A Case of Lichen Sclerosus of the Scalp Associated With Autoantibodies to Extracellular Matrix Protein 1

Kawakami, Yoshio; Oyama, Noritaka; Hanami, Yuka; Kimura, Tetsunori; Kishimoto, Kazuto; Yamamoto, Toshiyuki

doi:10.1001/archdermatol.2009.313

Cited by 3 publications

(3 citation statements)

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“…For an immunological background, male LS patients have a relatively lower prevalence of associated autoimmune and autoantibody disorders in comparison with female LS patients [66,67]. Thereafter, several case reports have revealed the presence of serum anti-ECM1 antibodies in LS with different clinical features, particularly extragenital lesions [68,69]. These evidence series provide further weight for a humoral autoimmunity to ECM1, without regard to genders and disease phenotype, being a pathogenic axis in LS.…”

Section: Matrix Protein 1 (Ecm1)mentioning

confidence: 94%

The Extracellular Matrix Protein 1 (ECM1) in Skin Biology: An Update for the Pleiotropic Action

Oyama¹,

Merregaert²

2013

TODJ

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Extracellular matrix protein 1 (ECM1) is a secreted glycoprotein that plays a pivotal role in the structural and homeostatic biology of the skin, particularly in angiogenesis, reconstitution of basement membrane, proliferation and differentiation of epidermal keratinocytes and dermal fibroblasts, and malignant transformation. This rationale is substantiated by loss-of-function mutations in the ECM1 gene in an autosomal recessive genodermatosis lipoid proteinosis and circulating IgG autoantibodies to this molecule in a humoral autoimmune condition lichen sclerosus, both of which are counterpart disease conditions sharing comparable skin pathology. In the recent decade, considerable progress has been made in determining the in vivo function of ECM1 in animal model studies. Furthermore, underlying insights arose for the genetic predisposition in inflammatory bowel disease ulcerative colitis, acquisition of immune tolerance and allergic responses via particular T cell subsets such as CD4+CD25+ regulatory T cells and Th2 cells, regeneration of certain organs, and clinical advantages for diagnostic and prognostic significance in various cancers. Following our latest review in 2009, we now update the most recent evidences for the pleiotropic action of ECM1 in skin research, and also highlight the novel pathogenic relevance of this molecule in a variety of human disorders.

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Section: Matrix Protein 1 (Ecm1)mentioning

confidence: 94%

The Extracellular Matrix Protein 1 (ECM1) in Skin Biology: An Update for the Pleiotropic Action

Oyama¹,

Merregaert²

2013

TODJ

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“…An itching sensation, among various symptoms, is primary and inevitable [ 38 ]. The lesion may account for the bullous and hemorrhagic appearance, but it causes a potential difficulty in arriving at an accurate diagnosis [ 39 , 40 ]. Several reports for extragenital LS cases have suggested a variety of affected skin sites including the head, neck, scalp, palms and soles, periorbital area, tongue, lip, and peristoma skin [ 41 ] ( Figure 2 ).…”

Section: Clinical Characteristics Of Lsmentioning

confidence: 99%

“…In another cohort study, ECM1 seroreactivity was also detectable in male patients with male penile LS [ 2 ]. The update series has further accelerated the serological diagnostic accuracy in individual cases suspicious of LS [ 39 , 75 ]. Irrespective of gender, humoral autoimmunity to ECM1 needs to be considered on the basis of its pathogenic significance in LS.…”

Section: Autoimmune Response In Lsmentioning

confidence: 99%

Lichen Sclerosus: A Current Landscape of Autoimmune and Genetic Interplay

Oyama

Hasegawa

2022

Diagnostics

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Lichen sclerosus (LS) is an acquired chronic inflammatory dermatosis predominantly affecting the anogenital area with recalcitrant itching and soreness. Progressive or persistent LS may cause urinary and sexual disturbances and an increased risk of local skin malignancy with a prevalence of up to 11%. Investigations on lipoid proteinosis, an autosomal recessive genodermatosis caused by loss-of-function mutations in the extracellular matrix protein 1 (ECM1) gene, led to the discovery of a humoral autoimmune response to the identical molecule in LS, providing evidence for an autoimmune and genetic counterpart targeting ECM1. This paper provides an overview of the fundamental importance and current issue of better understanding the immunopathology attributed to ECM1 in LS. Furthermore, we highlight the pleiotropic action of ECM1 in homeostatic and structural maintenance of skin biology as well as in a variety of human disorders possibly associated with impaired or gained ECM1 function, including the inflammatory bowel disease ulcerative colitis, Th2 cell-dependent airway allergies, T-cell and B-cell activation, and the demyelinating central nervous system disease multiple sclerosis, to facilitate sharing the concept as a plausible therapeutic target of this attractive molecule.

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EuroGuiderm guideline on lichen sclerosus—introduction into lichen sclerosus

Kirtschig,

Kinberger,

Kreuter

et al. 2024

Acad Dermatol Venereol

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IntroductionLichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness. It may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are redness and oedema, typically followed by whitening of the genital skin; sometimes fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early.ObjectivesThe guideline intends to provide guidance on the diagnostic of LS, highlight important aspects in the care of LS patients (part 1), generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow‐up of patients with LS and inform about new developments and potential research aspects.Materials and MethodsThe guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF‐EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives.ResultsUltrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co‐treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases.ConclusionsLS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long‐term.

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A Case of Lichen Sclerosus of the Scalp Associated With Autoantibodies to Extracellular Matrix Protein 1

Cited by 3 publications

References 5 publications

The Extracellular Matrix Protein 1 (ECM1) in Skin Biology: An Update for the Pleiotropic Action

The Extracellular Matrix Protein 1 (ECM1) in Skin Biology: An Update for the Pleiotropic Action

Lichen Sclerosus: A Current Landscape of Autoimmune and Genetic Interplay

EuroGuiderm guideline on lichen sclerosus—introduction into lichen sclerosus

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