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

Abstract

Keratosis palmoplantaris progrediens et transgrediens (Greither’s disease) was first described by Greither in 1952. The inheritance pattern is autosomal dominant with variable expression. The clinical manifestation is characterized by diffuse palmoplantar keratoderma associated with hyperhidrosis and progressive extension of keratoderma to the dorsum of the hands and feet. In addition, hyperkeratotic plaques may occur on the elbows and knees. We describe a 44-year-old patient with typical features of Greither’s disease. Immunohistologic and ultrastructural investigations are presented.

Greither's disease: a case report

“…[5][6][7][8] Greither's disease is characterized by diffuse keratoderma of the palms and soles, which may be slight, but by definition extends to the dorsal aspects (transgrediens), characteristically involving the skin over the Achilles tendon. Hyperkeratotic erythematous plaques may also be present over the knees and elbows.…”

“…Flückiger and Itin studied a case of Greither's disease ultrastructurally and found cytoskeleton aberrations, filament aggregations, and cellto-cell junctions with an imbricated pattern. 5 Some consider it as a distinct entity while others consider it as a variant of the Unna-Thost type of PPK. 9 It is claimed to differ from the Unna-Thost variety by showing extension to the extensor surface of the hands, knees and elbows and by showing a tendency to improve in the fifth decade.…”

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

“…Histopathology is non-specific and shows orthohyperkeratosis, acanthosis, absence of epidermolysis and perivascular infiltrate consisting mostly of lymphocytes. Electron microscopy shows tonofilament aggregation and an imbricated pattern of desmosomes and cell junctions [9].…”

“…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].…”

Les kératodermies palmo-plantaires héréditaires

“…L'accentuation de l'hyperkératose au niveau des tendons d'Achille est typique. Cette hyperkératose est parfois fissurée ; elle est toujours associée à une hyperhidrose [45,133]. Une acrocyanose est rapportée dans quelques cas [7].…”