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Dr. B.K.
Barik ,
Consultant,
Department
of
General
Medicine
V.S.S.
Medical
College,
Burla
Many of
the
systemic
disease
including
a number
of
endocrinopathies
manifest
on the
skin.
These
skin
markers,
many a
time are
the
characteristic
features
of the
endocrinopathies
or may a
pointer
towards
a group
of
disorders.
So,
these
skin
manifestations
are
important
in
differential
diagnosis
of many
disorders.
Diabetes
mellitus,
hyperthyroidism,
hypothroidism,
disorders
of
adrenals
and
gonads
are the
main
endocrinopathies
having
skin
manifestations
and at
time
these
signs
help in
reaching
a
definite
diagnosis.
Certain
skin
lesions
such as
hyperpigmentation,sclerederma,
xanthomas,
lipomas,
increased
muscle
mass ,
vesico
bullous
eruptions,
yellow
or red
paules,
fungal
or
bacterial
infection,
alopecia
etc need
the
exclusion
of
endocrinopathies.
INTRODUCTION:
Skin is
a very
good
reflector
of
internal
disease
in
general
and
endocrine
diseases
in
particular.
Specific
skin
lesions
may
reflect
a
variety
of
endocrine
diseases
as well
as
manifest
secondary
conditions
underlying
metabolic
disturbances.
A
knowledge
of the
same
makes
the
diagnosis
of
endocrine
diseases
easier,
which
avoids
delay
and
difficulty
in
diagnosis.
A timely
diagnosis
and
early
treatment
spares
future
complications
and
saves
the
patients
from
their
avoidable
suffering.
It is
important
that an
attempt
should
be made
to
review
the
available
literature
to
familiarize
the
treating
physicians
about
the
importance
of skin
markers
of
endocrine
diseases.
These
disorders
are
diabetes
mellitus
and
endocrine
disturbances
of
thyroid
,
adrenals
, gonads
and
others.
I.
DIABETES
MELLITUS
A.
PRIMARY
SKIN
DISEASES
ASSOCIATED
WITH
DIABETES
MELLITUS
1.
Diabetes
demopathy
(pigmented
pretibial
patches,
skin
spots):
Lesion
of
diabetic
demopathy
are less
than 1
cm and
are red
brown ,
atrophic
macules
on the
anterior
shins
which
may
begin as
papules
.
Capillary
basement
membranes
appear
to be
thickened.
2.
Necrobiosis
lipoidica
diabeticorum
(NLD):
MLD
lesions
also
characteristically
appear
on the
anterior
shins
and are
oval,
atrophic
plaques
that can
reach
several
centimeters
in size.
They
have a
raised ,
erythematous
border
and a
yellow –
brown,
waxy
central
atrophic
area
through
which
telangiectatic
subcutaneous
vessels
are
easily
visible.
These
lesions
may also
ulcerate.
3.
Disseminated
granuloma
annulare
(GA):
Multiple
small,
grouped
erythematous
to
flesh-coloured
papules,
which
coalesce
into
small
plaques
covering
the
entire
body
surface
have
been
associated
with
diabetes
mellitus.
However,
patients
with GA,
even if
it is
extensive,
do not
necessarily
have
diabetes
mellitus.
4.
Lipodystrophy.:
a.
The
Siep-Lawrence
syndrome
(and
related
disorders
with
lipodystrophy)
may be
cogential
and
include
insulin-
dependent
diabetes
mellitus,
hypertricosis,
hyperpigmentation,
increased
muscle
mass and
somatic
growth
elevated
trglycerides
and
cirrhosis
of the
liver.
b.
Local
lipoatrophy
or
lipohypertropy
may
appear
at sites
of
insulin
injection.
The
mechanism
is
unclear.
5.
Insulin
resistance
and
acanthosis
nigricans:
May be
associated
with
polycystic
ovaries
in young
women
(Type A)
or with
antibodies
to
insulin
receptors
and
other
autoimmune
phenomena
(Type
B).
6.
Bullous
diabeticorum:
Tense
blisters
that
appear
suddenly
on
apparently
normal
skin
usually
on the
extremities
may
occur in
diabetes.
Histologically
the
blister
may be
intraepidermla
.
Lesions
may tend
to
ulcerate.
7.
Eruptivexanthomas
are
accumulations
of lipid
in
macriphages
to form
widespread
yellow
to red
papules,
which
may
occur in
a linear
fashion
in
response
to
trauma
(the
Koebner
Phenomenon).
These
lesions
appear
in
patients
with
poorly
controlled
diabetes
mellitus
who have
massive
hypertriglyceridemia.
8.
Sclerederma
(adultorum
of
Buschke)
is a
rare
condition
seen in
severe
insulin-
dependent
diabetes
in which
there is
thickening
of the
collagen
in the
dermis
and
deposition
of acid
mucopolysaccarides
between
collagen
bundles.
The
lesions
are
broad
areas of
non-pitting
edema
and
hardening
of the
skin,
especially
on the
face,
neck,
and
upper
trunk.
9.
Idiopathic
hemochromatosis
, a
disorder
of
excessive
iron
storage,
consists
of a
triad of
hepatic
cirrhosis,
cardiac
insufficiency
and
diabetes.
There is
striking
hyperpigmentation
due to
increased
melanin
deposition
in the
basal
layer of
the
epidermis.
B.
SECONDARY
SKIN
DISORDERS.
1.)
Candida
infection
of the
skin and
mucous
membranes
occur in
similar
locations
as
described
in
glucocoriticoid
excess.
2.)
Cutaneous
bacterial
infections,
especially
staphylococcal
infection
such as
folliculitis
furinculocis
and
frank
abscess
formation
may
occur in
poorly
controlled
diabetes
patients.
Opportunistic
infection
by
fungal
organisms
such as
Mucor
and
Rhizopus
(Phycomycosis)
are rare
but are
seen in
diabetics.
Such
lesions
may be
fatal.
II
THYROID
A.
HYPERTHYROIDISM.
1.
General
features
of
hyperthyroidism
include
warmth,
increased
sweating
flushing
and a
smooth
velvety
texture
of the
skin. In
sever
cases
there
may be
generalized.
hyperpigmentation.
2.
Hair and
nails:
Hair may
becomethin,
fine,
limp and
oily.
There is
distal
onycholysis
(separation
of the
nail
plate
from the
nail
bed).
characteristically
starting
on the
fourth
finger (plummer
nail).
3.
Pretibial
myxedema
(PTM) :Localised
accumulations
of acid
mucopolysccharides
(hyaluronic
acid)
appear
as red
to brown
to
yellow
plaque,
usually
on the
anterior
shins.
There is
a “peau
f
orange”
pebbly
surface
with
dilated
hair
follicles
and
coarse
hairs.
Lesions
are
non-pitting,
cool,
non-tender
and may
be
pruritic.
PTM is
seen
with
active
Graves
Diseaseas
well as
or
euthyroid
“burnt
out” or
treated
patients.
High
long-acting
thyroid
stmultor
)LATS)
levels
have
been
associated
with PTM.
4.
Thyroid
acropachy:
Diffuse
thickening
of the
distal
extremities,
clubbing
and
periosteal
new bone
formation
with
characteristic
perpendicular
bone
spicules
are seen
associated
with
Graves
Disease,
often
with
opthalmopathy
and
pretibial
myxedema.
B.
HYPOTHYROIDISM
1.
Congential
signs of
hypothyrodism
are
shubtle
and
should
be
vanishing
with the
advent
of newly
started
screening
programs
for
hypothrroidsm.
General
features
includes
cool,
pale,
translucent,
“alabaster”
skin
with a
yellow
hue.
This
colour
and
texture
are due
to
decreased
blood
flow
anaeia.
infiltration
with
mucopolysaccharides,
prolonged
jaundice
and / or
cartoenmia.
Sweating
is
reduced.
Thermal
instability
and
frank
hypothermiaare
accompanied
by
persistent
cuits
marmorata
(purplish
discoloration),
a
violaceous
mottling
in a
vascular
pattern.
Umbilical
hernia,
short
proximal
limbs
depressed
nasal
bridge
and
large
tongue
complete
the
picture
of
congential
hypothyroidism.
2.
Acquired
hypothyroidism
has many
of the
same
general
feature
as seen
in the
congential
disease.
Children
and
adults,
in
addition
have
more
prominent
dry and
scaling
skin
resembling
ichthyosis.
Sweating
is
minimal
as the
eccrine
ducts
become
atrophic,
Generalised
myxydema
make the
skin
puffy,
pale and
doughy
with a
non-pitting
edema,
espically
noticeable
around
the face
and
eyelids
due to
the
accumulation
of acid
mucopoly
saccharides.
3.
Hair:Scalp
hair is
lost in
a
generalized
pattern.
The
remain
scalp
hair is
coarse
and dry.
Loss of
the
lateral
one
third of
the
eyebrows.
(St. Ann
Sign) is
characteristic.
Pubic
and
axillary
hair is
spares.
Children
may get
a
paradoxical
hypertrichosis
of arms.
back and
legs
which is
reversible
with
treatment.
III.
ADRENAL
A.
HYPOADRENALISM
(Adrenocortical
insufficiency.
Addition’s
disease)
1.
Patients
gradually
develop
generalized
hyperpigmentation,
there is
darkening
of the
aerolae,
genital
skin
preexistent
nevi,
palmar
and
plantar
creases
and
recent
scars.
Blue
black
pigment
deposit
are seen
on the
gum at
the toot
margin
and on
the hard
palate.
The
etiology
of
hyperpigmentationis
probably
due to
excessive
secretion
of
melanocyte
–stimulating
hormone
(MSH).
2.
2). Hair
in pubic
and
axillary
areas
becomes
sparse
and
nails
may
develop
hyperpigmented
and
linear
streaks.
3.
3).
Vitiligo
rarely
occurs
in
patients
with
Addison’s
disease.
B.
HYPERASRENALISM
Pituitary
cushing
syndrome,
adrenal
tumors,
ad
autonomous
nodlar
adrenal
hyperplasia
may have
features
of
androgen
excess
as well
as of
the
glucocorticold
excess.
Iatrogenic
Cushing
Syndrome,
resulting
from
systemic
or
topical
glucocorticosteroids,
does not
have the
androgenic
features.
a).
Primary
features
of
glucocorticoid
excess
1.
General
features
include
plethora,
telangiectasias
and
increased
growth
of fine,
downy
vellus
hair,
especially
on the
sides of
the
face.
With
prolonged
exposure,
there is
generalized
atrophy
of the
skin and
easy
bruisability.
2.
Striae
represents
both
dermal
and
epidermal
atrophy
and
occur in
areas of
skin
tension.
They are
pigmented
and red
to blue
because
the
dermal
and
subcutaneous
blood
vessels
show
through
the
translucent
and
atrophic
skin. In
some
cases,
they may
become
hyperpigmented
with
melanin.
3.
Steriod
acne
appears
on the
upper
arem,
chest
and back
as well
as on
the
face.
The
lesions
are
actually
perifolliclar,
hyperkeratotic
papules,
althroug
in late
stages
these
may
become
pustular
and
comedonal.
The
erupt at
the same
stage of
development.
4.
Redistribution
of
subcutaneous
fat to
the
cheeks,
upper
back,
buttocks
and
abdomen
is
characteristic.
5.
In
pituitary
disease
of in
syndromes
of
ectopic
adrenocorticotropic
hormone
(ACTH)
production,
there
may be
hyperpigmentaton,
such as
seen in
Addition’s
disease.
6.
The
androgenic
effects
from
weak
adrenal
androgens
such as
dehydropiandrosterone
(which
may be
converted
to more
potent
androgens
such as
testosterone
and
dihydrotestosterone)
produce
classic
acne
vulgaris
as well
as
hirsutism
and male
pattern
hair
loss in
women.
h.
Secondary
features
of
glucocorticoid
excess
1.
Tinea
versicolor
appear
as
scaly,
oval
macules
and
patches
on the
upper
trunk,
back and
arms wit
variable
hyperpigmentation
or
hypopigmentation.
it is
due to a
superficial
colonization
with the
fungus.
Malassezia
furfur.
2.
Candida
(Monilia)
infections
of the
skin and
mucous
membranes
include
buccal
mucosa
(thrush)
vaginal
mucosa,
nails
and
surrounding
tissues
(onychomycosis
and
paronychia).
Interdigital
webs,
corners
of the
mouth (perleche)
and
intertriginous
folds (intertigo).
3.
There is
an
increased
occurrence
of
cutaneous
bacterial
infections
with
pustules,
furuncles
and
abscesses
especially
caused
by
Staphylococcus
aureus.
C.
CONGENTIALADRENAL
HYPERPLASIA
(CAH)
1.
Infancy,
severe
forms of
CAH are
caused
by
enzyme
defects
in the
to the
synthesis
of
cortisol
and may
have
only
mild
cutaneous
signs,
primarily
hyperpigmentation
of
genital
skin
from
excessive
pituitary
production
of ACTH
or MSH
or both.
2.
In later
childhood,
early
onset of
pubic
and
auxiliary
hair and
acne are
signs of
androgen
excess.
3.
In
postpubertal
adult
women
and
perhaps
in some
men
“mild”
(or
“partial”
or late
onset”)
forms of
CAH are
now
being
recognized
primarily
from
their
skin
manifestation
of
severe
late
onset or
persistent
acne,
hair
loss and
hirsutism
in
women.
Hirustism
is
defined
as
excessive
hair in
androgen-dependent
areas ,
such as
upper
lip,
chin,
areolae,
linea
albha
and
upper
inner
thighs.
Male
pattern
hair
loss
(androgenic
alopecia)
is
thinning
of the
hair
over the
crown of
the
scalp
with
relative
sparing
of the
sides
and
back.
Affected
hair are
of
smaller
diameter
and grow
to
shoter
length
before
entering
the
resting
phase
and
falling
out.
The
diagnosis
of CAH
is made
by
finding
elevation
of the
hormone
that the
sustrate
for the
deficient
enzyme (e.g
17-alpha-hydroxyprogesterone
in 21
hydroxylase
deficiency,
II-deoxycortisolin
II-hydroxylase
deficiency
and
17-alpha-
hydroxypregnenolone
in
3-beta-hydroxysteroid
dehydrogenase
deficiency).
If
levels
are not
elevated
in basal
blood
samples,
these
may be
found as
abnormal
responses
to ACTH
stimulation.
IV.
GONADS
A.
Androgen
excess:
from the
ovary
will
cause
the same
skin
manifestations
of acne,
hair
loss,
and/or
hirsutism
as
described
above.
Rarely,
ovarial
tumors
are the
cause;
most
grequently,
polycystic
ovarian
disease
(PCOD)
is the
source
of
hyperandrogenemia
from the
ovary.
B.
In
gonadal
dysgenesis:
(Turner
Syndrome),
there
may be a
few
special
cutaneous
features
such as
multiple
brown
–black
acquired
nevi, a
double
row of
eyelashes
and low
W-shaped
posterior
hairline,
short
fourth
(and
often
fifth)
digits
on the
hands
and feet
with
dysplasia
or
absence
of nails
hyperconvexity
of
finger
nails
and
toenails.
V.
MISCELLANEOUS
ENDOCRINE
DISORDERS
A.
Mucocutaneous
candidiasis
has
been
associated
with
muliple
endocrinopathy
syndrome
of
autoimmune
disorders,
including
hypoparathyroidism,
Addison’s
disease,
premature
ovarian
failure,
diabetes
and
Hashimoto’s
thyroiditis.
Vitiligo
complete
loss of
pigment
in
patches
and
alopecia
areata
patchy
hair
loss
which
may
progress
to
total
loss of
scalp
hair
(alopecia
totails)
or of al
body
hair
(alopecia
universails)
have
also
been
seen in
association
with
these
endocrinopathies.
B.
Alopecia
has been
reported
in
association
with
vitamin
–D
resistant
rickets
in a few
well-studied
families.
C.
Multiple
mucosal
neuromas
are
found in
the
multiple
endocrine
neoplasia
(MEN)
syndrome
in
association
with a
marfanoid
habitus,
thick
lips hig
calcitonin
levels,
medullary
carcinoma
of the
thyroid
and
phenochromocytoma.
D.
The
glucagonoma
syndrome
consists
of a
diffuse,
scaly
brightly
erythematous
rash
made up
of
gyrate,
serpiginous
plaques
with
clear
centers
and
superficial
scales.
The
lesions
tend to
expand,
thus the
name
necrolytic
migratory
erythema.
Such
lesion
are seen
in
association
with
malignant
neoplasms
of the
alpha
cells of
the
pancreas
and have
regressed
when the
tumor
has been
surgically
removed.
E.
Acanthosis
nigricans
(AN) has
been
associated
with a
wide
variety
of
endocrine
disorders
including.
Cushing
syndrome,
pituitary
tumours,
acromegaly,
Addisons’s
disease
and
polycystic
ovarian
disease.
Most
cases of
AN have
underlying
insulin
resistance.
CLINICAL
FOCUS
·
Many
endocrinopathies
involve
skin as
primary
or
secondary
complication.
·
Skin is
a very
good
reflector
of
systemic
disorders
including
the
endocrinal
diseases.
·
Diabetes
mellitus
is
commonly
associated
with
varied
dermatological
lesions.
·
Thyroid
disorder
(hyper
or hypo)
involve
skin
through
various
mechanisms.
·
Adrenal
pathology
is
associated
with
various
manifestation
of skin
and its
appendages.
·
Other
uncommon
endocrinopathies
are also
someway
associated
with
dermatopathies.
CONCLUSION
Endocrinipathies
are
associated
with a
large
number
of skin
markers.
A
perfect
knowledge
about
the
pointers
is
mandatory
for an
endocrinologist
or a
physician.
The
services
of a
dermatologist
will be
of
immense
help in
such a
situation
and
hence
ideally
in case
of doubt
in any
cases
the
opinion
of the
dermatologist
should
be
obtained
in the
greater
interest
of
proper,
diagnosis
and
ideal
patient
care.
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