Variable erythrokeratoderma. An unusual case

Schellander, F

doi:10.1001/archderm.100.6.744

Cited by 9 publications

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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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Section: Discussionmentioning

confidence: 99%

Erythrokeratoderma en cocardes

Rajagopalan

Pulimood

George

et al. 1999

Clinical and Experimental Dermatology

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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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“…The patient now has many features of erythrokeratoderma variabilis although the onset of signs at birth is more in keeping with non-bullous ichthyosiform erythroderma. A very similar patient has been described as an unusual case of erythrokeratoderma variabilis (Schellander & Fritsch, 1969). The question of terminology may be important in assigning an accurate genetic prognosis.…”

Section: Case Reportsmentioning

confidence: 95%

Case Reports

2008

British Journal of Dermatology

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Variable erythrokeratoderma. An unusual case

Cited by 9 publications

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Erythrokeratoderma en cocardes

Erythrokeratoderma en cocardes

A case of erythrokeratoderma variabilis without mutations in connexin 31

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