numata S, Ishii N, Matsuda M, Ohata C, Furumura M, Hashimoto T
J Dermatol Sci 2014 Jul;75(1):36-42. Epub 2014 Apr 15 doi: 10.1016/j.jdermsci.2014.04.003. PMID: 24794518
Hashimoto K, Hamzavi I, Tanaka K, Shwayder T
J Am Acad Dermatol 2000 Dec;43(6):1112-9. doi: 10.1067/mjd.2000.103645. PMID: 11100033

Therapy

Srinivasaraghavan R, Krishnamurthy S, Chandar R, Mahadevan S, Chandrashekar L, Rajesh NG
Pediatr Dermatol 2015 Mar-Apr;32(2):244-7. Epub 2014 Sep 5 doi: 10.1111/pde.12374. PMID: 25196305

Prognosis

Srinivasaraghavan R, Krishnamurthy S, Chandar R, Mahadevan S, Chandrashekar L, Rajesh NG
Pediatr Dermatol 2015 Mar-Apr;32(2):244-7. Epub 2014 Sep 5 doi: 10.1111/pde.12374. PMID: 25196305
Janin A, Copin MC, Dubos JP, Rouland V, Delaporte E, Blanchet-Bardon C
Arch Pathol Lab Med 1996 Jul;120(7):662-5. PMID: 8757472

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