U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Porokeratosis 6, disseminated superficial actinic type(POROK6; DSAP4)

MedGen UID:
394063
Concept ID:
C2676508
Disease or Syndrome
Synonyms: POROK6; POROKERATOSIS 6, DISSEMINATED SUPERFICIAL; Porokeratosis 6, multiple types
 
Monarch Initiative: MONDO:0012874
OMIM®: 612353

Definition

Porokeratosis is a rare skin disorder characterized by one or more annular plaques with a surrounding raised horny border that spreads centrifugally. Variants of porokeratosis have been described that differ in morphologic shapes, distribution, and clinical course (Schamroth et al., 1997). However, as noted by Sybert (2010), reports of several families with expression of more than one variant of porokeratosis among members, and of individuals expressing more than one variant, suggest that the distinctions among these variants may be artificial. Disseminated superficial actinic porokeratosis (DSAP) is the most common subtype of porokeratosis. It is characterized by multiple small, annular, anhidrotic, keratotic lesions that are located predominantly on sun-exposed areas of the skin, such as the face, neck, and distal limbs. The lesions typically begin to develop in adolescence and reach near-complete penetrance by the third or fourth decade of life (summary by Wu et al., 2004 and Zhang et al., 2012). [from OMIM]

Clinical features

From HPO
Porokeratosis
MedGen UID:
56518
Concept ID:
C0162839
Congenital Abnormality
A clonal disorder of keratinization with one or multiple atrophic patches surrounded by a clinically and histologically distinctive hyperkeratotic ridgelike border called the cornoid lamella.

Recent clinical studies

Diagnosis

Sommerlad M, Lock A, Moir G, McGregor J, Bull R, Cerio R, Harwood C
Br J Dermatol 2016 Dec;175(6):1342-1345. Epub 2016 Aug 17 doi: 10.1111/bjd.14669. PMID: 27086492
Boente Mdel C, López-Baró AM, Frontini Mdel V, Asial RA
Pediatr Dermatol 2003 Nov-Dec;20(6):514-8. doi: 10.1111/j.1525-1470.2003.20613.x. PMID: 14651573
Patrizi A, D'Acunto C, Passarini B, Neri I
Acta Derm Venereol 2000 Jul-Aug;80(4):302-4. doi: 10.1080/000155500750012243. PMID: 11028869

Therapy

Sommerlad M, Lock A, Moir G, McGregor J, Bull R, Cerio R, Harwood C
Br J Dermatol 2016 Dec;175(6):1342-1345. Epub 2016 Aug 17 doi: 10.1111/bjd.14669. PMID: 27086492

Clinical prediction guides

Jurecka W, Neumann RA, Knobler RM
J Am Acad Dermatol 1991 Jan;24(1):96-101. doi: 10.1016/0190-9622(91)70019-x. PMID: 1999539

Supplemental Content

Recent activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...