Pathogenesis (development of disease) In people with healthy skin, there is a balance between cell renewal and sloughing off of dead skin cells. Approximately every four weeks, the epidermis (cuticle) renews itself. It consists of several layers. In the lowest layer, the basal layer, the horn-forming cells are created, which migrate upward through the other … Ichthyosis: Causes
General measures Permanent use of ointments and baths to hydrate and nourish the skin. Therapeutic shampoos should be left on the scalp for 5-10 minutes. In mild cases, shampoos with 5% urea pura are sufficient for keratolysis. For the care of the facial skin with existing ectropion (outward protrusion of the eyelid margin) are suitable … Ichthyosis: Therapy
The following are the most important diseases or complications that may be contributed to by ichthyosis: Eyes and eye appendages (H00-H59). If an ectropion is present, the following diseases may occur: Epiphora – leakage of tear fluid over the edge of the eyelid. Keratitis (inflammation of the cornea) Keratoconjunctivitis sicca (dry eyes) Conjunctivitis (conjunctivitis) Endocrine, … Ichthyosis: Complications
International experts settled on the following classification of ichthyoses in the fall of 2010: Primary ichthyoses Isolated common ichthyoses Ichthyosis vulgaris X-linked recessive ichthyosis Isolated congenital ichthyoses Lamellar ichthyoses: Autosomal recessive lamellar ichthyosis. Non-bullous congenital ichthyosiform erythroderma Autosomal dominant lamellar ichthyosis Epidermolytic (bullous) ichthyosis: Bullous congenital ichthyosiform erythroderma (Brocq). Ichthyosis hystrix (Curth-Macklin). Ichthyosis bullosa (Siemens) … Ichthyosis: Classification
A comprehensive clinical examination is the basis for selecting further diagnostic steps: General physical examination – including blood pressure, pulse, body weight, height; further: Inspection (viewing) – appearance depends on the form of ichthyosis (see “Symptoms – Complaints” below). Skin Blistering? Erythroderma (redness of the skin)? Rhagades? (fissures; narrow, cleft-shaped tear that cuts through all … Ichthyosis: Examination
The diagnosis of ichthyosis is usually made on the basis of physical examination findings. The following laboratory diagnostic procedures are available to determine the form of ichthyosis: 1st-order laboratory parameters-obligatory laboratory tests. Histology Filaggrin ↓ – histidine-rich cationic protein; produced in the cornification process of the skin; in ichthyosis vulgaris, it is absent or has … Ichthyosis: Test and Diagnosis
Therapy goals Hereditary (inherited) ichthyoses: no causal therapy possible → symptomatic therapy! Improvement of the skin condition Ensure moisture of the skin Dissolve cornification and dandruff Bring rhagades to heal and avoid new formation Prevent infections Relief of pruritus (itching) Improvement in the quality of life Acquired ichthyosis: Treatment of the underlying disease Therapy recommendations … Ichthyosis: Drug Therapy
The following symptoms and complaints may indicate ichthyosis: Pathognomonic (indicative of disease). Horny and scaly skin surface Other symptoms (depending on the form) Blistering Erythroderma (redness of the skin) Collodion membrane in the newborn (“collodion baby”) – hard, closed layer of skin that quickly tears, breaks open and peels off; under the collodion membrane is … Ichthyosis: Symptoms, Complaints, Signs
Medical history (history of illness) represents an important component in the diagnosis of ichthyosis. Family history Are there any diseases in your family that are common? (Skin diseases, hormonal disorders, metabolic diseases, tumor diseases). Current medical history/systemic history (somatic and psychological complaints). Has the condition existed since birth or occurred shortly thereafter, or has it … Ichthyosis: Medical History
Congenital malformations, deformities, and chromosomal abnormalities (Q00-Q99). Other hereditary forms of ichthyosis. Endocrine, nutritional and metabolic diseases (E00-E90). Acrodermatitis enteropathica – congenital zinc absorption disorder associated with dermatitis (inflammatory reaction of the skin), paronychia (nail bed inflammation), and alopecia (hair loss). Skin and subcutaneous (L00-L99). Other acquired ichthyosis Epidermolysis bullosa (butterfly disease) – genetic skin … Ichthyosis: Or something else? Differential Diagnosis
