ACQUIRED ICHTHYOSIS

Dr Yugandar

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.

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.

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.

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

nutritional disorders

chronic renal failure

bone marrow transplantation

HIV infection

Autoimmune diseases, including systemic lupus

erythematosus  and dermatomyositis

certain medications has been linked to acquired

ichthyosis

nicotinic acid, triparanol,

butyrophenones, dixyrazine,

cimetidine, and clofazimine

Histology: compact orthokeratosis and/ parakeratosis without spongiosis

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

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.

Most also have trichorrhexis invaginata

May clear completely in summer time

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

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

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.

CNS: MR, spastic diplegia with scissor gait

Eyes: atypical retinitis pigmentosa “glistening

dots” pattern-slit

Dental dysplasia

Tx: Low fat diet

•SJOGREN LARSSON SYNDROME - atypical retinitis pigmentosa “glistening dots” pattern on slit lamp exam.

Refsum’s Syndrome

Phytanol-CoA hydroxylase deficiency

Leads to phytanic acid deposition in…

Skin (ichthyosis)

CNS (ataxia, peripheral neuropathy)

Eyes (retininitis pigmentosa “salt & pepper”)

Ears (deafness)

Cardiac (arrhythmias, block, CHF)

Musculoskeletal (wasting, skeletal

anomalies)

TX: dietary restriction of phytanic acid.

Rud’s Syndrome

AR or X-linked

Ichthyosis

Hypogonadism

Small stature

MR, Epilepsy

Macrocytic anemia

Rule out Refsum’s Syndrome or Sjogren-

Larsson Syndrome

BIDS

Brittleness of hair d/t sulfur def Impairment of intelligence Decreased fertility Short stature

K/a Trichothiodystrophy

IBIDS

Some cases of BIDS a/w Lamellar Ichthyosis

I Stands for Ichthyosis

PIBIDS

AR inheritance P stands for Photosensitivity Defect in DNA repair Similar to cockayane syndrome &

xeroderma pigmentosum

KID Syndrome

Keratitis

Ichthyosis

Deafness

DISTINCTIVE LEATHERY VERRUCOID PLAQUES

Leathery verrucous plaques Nails may be dystrophic Eye brows and eye lashes absent Partial anhidrosis Hypotrichosis A/w Mucocutaneous candidiasis.

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.

CHIME syndrome

AR,first in 1983 identified Ocular COLOBOMAS Congenital HEART disease Migratory ICHTHYOSIFORM

Dermatosis Mental Retardation Epilepsy

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