A case of Greither’s disease with late onset of disease: a rare presentation

Greither’s disease, also known as transgrediens et progrediens keratoderma, is a non-epidermolytic palmoplantar keratoderma with autosomal dominant inheritance, usually presenting after 2 years of age and resolving after the fifth decade. It is characterized by diffuse involvement of palms and soles with an erythematous border [1, 2].
A 22-year-old male patient, born out of a non-consanguineous marriage, presented to our outpatient department with skin thickening over both palms and soles extending to the dorsal aspect for the last 7 years (fig. 1). The skin over knees, elbows and ankle joints was also thickened (fig. 1). It was not associated with hyperhidrosis. There was no history of similar lesions in the family members. Examination revealed diffuse palmoplantar keratoderma with erythematous borders, extending onto the dorsal aspect of hands and feet. Hyperkeratotic plaques were seen on bilateral knees and elbows and tendo-achilles areas. Hair, nails and teeth were normal. Systemic examination and routine investigations were within normal limits. Skin biopsy from the palm revealed irregular acanthosis and dermal mild inflammatory infiltrate.
In 1952, Greither first reported a case of this unique clinical phenotype and named it as “keratosis extremitatum hereditaria progrediens” [3]. This was followed by a few case reports of the same entity by the name of transgrediens et progrediens in the literature [2–4]. Non-epidermolytic palmoplantar keratoderma (PPK) with erythematous border extending to the dorsum (transgrediens) and the involvement of wrist flexures (progrediens) and tendon of Achilles are the salient morphological characteristics of the disease [5]. Hyperkeratotic plaques can be present on knees and elbows. Hyperhidrosis is also commonly seen [6]. Histopathological findings are not typical for the disease and include acanthosis and orthohyperkeratosis. Although Greither’s disease has a unique phenotype, there have been recent doubts on its existence as a discrete entity. It is believed by some to be an extensive variant of the Unna-Thost palmoplantar keratoderma [1]. However, the distinct features of the latter such as early age of onset before 2 years, lack of transgrediens and absence of spatial involution are not seen in Greither’s disease. The treatment is difficult like all PPKs, and treatment options include salt water soaks with paring, topical keratolytics and systemic retinoids [7].
We report this case in view of...


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