Treatment of pachyonychia congenita.
There are currently no specific treatments for pachyonychia congenita (PC). Available treatments generally are directed at specific manifestations of the disorder, and an effective treatment plan must recognize that different patients are more or less troubled by different manifestations of the disease. Treatment for all aspects of PC has been less than completely satisfactory. Very few studies have compared different approaches to treatment, and fewer still have given longitudinal follow-up of efficacy and patient acceptance. This review is essentially a compilation of anecdotes. It was collected from physicians' reports in the literature, from direct communication with physicians currently following patients with PC and from patients who answered a questionnaire on the Pachyonychia Congenita Project web page (http://www.pachyonychia.org/Registry.html).
Milstone
Leonard M
LM
Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut 06520-8059, USA. leonard.milstone@yale.edu
Fleckman
Philip
P
Leachman
Sancy A
SA
Leigh
Irene M
IM
Paller
Amy S
AS
van Steensel
Maurice A M
MA
Swartling
Carl
C
D007644:Darier Disease / Q000151:congenital / Q000235:genetics / Q000628:therapy; D004476:Ectodermal Dysplasia / Q000235:genetics / Q000628:therapy*; D005260:Female; D006801:Humans; D007633:Keratins / Q000235:genetics; D007645:Keratoderma, Palmoplantar / Q000151:congenital / Q000235:genetics / Q000628:therapy*; D008297:Male; D009154:Mutation; D009264:Nails, Malformed / Q000151:congenital / Q000235:genetics / Q000628:therapy*
D007633:Keratins