Inflammatory Familial Palmoplantar Keratoderma: Greither’s Disease?

Abstract

A case of a familial palmoplantar inflammatory keratoderma with autosomal dominant inheritance is reported. Associated clinical features included vasomotor troubles and hyperhidrosis consistent with a diagnosis of Greither’s disease. Light microscopy was nonspecific. Electron microscopy showed aggregated tonofilaments around the nucleus, without true clumps. Desmosomes were numerous and cell-cell junctions showed an imbricated pattern, well demonstrated in the stratum corneum. The diagnosis of Greither’s keratoderma is discussed.

Greither's disease: a case report

“…12 We think that the natural history and the unique clinical findings justify retaining the entity as Griether's disease. An electron microscopic study by Beylot-Barry et al 13 showed aggregated monofilaments around the nucleus, without true clump formation. Desmosomes were numerous and cell-cell junctions showed an imbricated pattern.…”

Keratosis extremitatum (Greither’s Disease): Clinical Features, Histology, infrastructure

“…Beylot-Barry et al [6] have documented aggregated tonofilaments around the nucleus and numerous desmosomes. In our case immunohistochemical investigations showed pro nounced staining with Ki-67 that documents a proliferative disorder.…”

“…The relationship between Grcithcr's disease, UnnaThost's keratoderma and the cpidermolytic type of Vomer has been discussed recently [2,6]. These entities were char acterized by their histologic and clinical differences.…”

Transgrediens et progrediens palmoplantar keratoderma: a case of Greither’s disease

“…Hyperhidrosis is usually associated [6]. Lesions begin after the child reaches 2 years, but a few reports of earlier onset have been described [7]. Spontaneous involution of the disease is recognized in the elderly [8].…”

“…Vorner palmoplantar keratoderma is autosomal dominant but begins soon after birth, is non-transgrediens and shows epidermolysis. Greither's disease is considered by some authors to be a variant of Unna-Thost palmoplantar keratoderma [8], but features such as early onset, absence of transgrediens and lack of involution in later life make most authors classify Greither's disease as a separate entity [3,7,9]. Treatment of Greither's disease is difficult and includes salt-water soaks, topical keratolytics and systemic steroids [10].…”