Congenital leukonychia totalis in two brothers.

Sir, was no known consanguinity in the reported family. For this Leukonychia, or white nails, is a common finding in nails of reason, the occurrence of white nails in the two brothers seems fingers or toes (1). It is a very heterogeneous condition, with to be an autosomal recessive segregation. There is only one many different causes. In a recent review, Grossmann & Scher case to our knowledge in the world literature, in which a similar listed over 70 such causes (2). Acquired forms must be constellation is reported (5). One can hypothesize about other differentiated from hereditary forms. Depending on the reasons for the appearance of leukonychia totalis in the two involvement of the nail plate, leukonychia is morphologically brothers, for instance common genetic phenomena such as classified into striata, partialis and finally leukonychia totalis, variable expressivity (6) and incomplete penetrance (7). This in which all nails are completely white (3). In most cases, seems unlikely, however, in our patients, because of the complete leukonychia appears secondarily, with incomplete involvement involvement of the nails and the lack of any nail affections in of the nails. Among the hereditary forms, a simple autosomal related persons. Another possible explanation for the unusual dominant form in different variations is known. Here we segregation is a spontaneous somatic mutation in one of the describe the extremely rare case of two brothers with total parents, which may then have been autosomally transmitted leukonychia, without any other symptoms and without with a gonadal mosaicism. Such mutations are well known in manifestation in any other family member. other autosomal dominant diseases (8). Finally, it is possible that the two brothers share a common, extramarital father and that leukonychia is the result of a simple autosomal dominant CASE REPORT transmission. Having read the case report by Frydman & Cohen A 28-year-old male patient was admitted to our hospital because of (5), we considered whether a somatic mutation may occur more contact dermatitis due to nickel. On physical examination, all twenty frequently than supposed, or whether this unusual segregation nails were completely porcelain white (Figs. 1 and 2). The patient and is a further sign of an autosomal recessive inheritance. Since especially the nails showed no other pathological symptoms. The hereditary forms of leukonychia are not always associated with parents of the patient had noticed the unusual colour of the nails other symptoms, one can speculate that there are more cases immediately after birth. Broad laboratory tests as well as X-rays of chest and head were without any pathological findings. Because of the than the occasional reports in the literature indicate. known common inheritance, we inquired about similar anomalies in Unfortunately, there is not sufficient data about the frequency other members of the family and learnt that the only brother, who was of leukonychia totalis. The histopathological findings in true 32-year-old, had the same condition. The physical examination, together leukonychia are not yet clear. Mostly it is believed that parakerawith laboratory findings and X-ray screenings in the brother, also totic foci could be the reason for leukonychia. revealed only a total leukonychia of all twenty nails. However, no other It should be mentioned that except from masking the discolmember of the extended family had features of any kind of leukonychia. oration with nail varnish, there is no possibility of successful treatment of leukonychia totalis. In our case, both brothers DISCUSSION were proud of their special condition; the nails were fluorescent in UV-light in discotheques, exerting a certain influence on Total leukonychia is a rare condition of the nails, with an interested women. autosomal dominant inheritance. The nails may be milky, In conclusion, these two brothers with leukonychia totalis, chalky, bluish or porcelain white (3). Earlier, Bauer reported without affection of members of the extended family, represent on 16 family members in 3 generations who had leukonychia an extremely rare constellation, in which the underlying genetic totalis (4). In our case, no other family member in the last 3 generations had had such a discoloration of the nails. There mechanisms are not yet clear.