LOCALIZATION OF THE GENE WHOSE MUTATIONS UNDERLIE HAILEY-HAILEY DISEASE TO CHROMOSOME

Hailey-Hailey disease (familial benign chronic pemphigus) is an autoso mal dominant skin disease characterized by impaired keratinocyte cohes ion and consequent blister formation. In the present study we have use d linkage analysis to map the gene for this disease to a region of chr omosome 3q between D3S1589 and D3S1316. The maximum combined two point lod score in four families studied was 14.60 at theta = 0 at the D3S1 290 microsatellite repeat. These findings suggest the presence of a ge ne not previously known to be involved in keratinocyte cohesion at thi s site.