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  1. Dry Skin.
    Ichthyosis ranges from mild but annoying dryness to severe dryness with scales and flaking that becomes disfiguring. Several ichthyotic skin diseases are inherited; ichthyosis is a symptom in several rare hereditary syndromes and in several systemic disorders.
  2. Xeroderma (xerosis), the mildest form of ichthyosis, is neither congenital nor associated with systemic abnormalities. It usually occurs on the lower legs of middle-aged or older patients, most often during cold weather and in those who bathe too frequently. There may be mild to moderate itching and an associated dermatitis caused by detergents or other irritants.


Defective DNA repair is associated with (AIIMS Nov 09)
a. Xeroderma pigmentosum
b. Albinism
c. Ichthyosis
d. Vitiligo


Ans- A. Xeroderma Pigmentosum

  1. Inherited Ichthyoses, which are characterized by excessive accumulation of scale on the skin surface, are classified according to clinical and genetic criteria.
    Ichthyosis is a symptom in Refsum's syndrome (rare hereditary ataxia with polyneuritic changes and deafness caused by a defect in the enzyme phytanic acid hydroxylase) and in Sjögren-Larsson syndrome (hereditary mental deficiency and spastic paralysis); both syndromes are autosomal recessive.
  2. Acquired Ichthyosis may be an early manifestation in some systemic diseases (eg, leprosy, hypothyroidism, lymphoma, AIDS).
    The dry scaling may be fine and localized to the trunk and legs, or it may be thick and widespread.
    Biopsy of ichthyotic skin is usually not diagnostic; however, there are exceptions, most notably sarcoidosis, in which a thick scaling may appear on the legs and biopsy usually shows typical granulomas.
  3. Treatment
    1. In any ichthyosis, minimizing bathing is helpful. Soaps should be used only in intertriginous areas. Hexachlorophene products should not be used because of increased absorption and toxicity.
    2. An emollient--preferably plain petrolatum, mineral oil, or lotions containing urea or -hydroxy acids--should be applied twice daily, especially after bathing (for 10 min to hydrate the stratum corneum) while the skin is still wet. Blotting dry with a towel will remove excess applied oil. Ichthyosis caused by an underlying systemic disease may slightly improve with lubrication using propylene glycol.
    3. To remove the scale in ichthyosis vulgaris, X-linked ichthyosis, and lamellar ichthyosis, a preparation containing 50% propylene glycol in water under occlusion every night after hydration of the skin is particularly effective. In children, it should be applied twice daily without an occlusive dressing overnight. Other useful agents include 5% or 6% salicylic acid gel, hydrophilic petrolatum and water (in equal parts), and cold cream and the -hydroxy acids (eg, lactic, glycolic, and pyruvic acids) in various bases.
    4. Patients with epidermolytic hyperkeratosis (bullous congenital ichthyosiform erythroderma) may need long-term cloxacillin 250 mg Potid or Qid or erythromycin as long as thick intertriginous scaling is present to prevent superinfection with painful, foul-smelling pustules.
    5. The most effective therapies for most ichthyoses are oral synthetic retinoids. Etretinate is effective in X-linked ichthyosis and epidermolytic hyperkeratosis. In lamellar ichthyosis, 0.1% tretinoin (vitamin A acid, retinoic acid) cream or oral isotretinoin may be effective. The lowest effective dosage should be used. Long-term (>= 1 yr) treatment with oral isotretinoin has resulted in bony exostoses in some patients, and other long-term side effects may arise. (Caution: Oral retinoids are contraindicated in pregnancy because of their teratogenicity, and etretinate should be avoided in women of childbearing potential because of its teratogenicity and long half-life.)

  1. Granuloma Annulare
    1. A benign, chronic idiopathic dermatosis characterized by papules or nodules that spread peripherally to form a ring around normal or slightly depressed skin.
    2. The lesions are yellowish tan, erythematous,  or skin colour, and one or more may be present. They are usually asymptomatic and usually occur acrally on the feet, legs, hands, or fingers.
    3. The condition may be seen in children or adults. It is not associated with systemic diseases, except that the incidence of abnormal glucose metabolism is increased among adults with many lesions.
    4. In about 5% of cases, exposure to sunlight has induced disease flares.
    5. Spontaneous resolution is common.
    6. No treatment is necessary. In addition to reassurance and explanation of the benign nature of the disease, the use of high-strength topical corticosteroids under occlusive polyethylene dressings every night, corticosteroid-containing (flurandrenolide) tape, or intralesional corticosteroids may hasten disease involution.

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