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Skin plaque

MedGen UID:
69134
Concept ID:
C0241148
Finding
Synonym: Cutaneous plaque
SNOMED CT: Cutaneous plaque (128177006); Cutaneous patch (128177006); Skin plaque (276322001)
 
HPO: HP:0200035

Definition

A plaque is a solid, raised, plateau-like (flat-topped) lesion greater than 1 cm in diameter. [from HPO]

Conditions with this feature

Lipid proteinosis
MedGen UID:
6112
Concept ID:
C0023795
Disease or Syndrome
Lipoid proteinosis (LP) is characterized by deposition of hyaline-like material in various tissues resulting in a hoarse voice from early infancy, vesicles and hemorrhagic crusts in the mouth and on the face and extremities, verrucous and keratotic cutaneous lesions on extensor surfaces (especially the elbows), and moniliform blepharosis (multiple beaded papules along the eyelid margins and inner canthus). Extracutaneous manifestations may include epilepsy, neuropsychiatric disorders, spontaneous CNS hemorrhage, and asymptomatic multiple yellowish nodules throughout the gastrointestinal tract. Generally, the disease course is chronic and fluctuating. Males and females are affected equally. Affected individuals have a normal life span unless they experience laryngeal obstruction.
Mycosis fungoides
MedGen UID:
7771
Concept ID:
C0026948
Neoplastic Process
Mycosis fungoides is a malignant T-cell lymphoma of the skin, first reported (and named) by Alibert (1835). Sezary syndrome is a leukemic variant of mycosis fungoides defined by erythroderma with greater than 80% of the skin showing redness, adenopathy and greater than 1,000 circulating Sezary cells/microliter with a CD4+CD26- or CD4+CD7- phenotype. Sezary cells have a type 2 helper T cell cytokine profile. Sezary syndrome has a median overall survival time of only 2.4 years in patients with Sezary cells at a density of greater than 10,000 cells/microliter or 5.4 years in patients with 1,000-10,000 Sezary cells/microliter. Mycosis fungoides and Sezary syndrome are the most common cutaneous T-cell lymphomas. Sezary syndrome can arise de novo or can appear following years of chronic mycosis fungoides. Both are thought to arise from clonal expansion of CD4+ helper T cells responding to chronic antigen stimulation (summary by Wang et al., 2015).
Hereditary papulotranslucent acrokeratoderma
MedGen UID:
350144
Concept ID:
C1863343
Disease or Syndrome
A keratosis of the hands and feet characterized by persistent, asymptomatic, yellowish to white papules and plaques associated with fine-textured scalp hair and an atopic diathesis.
Erythrokeratodermia variabilis et progressiva 2
MedGen UID:
1379712
Concept ID:
C4479618
Disease or Syndrome
Erythrokeratodermia variabilis et progressiva-2 (EKVP2) is a genodermatosis characterized by persistent plaque-like or generalized hyperkeratosis and transient red patches of variable size, shape, and location. The severity and dominating features of the disease vary strikingly within families and also during an individual's course of disease. The erythematous component usually prevails in young children, whereas hyperkeratosis is the dominant or sole feature in adults. Some patients with EKVP2 display lesions resembling erythema gyratum repens (summary by Richard et al., 2003). EKVP was previously thought to be separate disorders: erythrokeratodermia variabilis (EKV) and progressive symmetric erythrokeratodermia (PSEK) (van Steensel et al., 2009). For a discussion of genetic heterogeneity of EKVP, see EKVP1 (133200).
Proteasome-associated autoinflammatory syndrome 1
MedGen UID:
1648310
Concept ID:
C4746851
Disease or Syndrome
This autosomal recessive systemic autoinflammatory disorder is characterized by early childhood onset of annular erythematous plaques on the face and extremities with subsequent development of partial lipodystrophy and laboratory evidence of immune dysregulation. More variable features include recurrent fever, severe joint contractures, muscle weakness and atrophy, hepatosplenomegaly, basal ganglia calcifications, and microcytic anemia (summary by Agarwal et al., 2010; Kitamura et al., 2011; Arima et al., 2011). This disorder encompasses Nakajo-Nishimura syndrome (NKJO); joint contractures, muscular atrophy, microcytic anemia, and panniculitis-induced lipodystrophy (JMP syndrome); and chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome (CANDLE). Among Japanese patients, this disorder is best described as Nakajo-Nishimura syndrome, since both Nakajo (1939) and Nishimura et al. (1950) contributed to the original phenotypic descriptions. Genetic Heterogeneity of Proteasome-Associated Autoinflammatory Syndrome See also PRAAS2 (618048), caused by mutation in the POMP gene (613386) on chromosome 13q12; PRAAS3 (617591), caused by mutation in the PSMB4 gene (602177) on chromosome 1q21; PRAAS4 (619183), caused by mutation in the PSMG2 gene (609702) on chromosome 18p11; and PRAAS5 (619175), caused by mutation in the PSMB10 gene (176847) on chromosome 16q22.
VEXAS syndrome
MedGen UID:
1765785
Concept ID:
C5435753
Disease or Syndrome
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome) is an adult-onset inflammatory disease that primarily affects males and is caused by somatic, not germline, mutations. The disorder is characterized by adult onset of rheumatologic symptoms at a mean age of 64 years. Features include recurrent fevers, pulmonary and dermatologic inflammatory manifestations, vasculitis, deep vein thrombosis, arthralgias, and ear and nose chondritis. Laboratory studies indicate hematologic abnormalities, including macrocytic anemia, as well as increased levels of acute-phase reactants; about half of patients have positive autoantibodies. Bone marrow biopsy shows degenerative vacuolization restricted to myeloid and erythroid precursor cells, as well as variable hematopoietic dyspoiesis and dysplasias. The condition does not respond to rheumatologic medications and the features may result in premature death (summary by Beck et al., 2020).

Professional guidelines

PubMed

Reich K, Warren RB, Coates LC, Di Comite G
J Eur Acad Dermatol Venereol 2020 Jun;34(6):1161-1173. Epub 2020 Jan 7 doi: 10.1111/jdv.16124. PMID: 31785165
Chong HT, Kopecki Z, Cowin AJ
Biomed Res Int 2013;2013:168321. Epub 2013 Aug 25 doi: 10.1155/2013/168321. PMID: 24062996Free PMC Article
Hern S, Stanton AW, Mellor RH, Harland CC, Levick JR, Mortimer PS
Br J Dermatol 2005 Mar;152(3):505-11. doi: 10.1111/j.1365-2133.2005.06435.x. PMID: 15787819

Recent clinical studies

Etiology

Rosenblatt NJ, Stachowiak A, Reddin C
Adv Wound Care (New Rochelle) 2021 Sep;10(9):529-533. Epub 2019 Dec 4 doi: 10.1089/wound.2019.1086. PMID: 34232743Free PMC Article
Lin CC, Lin HC, Chiu HW
Am J Clin Dermatol 2019 Jun;20(3):457-463. doi: 10.1007/s40257-018-00420-8. PMID: 30649680
Gisondi P, Di Mercurio M, Idolazzi L, Girolomoni G
J Rheumatol Suppl 2015 Nov;93:57-60. doi: 10.3899/jrheum.150638. PMID: 26523059
Harrington L, Sokol L, Holdener S, Shao H, Zhang L
J Cutan Pathol 2014 Dec;41(12):936-43. Epub 2014 Nov 11 doi: 10.1111/cup.12395. PMID: 25292289
Govindarajan V, Agarwal V, Aggarwal A, Misra R
J Assoc Physicians India 2001 Dec;49:1145-7. PMID: 11996432

Diagnosis

Lin CC, Lin HC, Chiu HW
Am J Clin Dermatol 2019 Jun;20(3):457-463. doi: 10.1007/s40257-018-00420-8. PMID: 30649680
Castellanos E, Plana A, Carrato C, Carrió M, Rosas I, Amilibia E, Roca-Ribas F, Hostalot C, Castillo A, Ros A, Quer A, Becerra JL, Salvador H, Lázaro C, Blanco I, Serra E, Bielsa I; for CSUR Phakomatoses Multidisciplinary Clinics HUGTiP-ICO-IGTP
JAMA Dermatol 2018 Mar 1;154(3):341-346. doi: 10.1001/jamadermatol.2017.5464. PMID: 29322178Free PMC Article
Disharoon M, Kozlowski KF, Kaniowski JM
Radiology 2017 Jun;283(3):909-916. doi: 10.1148/radiol.2017150456. PMID: 28514220
Gisondi P, Di Mercurio M, Idolazzi L, Girolomoni G
J Rheumatol Suppl 2015 Nov;93:57-60. doi: 10.3899/jrheum.150638. PMID: 26523059
Govindarajan V, Agarwal V, Aggarwal A, Misra R
J Assoc Physicians India 2001 Dec;49:1145-7. PMID: 11996432

Therapy

Reich K, Warren RB, Coates LC, Di Comite G
J Eur Acad Dermatol Venereol 2020 Jun;34(6):1161-1173. Epub 2020 Jan 7 doi: 10.1111/jdv.16124. PMID: 31785165
Lin CC, Lin HC, Chiu HW
Am J Clin Dermatol 2019 Jun;20(3):457-463. doi: 10.1007/s40257-018-00420-8. PMID: 30649680
Gisondi P, Di Mercurio M, Idolazzi L, Girolomoni G
J Rheumatol Suppl 2015 Nov;93:57-60. doi: 10.3899/jrheum.150638. PMID: 26523059
Zhang Y, Meng XM, Huang XR, Wang XJ, Yang L, Lan HY
Clin Exp Pharmacol Physiol 2014 Nov;41(11):921-32. doi: 10.1111/1440-1681.12294. PMID: 25132073

Prognosis

Santonja C, Carrasco L, Pérez-Sáenz MLÁ, Rodríguez-Pinilla SM
Am J Dermatopathol 2020 May;42(5):364-367. doi: 10.1097/DAD.0000000000001569. PMID: 31725483
Gisondi P, Di Mercurio M, Idolazzi L, Girolomoni G
J Rheumatol Suppl 2015 Nov;93:57-60. doi: 10.3899/jrheum.150638. PMID: 26523059

Clinical prediction guides

Lin CC, Lin HC, Chiu HW
Am J Clin Dermatol 2019 Jun;20(3):457-463. doi: 10.1007/s40257-018-00420-8. PMID: 30649680
Gisondi P, Di Mercurio M, Idolazzi L, Girolomoni G
J Rheumatol Suppl 2015 Nov;93:57-60. doi: 10.3899/jrheum.150638. PMID: 26523059
Zhang Y, Meng XM, Huang XR, Wang XJ, Yang L, Lan HY
Clin Exp Pharmacol Physiol 2014 Nov;41(11):921-32. doi: 10.1111/1440-1681.12294. PMID: 25132073
Peternel S, Manestar-Blažić T, Brajac I, Prpić-Massari L, Kaštelan M
J Cutan Pathol 2011 Oct;38(10):780-9. Epub 2011 Jul 29 doi: 10.1111/j.1600-0560.2011.01762.x. PMID: 21797919
Govindarajan V, Agarwal V, Aggarwal A, Misra R
J Assoc Physicians India 2001 Dec;49:1145-7. PMID: 11996432

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