Acquired ichthyosis

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Transcript of Acquired ichthyosis

1. Dr Yugandar 2. 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. 3. 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. 4. 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. 5. 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 6. nutritional disorders chronic renal failure bone marrow transplantation HIV infection Autoimmune diseases, including systemic lupus erythematosus and dermatomyositis 7. certain medications has been linked to acquired ichthyosis nicotinic acid, triparanol, butyrophenones, dixyrazine, cimetidine, and clofazimine 8. Histology: compact orthokeratosis and/ parakeratosis without spongiosis 9. 24 cases Lethal, AR Prematurity, fixed facial expression, micrognathia, mouth in O position Rigid and tense skin with erosions and denudations & multiple joint contractures 10. 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. 11. Most also have trichorrhexis invaginata May clear completely in summer time 12. Ichthyosis, Myopathy and lipid vacuoles -> Impaired degradation of triacylglycerol- derived diacylglycerol Dietary modulation of fats aids in controlling the disease Lipid vacuoles in granulocytes and monocytes but not lymphocytes or erythrocytes 13. 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 14. 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. 15. CNS: MR, spastic diplegia with scissor gait Eyes: atypical retinitis pigmentosa glistening dots pattern-slit Dental dysplasia Tx: Low fat diet 16. SJOGREN LARSSON SYNDROME - atypical retinitis pigmentosa glistening dots pattern on slit lamp exam. 17. Phytanol-CoA hydroxylase deficiency Leads to phytanic acid deposition in Skin (ichthyosis) CNS (ataxia, peripheral neuropathy) 18. Eyes (retininitis pigmentosa salt & pepper) Ears (deafness) Cardiac (arrhythmias, block, CHF) Musculoskeletal (wasting, skeletal anomalies) TX: dietary restriction of phytanic acid. 19. AR or X-linked Ichthyosis Hypogonadism Small stature 20. MR, Epilepsy Macrocytic anemia Rule out Refsums Syndrome or Sjogren- Larsson Syndrome 21. Brittleness of hair d/t sulfur def Impairment of intelligence Decreased fertility Short stature K/a Trichothiodystrophy 22. Some cases of BIDS a/w Lamellar Ichthyosis I Stands for Ichthyosis 23. AR inheritance P stands for Photosensitivity Defect in DNA repair Similar to cockayane syndrome & xeroderma pigmentosum 24. Keratitis Ichthyosis Deafness DISTINCTIVE LEATHERY VERRUCOID PLAQUES 25. Leathery verrucous plaques Nails may be dystrophic Eye brows and eye lashes absent Partial anhidrosis Hypotrichosis A/w Mucocutaneous candidiasis. 26. 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. 27. AR,first in 1983 identified Ocular COLOBOMAS Congenital HEART disease Migratory ICHTHYOSIFORM Dermatosis Mental Retardation Epilepsy 28. Thank You Very Much for Attention Have a Nice Day Thank You Very Much for ur Attention