Mal de Meleda (MDM), also known as keratoderma palmoplantaris transgrediens, is a rare inherited form of palmoplantar keratoderma. It is characterized by erythema and hyperkeratosis of the palms and soles, extending to the dorsal aspects of the hands and feet. A 15-year-old Korean female presented with sharply demarcated hyperkeratotic plaques on the palms and soles, which extended to the dorsa...

Papillon-Lefevre syndrome is a very rare syndrome of autosomal recessive inheritance characterized by palmar-plantar hyperkeratosis and early onset of a severe destructive periodontitis, leading to premature loss of both primary and permanent dentitions. Various etiopathogenic factors are associated with the syndrome but a recent report has suggested that the condition is linked to mutations of...

PapillonLefevre syndrome (PLS) is a very rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and severe early onset of destructive periodontitis leading to premature loss of both primary and permanent dentition.PLS is transmitted as an autosomal recessive condition and consanguinity of parents is evident in about one third of cases. Here we report a case of a 13 year ...

Keratodermas encompass a wide spectrum of disorders of keratinization that may be acquired or hereditary. We present two cases of focal acral hyperkeratosis (FAH), a subtype of punctate palmoplantar keratoderma. We review the literature and attempt to clarify the confusing classification of the heritable punctate palmoplantar keratodermas.

Keratosis follicularis spinulosa decalvans is a rare disease, with genetic transmission either X-linked or sporadic, characterized by follicular hyperkeratosis and cicatricial alopecia. The disease usually begins in early childhood exacerbating throughout adolescence. The therapies are somewhat effective, with frustrating treatment when there are changes which are predominantly cicatricial. It ...

1. A study was undertaken to investigate the role of vitamin E in the aetiology of phrynoderma (follicular hyperkeratosis). Fifty-six children with the disease and twenty-one normal children were investigated for this purpose. 2. Plasma vitamin E levels (mean +/- SE; mg/l) were found to be low in phrynoderma (3.7 +/- 0.19) in contrast with normal children (6.6 +/- 0.40) and therapy with a combi...

Sir, Spiny keratoderma is a rare disease characterized by keratotic spicules resembling a “music box spine” located on the palms and soles (1). This entity has been reported under several different names, such as punctate keratoderma (2), punctate porokeratotic keratoderma (3), palmar filiform hyperkeratosis (4), and spiny keratoderma of the palms and soles (1). We present here a case of spiny ...