Variable Erythrokeratoderma

Schellander, F

doi:10.1001/archderm.1969.01610300094017

Cited by 8 publications

(4 citation statements)

References 6 publications

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“…A shift of intermediate filament configuration to that of the basal layer cells might account for closer adhesion of stratum corneum cells in EKV lesional epidermis, resulting in a retention type of hyperkeratosis. This would agree with the findings of Schellander & Fritsch, who found normal proliferation rate in EKV epidermis by labelling with tritiated thymidine (14). Immunoelectron microscopy might help to further define this disorder of keratinization.…”

Section: Discussionsupporting

confidence: 92%

Erythrokeratodermia variabilis: immunohistochemical and ultrastructural studies of the epidermis

McFadden,

Oppedal BR,

Ree

et al. 1987

Acta Derm Venereol

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Immunohistochemical and ultrastructural study of the epidermis was performed in a 53-year-old female with erythrokeratodermia variabilis (EKV) before and after treatment with the aromatic retinoid Etretinate (RO 10-9359). Aberrant expression of cytokeratin PKK2 (Labsystems, Helsinki, Finland) in lesional EKV stratum corneum was observed; this feature disappeared after Etretinate therapy. A normal distribution of DR-positive dendritic Langerhans´ cells was seen in diseased, control and post-treatment skin specimens. The striking finding of this study was thus the shift to a basal cell-type keratin reactivity in stratum corneum in lesional skin, perhaps a reflection of cytoskeleton features related to cell adhesion. Increased adhesion between the cells in stratum corneum might account for the retention type of hyperkeratosis characteristic of EKV.

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Section: Discussionsupporting

confidence: 92%

Erythrokeratodermia variabilis: immunohistochemical and ultrastructural studies of the epidermis

McFadden,

Oppedal BR,

Ree

et al. 1987

Acta Derm Venereol

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“…A clinical diagnosis of erythrokeratoderma en cocardes 1 , 2 was made, based on: the presence of polycyclic plaques with concentric erythema, the scaling that showed an ‘en cocarde’ appearance and erythematous scaly plaques suggestive of erythrokertoderma variabilis 3 ,. 4 , 6 In view of the concentric appearance of some of the lesions, a differential diagnosis of tinea imbricata was considered, but this was ruled out by a negative potassium hydroxide preparation.…”

Section: Discussionmentioning

confidence: 99%

Erythrokeratoderma en cocardes

Rajagopalan

Pulimood

George

et al. 1999

Clinical and Experimental Dermatology

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The erythrokeratodermas are a distinct but clinically variable group of rare geno-dermatoses, characterized by circumscribed erythematous and hyperkeratotic lesions. All attempts to establish a valid classification have been based on purely clinical and morphologic criteria. Erythrokeratoderma en cocardes, also known as genodermatose en cocardes or Degos' syndrome, was first described by Degos in 1947. The condition is characterized by large round plaques with concentric erythema and scaling having a target configuration, which remit and recur, in addition to scaly plaques as seen in erythrokeratoderma variabilis. A case of this rare genodermatosis is described.

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“…Because of the presence of various subtypes, 1,3 , 19,20 the classification of EK has been a matter of debate. The classical EK variabilis described by Mendes da Costa is characterized by two types of skin lesions: figurate hyperkeratotic plaques and transient erythematous areas 4,21 .…”

Section: Discussionmentioning

confidence: 99%

A case of erythrokeratoderma variabilis without mutations in connexin 31

Ishida‐Yamamoto

Kelsell

Common

et al. 2000

British Journal of Dermatology

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Erythrokeratoderma (EK) variabilis is a heterogeneous group of diseases characterized by migratory erythematous patches and hyperkeratotic plaques. Mutations in connexin 31 have recently been found to underlie several cases of EK variabilis. We describe a Japanese girl with extensive lesions that appeared to be a form of EK variabilis, clinically resembling genodermatose en cocardes (Degos). Our patient had characteristic migratory rosette or target‐like erythematous keratotic plaques with peripheral scaling in addition to relatively fixed keratotic plaques. Sequencing of the connexin 31 gene did not detect mutations. Skin biopsy showed parakeratotic hyperkeratosis with hypergranulosis. Immunohistochemically, suprabasal keratins, involucrin and profilaggrin were unequivocally expressed, while loricrin expression was greatly diminished and deiminated K1 was undetectable. Our results confirm aetiological heterogeneity in EK. The histological features suggest disruption of keratinocyte terminal differentiation at a very late stage.

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Variable Erythrokeratoderma

Cited by 8 publications

References 6 publications

Erythrokeratodermia variabilis: immunohistochemical and ultrastructural studies of the epidermis

Erythrokeratodermia variabilis: immunohistochemical and ultrastructural studies of the epidermis

Erythrokeratoderma en cocardes

A case of erythrokeratoderma variabilis without mutations in connexin 31

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