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Actinic prurigo(HPLE)

MedGen UID:
98348
Concept ID:
C0406217
Disease or Syndrome
Synonyms: Actinic Prurigo; Polymorphic Light Eruption, Hereditary
SNOMED CT: Summer prurigo of Hutchinson (201015007); Hutchinson's summer prurigo (201015007); Actinic prurigo (201015007); Familial actinic prurigo (201015007); Hydroa estivale (201015007); Hereditary polymorphous light eruption of American Indians (201015007); Familial polymorphous light eruption of American Indians (201015007)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0008273
OMIM®: 174770
Orphanet: ORPHA330061

Definition

Hereditary polymorphic light eruption is a form of photosensitivity found in the American Indians of the central plains of Canada and the United States and in the Indians of Central and South America. The disorder has also been called familial actinic prurigo, solar dermatitis, and hydroa aestivale. In northern latitudes, skin lesions appear on exposed areas early in spring, become severe during the summer, and abate in the fall. Usually the disorder appears in childhood with eczematous crusted eruptions on the face and arms. Fissured, crusted exudative cheilitis develops on the lips, especially the lower lip. The dorsum of the hands, the laterodorsal aspects of the forearms, and the lower half of the arms often show excoriated papular and nodular lesions. Children frequently have complicating pyoderma. Adults usually exhibit an erythematous plaquelike eruption on the face and other exposed areas. The disease is more severe in children than in adults. Glomerulonephritis can follow streptococcal pyoderma (summary by Fusaro and Johnson, 1980). [from OMIM]

Clinical features

From HPO
Glomerulonephritis
MedGen UID:
6616
Concept ID:
C0017658
Disease or Syndrome
Inflammation of the renal glomeruli.
Pyoderma
MedGen UID:
18783
Concept ID:
C0034212
Disease or Syndrome
Any manifestation of a skin disease associated with the production of pus.
Cheilitis
MedGen UID:
3349
Concept ID:
C0007971
Disease or Syndrome
Inflammation of the lip.
Cutaneous photosensitivity
MedGen UID:
87601
Concept ID:
C0349506
Pathologic Function
An increased sensitivity of the skin to light. Photosensitivity may result in a rash upon exposure to the sun (which is known as photodermatosis). Photosensitivity can be diagnosed by phototests in which light is shone on small areas of skin.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVActinic prurigo

Professional guidelines

PubMed

Jiang AJ, Lim HW
Dermatol Clin 2020 Jan;38(1):71-77. Epub 2019 Oct 18 doi: 10.1016/j.det.2019.08.007. PMID: 31753194
Sharma D, Kwatra SG
J Am Acad Dermatol 2016 Feb;74(2):363-9. Epub 2015 Nov 11 doi: 10.1016/j.jaad.2015.09.039. PMID: 26577510
Hojyo-Tomoka MT, Vega-Memije ME, Cortes-Franco R, Domínguez-Soto L
Dermatol Ther 2003;16(1):40-4. doi: 10.1046/j.1529-8019.2003.01606.x. PMID: 12919125

Recent clinical studies

Etiology

Khalil S, Donthi D, Gru AA
J Cutan Pathol 2022 Oct;49(10):898-916. Epub 2022 Aug 4 doi: 10.1111/cup.14264. PMID: 35656820
Sharma D, Kwatra SG
J Am Acad Dermatol 2016 Feb;74(2):363-9. Epub 2015 Nov 11 doi: 10.1016/j.jaad.2015.09.039. PMID: 26577510
Sharma VK, Sahni K, Wadhwani AR
Photochem Photobiol Sci 2013 Jan;12(1):65-77. doi: 10.1039/c2pp25182e. PMID: 23123922
Gambichler T, Al-Muhammadi R, Boms S
Am J Clin Dermatol 2009;10(3):169-80. doi: 10.2165/00128071-200910030-00003. PMID: 19354331
Cornelison RL Jr
Dermatol Clin 2003 Oct;21(4):699-702. doi: 10.1016/s0733-8635(03)00076-7. PMID: 14717410

Diagnosis

Khalil S, Donthi D, Gru AA
J Cutan Pathol 2022 Oct;49(10):898-916. Epub 2022 Aug 4 doi: 10.1111/cup.14264. PMID: 35656820
Lee CN, Chen TY, Wong TW
Adv Exp Med Biol 2022;1367:369-381. doi: 10.1007/978-3-030-92616-8_14. PMID: 35286703
Rodríguez-Carreón AA, Rodríguez-Lobato E, Rodríguez-Gutiérrez G, Cuevas-González JC, Mancheno-Valencia A, Solís-Arias MP, Vega-Memije ME, Hojyo-Tomoka MT, Domínguez-Soto L
Skinmed 2015 Jul-Aug;13(4):287-95; quiz 296. Epub 2015 Aug 1 PMID: 26861426
Valbuena MC, Muvdi S, Lim HW
Dermatol Clin 2014 Jul;32(3):335-44, viii. doi: 10.1016/j.det.2014.03.010. PMID: 24891055
Scheen SR 3rd, Connolly SM, Dicken CH
J Am Acad Dermatol 1981 Aug;5(2):183-90. doi: 10.1016/s0190-9622(81)70086-0. PMID: 7263963

Therapy

Lee CN, Chen TY, Wong TW
Adv Exp Med Biol 2022;1367:369-381. doi: 10.1007/978-3-030-92616-8_14. PMID: 35286703
Sharma D, Kwatra SG
J Am Acad Dermatol 2016 Feb;74(2):363-9. Epub 2015 Nov 11 doi: 10.1016/j.jaad.2015.09.039. PMID: 26577510
Rodríguez-Carreón AA, Rodríguez-Lobato E, Rodríguez-Gutiérrez G, Cuevas-González JC, Mancheno-Valencia A, Solís-Arias MP, Vega-Memije ME, Hojyo-Tomoka MT, Domínguez-Soto L
Skinmed 2015 Jul-Aug;13(4):287-95; quiz 296. Epub 2015 Aug 1 PMID: 26861426
Valbuena MC, Muvdi S, Lim HW
Dermatol Clin 2014 Jul;32(3):335-44, viii. doi: 10.1016/j.det.2014.03.010. PMID: 24891055
Scheen SR 3rd, Connolly SM, Dicken CH
J Am Acad Dermatol 1981 Aug;5(2):183-90. doi: 10.1016/s0190-9622(81)70086-0. PMID: 7263963

Prognosis

Rodríguez-Carreón AA, Rodríguez-Lobato E, Rodríguez-Gutiérrez G, Cuevas-González JC, Mancheno-Valencia A, Solís-Arias MP, Vega-Memije ME, Hojyo-Tomoka MT, Domínguez-Soto L
Skinmed 2015 Jul-Aug;13(4):287-95; quiz 296. Epub 2015 Aug 1 PMID: 26861426
Fung MA, Barr KL
Dermatol Clin 2012 Oct;30(4):667-84, vii. Epub 2012 Jul 28 doi: 10.1016/j.det.2012.06.004. PMID: 23021053
Cornelison RL Jr
Dermatol Clin 2003 Oct;21(4):699-702. doi: 10.1016/s0733-8635(03)00076-7. PMID: 14717410
Lane PR, Hogan DJ, Martel MJ, Reeder B, Irvine J
J Am Acad Dermatol 1992 May;26(5 Pt 1):683-92. doi: 10.1016/0190-9622(92)70093-u. PMID: 1583166
Scheen SR 3rd, Connolly SM, Dicken CH
J Am Acad Dermatol 1981 Aug;5(2):183-90. doi: 10.1016/s0190-9622(81)70086-0. PMID: 7263963

Clinical prediction guides

Alamillo-Velazquez J, Ortiz-Morales G, Gonzalez-Gonzalez SE, Lopez-Altamirano DF, Rodríguez-Garcia A
Int Ophthalmol 2024 Feb 5;44(1):4. doi: 10.1007/s10792-024-02925-0. PMID: 38315255
Khalil S, Donthi D, Gru AA
J Cutan Pathol 2022 Oct;49(10):898-916. Epub 2022 Aug 4 doi: 10.1111/cup.14264. PMID: 35656820
Yang CS, Kim C, Antaya RJ
J Am Acad Dermatol 2015 Apr;72(4):703-11. Epub 2015 Jan 21 doi: 10.1016/j.jaad.2015.01.002. PMID: 25617013
Grover JK, Vats V, Gopalakrishna R, Ramam M
Natl Med J India 2000 May-Jun;13(3):132-41. PMID: 11558112
Scheen SR 3rd, Connolly SM, Dicken CH
J Am Acad Dermatol 1981 Aug;5(2):183-90. doi: 10.1016/s0190-9622(81)70086-0. PMID: 7263963

Recent systematic reviews

Burfield L, Rutter KJ, Thompson B, Marjanovic EJ, Neale RE, Rhodes LE
J Eur Acad Dermatol Venereol 2023 Mar;37(3):511-520. Epub 2022 Dec 3 doi: 10.1111/jdv.18772. PMID: 36433668

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