acquired ichthyosis
TRANSCRIPT
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ACQUIRED ICHTHYOSIS
Dr Yugandar
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The word ichthyosis comes from the Greek word for
a fish.
ichthyosis : is a group of disorders that are
characterized by a persistent, non-inflammatory
scaling disorder of the skin surface.
It is caused by abnormality in keratinization and
exfoliation of the horny cell layer.
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The ichthyoses are a clinically and genetically
heterogeneous group of skin disorders,
characterized by a diffuse, generally uniform and
persistent pattern of scaling without mucosal or
extracutaneous (except in ichthyosiform syndromes)
involvement.
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Acquired ichthyosis is clinically indistinguishable
from hereditary ichthyosis; however, acquired
ichthyosis is associated with various systemic
diseases.
The appearance of ichthyosis in adulthood can
occur before or after the diagnosis of a systemic
condition.
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cancer (especially lymphoma, Hodgkin disease,
non-Hodgkin lymphoma,mycosis
fungoides, myeloma, Kaposi sarcoma,
leiomyosarcoma & carcinomas of the
lung, breast, ovary & cervix)
sarcoidosis
leprosy
thyroid disease, hyperparathyroidism
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nutritional disorders
chronic renal failure
bone marrow transplantation
HIV infection
Autoimmune diseases, including systemic lupus
erythematosus and dermatomyositis
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certain medications has been linked to acquired
ichthyosis
nicotinic acid, triparanol,
butyrophenones, dixyrazine,
cimetidine, and clofazimine
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Histology: compact orthokeratosis and/ parakeratosis without spongiosis
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Restrictive Dermopathy
24 cases Lethal, AR Prematurity, fixed facial expression,
micrognathia, mouth in “O” position Rigid and tense skin with erosions
and denudations & multiple joint contractures
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Ichthyosis Linearis Circumflexa
Disorder of keratinization in which bizzare
migratory annular and polycyclic patches
occur
Leave no scarring or pigmentary changes.
Inheritance AR, patients are born
erythrodermic
fatal complications.
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Most also have trichorrhexis invaginata
May clear completely in summer time
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Chanarin-Dorfman Syndrome - Neutral Lipid Storage Disease
Ichthyosis, Myopathy
and lipid vacuoles ->
Impaired degradation
of triacylglycerol-
derived diacylglycerol
Dietary modulation of
fats aids in controlling
the diseaseLipid vacuoles in granulocytes and monocytes but not lymphocytes or erythrocytes
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Ichthyosis Follicularis (IFAP Syndrome)
IFAP = Ichthyosis Follicularis, Alopecia,
Photophobia
Generalized spiny follicular lesions with xerosis
of non-follicular skin, striking alopecia.
M>F 5:1
X-linked recessive and AD forms reported
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Sjogren-Larsson Syndrome
Fatty alcohol oxidoreductase deficiency
Infancy: generalized erythroderma, ichthyosis,
fine to large lamellar scaling
After Infancy: generalized darker scale without
erythema accentuated in flexures and lower
abdomen; spares central face.
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CNS: MR, spastic diplegia with scissor gait
Eyes: atypical retinitis pigmentosa “glistening
dots” pattern-slit
Dental dysplasia
Tx: Low fat diet
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•SJOGREN LARSSON SYNDROME - atypical retinitis pigmentosa “glistening dots” pattern on slit lamp exam.
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Refsum’s Syndrome
Phytanol-CoA hydroxylase deficiency
Leads to phytanic acid deposition in…
Skin (ichthyosis)
CNS (ataxia, peripheral neuropathy)
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Eyes (retininitis pigmentosa “salt & pepper”)
Ears (deafness)
Cardiac (arrhythmias, block, CHF)
Musculoskeletal (wasting, skeletal
anomalies)
TX: dietary restriction of phytanic acid.
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Rud’s Syndrome
AR or X-linked
Ichthyosis
Hypogonadism
Small stature
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MR, Epilepsy
Macrocytic anemia
Rule out Refsum’s Syndrome or Sjogren-
Larsson Syndrome
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BIDS
Brittleness of hair d/t sulfur def Impairment of intelligence Decreased fertility Short stature
K/a Trichothiodystrophy
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IBIDS
Some cases of BIDS a/w Lamellar Ichthyosis
I Stands for Ichthyosis
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PIBIDS
AR inheritance P stands for Photosensitivity Defect in DNA repair Similar to cockayane syndrome &
xeroderma pigmentosum
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KID Syndrome
Keratitis
Ichthyosis
Deafness
DISTINCTIVE LEATHERY VERRUCOID PLAQUES
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Leathery verrucous plaques Nails may be dystrophic Eye brows and eye lashes absent Partial anhidrosis Hypotrichosis A/w Mucocutaneous candidiasis.
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CHILD syndrome
Cong Hemidysplasia Ichthyosiform erythroderma Limb defects Present at birth,females affected Ichthyosis confined to one half of body
& ipsilateral hypoplasia of bony structures and brain.
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CHIME syndrome
AR,first in 1983 identified Ocular COLOBOMAS Congenital HEART disease Migratory ICHTHYOSIFORM
Dermatosis Mental Retardation Epilepsy
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Thank You Very Much for Attention
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