Circumscribed Palmoplantar Keratoderma

Abstract

Melbourne SUMMARY A case of focal tender thickening of the palms and soies, with associated abnormalities of the cornea, is described. It is believed to be an example of circumscribed paimo-plantar keratoderma, of autosomai recessive inheritance. Possible mechanisms involved in the production of the lesions are considered, and an approach to treatment discussed.The classjlicatiou of the hereditary palmo-plantar keratodermas is difficult, as ideally both clinical and genetic patterns must be taken into account. However, classification is usually based upon the mode of inheritance, the conditions being divided into dominant and recessive forms (Eook, 1968). In general, those of recessive type are more commonly accompanied by other ectodermal abnormalities than are the dominant types.Among the autosomai recessive syndromes, there is included circumscribed palmo-plantar keratoderma, which itself has been further sub-divided into three varieties. In the first, the plantar lesions are both diffuse and circumscribed, and on the palms there is a radiating keratosis and sub-ungual keratosis. In the second, there are painful calluses, with associated herpetiform corneal dystrophy, oligophrenia, brachytelephalangia, and liyperhidrosis. In the third, there are calluses and hypotrichosis, oligoi)hrenia, leukokeratosis of the buccal mucous membrane, pachyonychia, and liyperhidrosis (Fi-anceschetti and Schnyder 1960) (Baer and Witten 1960-61). The case described is believed to belong to the second of these sub types. CASE EEPORTThe patient, an only child, is a four year old Italian girl. Both parents were born in northern Italy, and have no cutaneons abnormality. Maternal and paternal grandpai-ents were also unaffected. There was no consanguinity.At the age of three months, it was noticed by the parents that the child's eyes were red and watery, and that she tended to keep her eyes closed on exposure to light. There was associated nasal congestion and rhinorrhea. Ophthalmological examination at this stage revealed mild conjunctival injection only.However, tlie symptoms persisted and at the age of seven months she was found to have a dendritic ulcer of the left cornea and a central punctate keratitis on the right. The lesions appeared to heal within a few weeks but recurred a month later, when she presented with bilateral dendritic ulcers. Again, healing followed. It Avas felt at this stage that the condition was caused by herpes simplex infection.At the age of fifteen months, callus-like thickenings began to appear on the palms, and at twenty four months, the soles of the feet were also involved. These lesions were quite characteristic in their distribution, and mode of evolution. On the palms, they involved the volar surfaces of the distal phalanges, and the hypothenar eminences (Figure 1). On the soles, the pressure areas were affected (Figure 2). The callosities were circumscribed and 1-2 cm in diametei". The most striking feature of the syndrome, however.

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