Congenital atrichia or papular atrichia is a unique condition in terms of
the hair loss pattern. It was the first human hair loss disease researchers
determined was caused by a single gene defect. Although the condition is
generally regarded as a hypotrichosis, it is not strictly so. People with
congenital atrichia can be born with a full head of hair like any normal baby.
But in early childhood they lose all their hair, and it never regrows.
Normal hair follicles rely on chemical communication between two basic cells
types: modified keratinocytes, which form the outer skin epithelium, and
modified fibroblasts, called dermal papilla cells. These two cell groups must
"talk" to each other through biochemical signals to ensure that hair growth and
cycling occurs. The cells must stay in close contact with each other to keep
the process going. One cell population cannot grow hair without receiving
signals from the other cell population.
The mechanism of congenital atrichia is not fully understood, but it seems
that as the hair follicles enter their first resting (telogen) state in early
childhood, the two cell types get separated from each other. The epithelial
cell component of the hair follicle retracts, as it should when hair follicles
go into the resting phase of the hair cycle. Normally the dermal papilla cells
would also go with the retracting epithelial cells, keeping in close contact,
but in congenital atrichia they don't.
Instead the dermal papilla cells get left behind deep in the skin and too
far away from the epithelial cells to talk to them. Without this communication
a new anagen growth phase cannot occur and hair never grows again. While
congenital atrichia is genetic and runs in families, it is a gene defect that
can spontaneously develop in some embryos born to parents who do not have the
condition. Some people diagnosed with alopecia universalis have congenital
Published on March 1, 2010