Epidermolytic Hyperkeratosis


Onset of ichthyosis, say before 5 years of age.

Ichthyosis Vulgaris Ichthyosis vulgaris (IV) is the most common ichthyosis that results in diffuse fine scaling, usually worse on the legs.
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Lamellar Ichthosis One of the classic autosomal recessive congenital ichthyosis (ARCI) which in many patients is caused by mutations in the gene encoding the transglutaminase 1 (TGM1) enzyme, which is responsible for assembly of the cornified envelope.

Recessive X-Linked Ichthyosis RXLI is a is an X-linked ichthyosis and thus predominantly affects male patients. Cryptorchidism and testicular cancer have been associated. Female carriers may exhibit a mild ichthyosis and during pregnancy, they may fail to spontaneously enter labor.
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Sjögren-Larsson Syndrome Sjögren-Larsson syndrome combines ichthyosis, mental retardation and spastic diplegia or tetraplegia.

Chanarin-Dorfman syndrome Peripheral blood smear shows lipid vacuoles in granulocytes. Liver biopsy may also be done, showing steatosis and large lipid vacuoles in the cytoplasms of hepatocytes.

Bathing Suit Ichthyosis The ichthyosis is limited to the trunk, neck and scalp.

Superficial Epidermolytic Ichthyosis Mild hyperkeratosis, superficial blisters and shedding.

Acquired, in an Adult

Medications: Drugs have been implicated in the etiology and include lipid-lowering agents, such as nicotinic acid and triparanol, and butyrophenones, such as haloperidol. Other reported medications include cimetidine, clofazimine, and allopurinol.

Malignancies: Hodgkin disease accounts for more than half of cases. Other reported malignancies include non-Hodgkin lymphoma, mycosis fungoides, carcinoma of the breast, lung, and cervix, multiple myeloma, Kaposi sarcoma, and leimoyosarcoma.

HIV infection and AIDS
Lymphoma. A 65-year-old woman with a 3-month history of dry patches with large scales, diffuse pruritus, and hair loss. Examination showed enlarged lymph nodes. Hodgkin lymphoma was found. JAAD 2011;65, Issue 2, Pages 465–467

Acquired, Round Lesions

Pityriasis Rotunda


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