HHD, a
short introduction:
Hailey-Hailey (HHD) is
not an autoimmune disease. It is genetic, and is seen intergenerationally, and
often among siblings and extended family members. There is a 1:2 (50%) chance of
inheriting the disorder from an affected parent.
There is a cell-cell
detachment that resembles the process seen in true pemphigus, but there is no
autoantibody causing the blisters. The detachment is found at the cell level and
is exacerbated by friction and sweat. Loss of one functional copy of the gene
coding for the protein SPCA1 (ATP2C1) causes Hailey-Hailey disease. This protein
functions in skin cells to transport calcium and manganese ions. The gene has
been mapped to chromosome 3, specifically 3q21-q24.
Because the disease is
genetic, there is no cure. Treatment consists of oral antibiotics, anti-fungals
and/or anti-virals to control secondary infection, astringents, cool compresses
and dressings. Also, topical steroid creams applied have been known to help in
most cases, although their long-term use is discouraged due to thinning of the
already sensitive skin. Oral steroids and Dapsone are tried to help severe
breakouts. Laser resurfacing, dermabrasion and surgical excision and skin
grafting are tried in severe cases.
Good description of HHD in lay terms.
What is all this about a calcium pump anyway?
What works for one may not work for another...
Check these out for more information.
If you have questions about HHD in "wet" places, this may help.
Here's what you should remember about HHD, in a nutshell.
