(ACA) Acrodermatitis Chronica Atrophicans, also known as Primary diffuse atrophy or Herxheimer disease is indicated by skin rashes, which is the sign of late or third stage European Lyme borreliosis

1. Introduction to
Acrodermatitis
Chronica Atrophicans

2. (ACA) Acrodermatitis Chronica
Atrophicans, also known as Primary
diffuse atrophy or Herxheimer disease is
indicated by skin rashes, which is the
sign of late or third stage European Lyme
borreliosis. ACA is basically a
dermatological condition that faces
chronical progressive course which finally
leads to widespread skin atrophy.
Peripheral nervous system’s involvement
is observed, mainly polyneuropathy.

3. This skin process progression is because
of the effect of continuous and active
infection with spirochete Borrelia afzelii.
In this condition extreme rashes are
caused. In the initial stage, cutaneous
swelling, and bluish red discolouration is
observed. This continues for several
months or even years, after which
atrophic phase is reached. Sclerotic skin
plagues can also develop in some cases.
Wrinkles starts appearing as ACA
progresses.

4. Acrodermatitis Chronica Atrophicans
is long standing as it may last from some
to several years. It leads to immense
skin atrophy in some patients. It may
also limit joint mobility of lower and
upper limb. If ACA in its acute
inflammatory stage gets treated, then
the result will be good. Physicians usually
use histologic and serologic examination
so as to confirm that whether or not a
person is suffering from ACA.

5. This disease is most of the times
unilateral but bilateral ACA is also very
common. Some characteristics symptoms
of Acrodermatitis Chronica
Atrophicans include exaggerated pain
reaction, progressive allodynia, and so
on. If you suffer from any of these
symptoms then it may be a case that you
suffer from ACA. In such a situation,
patients complain of paresthesia,
spontaneous acral pain, cognitive
dysfunction or dysesthesia.

6. Acrodermatitis Chronica Atrophicans
begins with the inflammatory phase
which is characterized by flat infiltrations
whose size varies, or diffuse bluish red
discoloration as well as skin edema. This
condition mostly appears on at least one
distal part of extremity, mainly on bony
prominences extensor surfaces. ACA
frequency is about 1% to 10% in Europe
and these statistics vary from region to
region.

7. This skin condition was first delineated
by Buchwald in 1883. In 1902, Hartmann
and Herxheimer described this condition
as tissue paper and like cutaneous
atrophy. The only type of LB is ACA in
which non-spontaneous remissions occur.
Moreover, the pathophysiology of ACA is
not fully understood. This manifestation
is caused because of the togetherness of
several nonspecific reactions with specific
immune response.

8. Absence of protective antibodies, weak
cellular response, narrow antibody
spectrum, major histocompatibility
systems down-regulation on Langerhans
cells are some of the conditions which
are faced by patients suffering with LB.
The chronicity of Acrodermatitis
Chronica Atrophicans may increase
due to a restricted cytokine expression
pattern, including absence of interferon-
gamma.

9. Autoimmune damage can occur when
responses of cross-reactive antibody take
part in it. It is unclear that whether
autoimmune reactions, play some
significant role in pathogenesis or not.
Periarticular regions are favourable sites
due to reduced oxygen pressure and
acral skin temperature. It should be
noted that timely diagnosis and
treatment can help in controlling the
disease from spreading further.
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