The Experts below are selected from a list of 78 Experts worldwide ranked by ideXlab platform

Marcel F Jonkman - One of the best experts on this subject based on the ideXlab platform.

  • keratolysis exfoliativa Dyshidrosis lamellosa sicca a distinct peeling entity
    British Journal of Dermatology, 2012
    Co-Authors: Y Y Chang, J Van Der Velden, G Van Der Wier, Duco Kramer, Gilles F H Diercks, M Van Geel, P J Coenraads, Patrick L J M Zeeuwen, Marcel F Jonkman
    Abstract:

    Background Keratolysis exfoliativa (KE), also known as Dyshidrosis lamellosa sicca, is a palmoplantar dermatosis characterized by air-filled blisters and collarette desquamation. It has been regarded as a subtype of dyshidrotic eczema, a fungal infection or a dermatophytid reaction. KE may also resemble acral peeling skin syndrome and localized epidermolysis bullosa simplex. Although KE is a common disorder, it is a rarely reported and is an under-recognized dermatosis. Objectives To delineate the characteristic features of KE. Methods We investigated the clinical, immunohistopathological, ultrastructural and molecular features of KE. Patients were included from the clinical records. Additional diagnostic research consisted of mutation analysis of the candidate genes TGM5, KRT5, KRT14, FLG, SPINK6 and SPINK9. Results A total of 24 patients with KE were identified, six with familial and 18 with sporadic KE. Lesions consisted of air-filled blisters only on palmoplantar skin, followed by collarette and lamellar peeling. Both light microscopy and electron microscopy showed cleavage and partially degraded corneodesmosomes within the stratum corneum, whereas immunofluorescence microscopy showed normal expression of corneodesmosomal components. No mutations were found in TGM5, KRT5/14 and SPINK6/9. There was no clear link with atopy or with FLG mutations. Conclusions Our study suggests premature corneodesmolysis as the main pathological mechanism of this palmoplantar skin disorder. We conclude that KE appears to be a distinct peeling entity.

Y Y Chang - One of the best experts on this subject based on the ideXlab platform.

  • keratolysis exfoliativa Dyshidrosis lamellosa sicca a distinct peeling entity
    British Journal of Dermatology, 2012
    Co-Authors: Y Y Chang, J Van Der Velden, G Van Der Wier, Duco Kramer, Gilles F H Diercks, M Van Geel, P J Coenraads, Patrick L J M Zeeuwen, Marcel F Jonkman
    Abstract:

    Background Keratolysis exfoliativa (KE), also known as Dyshidrosis lamellosa sicca, is a palmoplantar dermatosis characterized by air-filled blisters and collarette desquamation. It has been regarded as a subtype of dyshidrotic eczema, a fungal infection or a dermatophytid reaction. KE may also resemble acral peeling skin syndrome and localized epidermolysis bullosa simplex. Although KE is a common disorder, it is a rarely reported and is an under-recognized dermatosis. Objectives To delineate the characteristic features of KE. Methods We investigated the clinical, immunohistopathological, ultrastructural and molecular features of KE. Patients were included from the clinical records. Additional diagnostic research consisted of mutation analysis of the candidate genes TGM5, KRT5, KRT14, FLG, SPINK6 and SPINK9. Results A total of 24 patients with KE were identified, six with familial and 18 with sporadic KE. Lesions consisted of air-filled blisters only on palmoplantar skin, followed by collarette and lamellar peeling. Both light microscopy and electron microscopy showed cleavage and partially degraded corneodesmosomes within the stratum corneum, whereas immunofluorescence microscopy showed normal expression of corneodesmosomal components. No mutations were found in TGM5, KRT5/14 and SPINK6/9. There was no clear link with atopy or with FLG mutations. Conclusions Our study suggests premature corneodesmolysis as the main pathological mechanism of this palmoplantar skin disorder. We conclude that KE appears to be a distinct peeling entity.

Texia Researcheuturin - One of the best experts on this subject based on the ideXlab platform.

Lorenzo Martini - One of the best experts on this subject based on the ideXlab platform.

Nurcan Uceyler - One of the best experts on this subject based on the ideXlab platform.

  • Dyshidrosis is associated with reduced amplitudes in electrically evoked pain-related potentials in women with Fabry disease.
    Clinical Neurophysiology, 2019
    Co-Authors: Gabriela Siedler, Ann-kathrin Kahn, Frank Weidemann, Claudia Sommer, Christoph Wanner, Nurcan Uceyler
    Abstract:

    Abstract Objective To investigate A-delta fiber conduction in mild to moderate Fabry disease (FD) patients using pain-related evoked potentials (PREP). Methods In this case-control study we prospectively investigated 58 patients with mild to moderate FD and compared data with those of healthy controls. Small fiber function (quantitative sensory testing, QST and sympathetic skin response, SSR), morphology (intraepidermal nerve fiber density, IENFD), and electrical conduction (PREP) were assessed and correlated with sweating as major autonomic function disturbed in FD. Patients were further stratified for gender, disease severity as reflected by renal and cardiac function, and genetics. Results An- or hypohidrosis (i.e. Dyshidrosis) was reported by 7/32 (22%) women and 15/26 (58%) men with FD (p  Conclusion A-delta fiber conduction investigated using PREP is impaired in mild to moderately affected female FD patients with clinical signs of hypohidrosis. Significance Small fiber assessment in FD is of diagnostic value already in mild to moderate stages of disease.