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Lichenification

MedGen UID:
452108
Concept ID:
C0023653
Pathologic Function
SNOMED CT: Lichenification (19940005)
 
HPO: HP:0100725

Definition

Thickening and hardening of the epidermis seen with exaggeration of normal skin lines. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLichenification

Conditions with this feature

Hereditary insensitivity to pain with anhidrosis
MedGen UID:
6915
Concept ID:
C0020074
Disease or Syndrome
NTRK1 congenital insensitivity to pain with anhidrosis (NTRK1-CIPA) is characterized by insensitivity to pain, anhidrosis (the inability to sweat), and intellectual disability. The ability to sense all pain (including visceral pain) is absent, resulting in repeated injuries including: oral self-mutilation (biting of tongue, lips, and buccal mucosa); biting of fingertips; bruising, scarring, and infection of the skin; multiple bone fractures (many of which fail to heal properly); and recurrent joint dislocations resulting in joint deformity. Sense of touch, vibration, and position are normal. Anhidrosis predisposes to recurrent febrile episodes that are often the initial manifestation of NTRK1-CIPA. Hypothermia in cold environments also occurs. Intellectual disability of varying degree is observed in most affected individuals; hyperactivity and emotional lability are common.
Peeling skin syndrome 4
MedGen UID:
895692
Concept ID:
C4225407
Disease or Syndrome
Any peeling skin syndrome in which the cause of the disease is a mutation in the CSTA gene.
Amyloidosis, primary localized cutaneous, 1
MedGen UID:
1639046
Concept ID:
C4551501
Disease or Syndrome
In some affected individuals, the patches have characteristics of both lichen and macular amyloidosis. These cases are called biphasic amyloidosis.\n\nIn all forms of PLCA, the abnormal patches of skin usually arise in mid-adulthood. They can remain for months to years and may recur after disappearing, either at the same location or elsewhere. Very rarely, nodular amyloidosis progresses to a life-threatening condition called systemic amyloidosis, in which amyloid deposits accumulate in tissues and organs throughout the body.\n\nNodular amyloidosis is characterized by firm, raised bumps (nodules) that are pink, red, or brown. These nodules often occur on the face, torso, limbs, or genitals and are typically not itchy.\n\nIn macular amyloidosis, the patches are flat and dark brown. The coloring can have a lacy (reticulated) or rippled appearance, although it is often uniform. Macular amyloidosis patches are most commonly found on the upper back, but they can also occur on other parts of the torso or on the limbs. These patches are mildly itchy.\n\nLichen amyloidosis is characterized by severely itchy patches of thickened skin with multiple small bumps. The patches are scaly and reddish brown in color. These patches usually occur on the shins but can also occur on the forearms, other parts of the legs, and elsewhere on the body.\n\nPrimary localized cutaneous amyloidosis (PLCA) is a condition in which clumps of abnormal proteins called amyloids build up in the skin, specifically in the wave-like projections (dermal papillae) between the top two layers of skin (the dermis and the epidermis). The primary feature of PLCA is patches of skin with abnormal texture or color. The appearance of these patches defines three forms of the condition: lichen amyloidosis, macular amyloidosis, and nodular amyloidosis.

Professional guidelines

PubMed

Mandlik DS, Mandlik SK
Immunopharmacol Immunotoxicol 2021 Apr;43(2):105-125. Epub 2021 Feb 28 doi: 10.1080/08923973.2021.1889583. PMID: 33645388
Giavina-Bianchi M, Giavina-Bianchi P
Arch Immunol Ther Exp (Warsz) 2019 Apr;67(2):69-78. Epub 2018 Aug 22 doi: 10.1007/s00005-018-0521-y. PMID: 30159581
Chang C, Keen CL, Gershwin ME
Clin Rev Allergy Immunol 2007 Dec;33(3):204-25. doi: 10.1007/s12016-007-0033-8. PMID: 18163227

Recent clinical studies

Therapy

Mandlik DS, Mandlik SK
Immunopharmacol Immunotoxicol 2021 Apr;43(2):105-125. Epub 2021 Feb 28 doi: 10.1080/08923973.2021.1889583. PMID: 33645388
Giavina-Bianchi M, Giavina-Bianchi P
Arch Immunol Ther Exp (Warsz) 2019 Apr;67(2):69-78. Epub 2018 Aug 22 doi: 10.1007/s00005-018-0521-y. PMID: 30159581
Yale K, Awosika O, Rengifo-Pardo M, Ehrlich A
Dermatitis 2018 May/Jun;29(3):112-119. doi: 10.1097/DER.0000000000000371. PMID: 29698355
Yost JM, Boyd KP, Patel RR, Ramachandran S, Franks AG Jr
Dermatol Online J 2013 Dec 16;19(12):20709. PMID: 24365000
Chang C, Keen CL, Gershwin ME
Clin Rev Allergy Immunol 2007 Dec;33(3):204-25. doi: 10.1007/s12016-007-0033-8. PMID: 18163227

Prognosis

Choo ZY, Mehlis SL, Joyce JC
Dis Mon 2024 Apr;70(4):101687. Epub 2024 Jan 25 doi: 10.1016/j.disamonth.2024.101687. PMID: 38278753
Yew YW, Thyssen JP, Silverberg JI
J Am Acad Dermatol 2019 Feb;80(2):390-401. Epub 2018 Oct 1 doi: 10.1016/j.jaad.2018.09.035. PMID: 30287309
Silvestre Salvador JF, Romero-Pérez D, Encabo-Durán B
J Investig Allergol Clin Immunol 2017;27(2):78-88. Epub 2017 Jan 10 doi: 10.18176/jiaci.0138. PMID: 28071589
Lopes Filho LL, Lopes IM, Lopes LR, Enokihara MM, Michalany AO, Matsunaga N
An Bras Dermatol 2015 Mar-Apr;90(2):225-31. doi: 10.1590/abd1806-4841.20153189. PMID: 25830993Free PMC Article
Ooi ET, Tidman MJ
Practitioner 2014 Feb;258(1768):23-6, 3. PMID: 24689165

Clinical prediction guides

Wollenberg A, Simon D, Kulthanan K, Figueras-Nart I, Misery L, Tangsirisap N, Spina L, Lu N, Grond S, Eyerich K
J Eur Acad Dermatol Venereol 2024 Apr;38(4):695-702. Epub 2023 Dec 2 doi: 10.1111/jdv.19669. PMID: 38041556
Bang CH, Yoon JW, Ryu JY, Chun JH, Han JH, Lee YB, Lee JY, Park YM, Lee SJ, Lee JH
Sci Rep 2021 Mar 15;11(1):6049. doi: 10.1038/s41598-021-85489-8. PMID: 33723375Free PMC Article
Hon KL, Kung JSC, Ng WG, Tsang KYC, Cheng N, Leung TF
J Dermatolog Treat 2021 Feb;32(1):45-48. Epub 2020 Oct 7 doi: 10.1080/09546634.2018.1442551. PMID: 29460656
Silvestre Salvador JF, Romero-Pérez D, Encabo-Durán B
J Investig Allergol Clin Immunol 2017;27(2):78-88. Epub 2017 Jan 10 doi: 10.18176/jiaci.0138. PMID: 28071589
Dermatology 1993;186(1):23-31. doi: 10.1159/000247298. PMID: 8435513

Recent systematic reviews

Yew YW, Thyssen JP, Silverberg JI
J Am Acad Dermatol 2019 Feb;80(2):390-401. Epub 2018 Oct 1 doi: 10.1016/j.jaad.2018.09.035. PMID: 30287309
Chalmers JR, Schmitt J, Apfelbacher C, Dohil M, Eichenfield LF, Simpson EL, Singh J, Spuls P, Thomas KS, Admani S, Aoki V, Ardeleanu M, Barbarot S, Berger T, Bergman JN, Block J, Borok N, Burton T, Chamlin SL, Deckert S, DeKlotz CC, Graff LB, Hanifin JM, Hebert AA, Humphreys R, Katoh N, Kisa RM, Margolis DJ, Merhand S, Minnillo R, Mizutani H, Nankervis H, Ohya Y, Rodgers P, Schram ME, Stalder JF, Svensson A, Takaoka R, Teper A, Tom WL, von Kobyletzki L, Weisshaar E, Zelt S, Williams HC
Br J Dermatol 2014 Dec;171(6):1318-25. Epub 2014 Nov 14 doi: 10.1111/bjd.13237. PMID: 24980543Free PMC Article

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