Diagnosis

Ichthyosis vulgaris

Etiology

Cause [duv]

• Acquired ichthyosis: detected in adulthood

  • Underactive thyroid gland

  • Kidney disease

  • Cancer: Hodgkin's lymphoma, multiple myeloma, cutaneous T cell lymphoma, SLE

  • Infection: HIV/AIDS, Leprosy

  • Inflammation: Sarcoidosis, celiac disease and Crohn’s disease

  • Drugs: hydroxyurea, vemurafenib, protease inhibitors (HIV treatment), nicotinic acid, cimetidine, clofazimine, allopurinol, EGFR inhibitors, BRAF inhibitors

• Hereditary ichthyosis: detected in childhood (>30 types exist)

  • Ichthyosis vulgaris: most common, mild, detected usually in infancy

  • X-linked recessive ichthyosis: 1 of 6,000 people, usually males

  • Harlequin ichthyosis

  • Epidermolytic ichthyosis

  • Lamellar ichthyosis

  • Congenital ichthyosiform erythroderma

  • Erythrokeratodermia variabilis

  • Progressive symmetric erythrokeratoderma

Pathophysiology [duv]

1. Hereditary: Autosomal semi-dominant disorder with mutation in the filaggrin gene (responsible for aggregating keratin filaments in the cornified cell envelope)

2. Disruption of the epidermal barrier

3. Increased risk of penetration of allergens and microorganisms

4. Higher tendency of atopic dermatitis, hand eczema, irritant contact dermatitis, and allergic contact dermatitis

Epidemiology

Prevalence per 100.000 [ua3]

Incidence per 100.000 [ua3]

Approach

• Family history (genetic disorder)

• Dermatoscopy

• Biopsy

• Genetic testing

Treatment

1. Rehydrate the skin: [yzb]

   • Air humidifier

   • Frequent baths

   • Apply moisturizer immediately after the bathing

   • Alpha-hydroxy acids (lactic, glycolic, or pyruvic acids)

   • Topical retinoids

2. Remove the scales:

   • Soften the scales during baths and then remove them

   • Keratolytics (salicylic acid)

3. Treat skin damage:

   • Apply petroleum jelly to skin cracks

   • Treat a skin infection

Differential diagnoses

Contact dermatitis, Drug eruptions, Eczema, Impetigo, X-linked recessive ichtyosis