eMedicine Specialties > Ophthalmology > Dermatologic Disorders
Ichthyosis: Treatment & Medication
Updated: Feb 18, 2010
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Systemic
- Oral retinoids display an impressive antikeratinizing action in ichthyosiform dermatoses. Etretinate (1 mg/kg/d) and isotretinoin (2 mg/kg/d) have been shown to reduce scaling, discomfort, and disfigurement. However, when these drugs are discontinued, the ichthyotic skin recurs, thereby necessitating long-term use. Similarly, liarozole (150 mg bid), an imidazole derivative, inhibits the cytochrome P450-dependent 4-hydroxylation of retinoic acid, resulting in increased tissue levels of retinoic acid and a reduction in epidermal proliferation and scaling.
- Patients with epidermolytic hyperkeratosis may develop chronic bacterial infections of the skin necessitating long-term antibiotic therapy. In these cases, benzathine penicillin G 1.2 million U intramuscularly (IM) is given every 2-3 weeks until the skin infection subsides. Oral erythromycin ethyl succinate 800 mg 4 times daily may be substituted in penicillin-allergic patients.
- Patients with Sjögren-Larsson syndrome have a deficiency of fatty aldehyde dehydrogenase (FALDH). Data suggest that bezafibrate, a hypolipidemic drug, induces the activity of FALDH in patients with some residual enzyme activity.
- Ocular
- In chronic ocular surface disorders associated with ichthyosis, nonpreserved artificial tears (carboxymethylcellulose sodium 0.5-1.0%) and ointment (white petrolatum 56.8%, mineral oil 41.5%) are preferred to prevent complications from dryness and exposure.
- Preservative-free lubricants may be used as often as needed while decreasing the incidence of preservative-related allergies.
- In cases where poor corneal epithelial adhesion is present, bandage contact lenses and temporary collagen shields may decrease symptoms and promote surface healing.
- Topical
- Topical cyclosporine A 2% given 3 times daily has been shown to be beneficial in the treatment of deep stromal keratitis associated with KID syndrome.
- To prevent cicatricial ectropion in lamellar ichthyosis, a humidified atmosphere combined with the use of topical moisturizing agents is beneficial. Petrolatum ointment and 10% urea cream applied to the eyelid skin several times daily helps to prevent skin contracture. Salicylic acid 2% and retinoic acid 0.1% ointments also are effective, but local irritation may limit their frequency of use. In the hyperproliferative variants of ichthyosis, calcipotriene ointment has been shown to be beneficial. However, the use of calcipotriene in treating congenital hyperproliferative disorders is limited by the theoretical risk of hypercalcemia from absorption of the drug from the skin.
- In addition to systemic broad-spectrum penicillins, pimecrolimus cream 1% has been shown to be effective in patients with Netherton syndrome. The immunomodulating effects are similar to tacrolimus but without evidence of lipophilic adverse effects.
Surgical Care
- When cicatricial ectropion develops in patients with lamellar ichthyosis despite room humidification and vigorous skin lubrication, the danger of corneal breakdown and perforation is noted. Full-thickness skin grafts from the forearm, postauricular, and groin areas may be used to successfully repair the abnormalities. In addition, Apligraf (Organogenesis Inc, Canton, Mass), a human skin equivalent, may facilitate the repair of cicatricial ectropion in severe cases when autologous donor graft tissue is not available. A concomitant medial and/or lateral lid tarsorrhaphy is recommended in severe cases. The incidence of ectropion recurrence may be decreased if surgery can be postponed until suitable nonscaly patches of skin can be clearly identified to serve as graft donor sites.
- For a persistent corneal epithelial defect, an amniotic membrane transplantation may be necessary to promote epithelial wound healing.
- For diffuse limbal stem cell deficiency, keratolimbal allografting with chronic systemic immunosuppression may be necessary, although the success rate has been poor.
Consultations
- Because ichthyosis is primarily a skin disorder, periodic evaluation by a dermatologist is recommended.
- The ophthalmologist may be helpful in the treatment of ocular manifestations and in the identification of the specific type of ichthyosis, particularly, lamellar and X-linked forms.
Diet
- In patients with Refsum disease (ichthyosis and pigmentary retinopathy), chlorophyll in the diet should be excluded (ie, green vegetables [phytanic acid], animal fat [phytol]). Because rapid weight loss mobilizes tissue phytanic acid, this should be avoided.
Medication
Oral retinoids display an impressive antikeratinizing action in ichthyosiform dermatoses. Isotretinoin (2 mg/kg/d) has been shown to reduce scaling, discomfort, and disfigurement. However, when these drugs are discontinued, the ichthyotic skin recurs, thereby necessitating long-term use.
Liarozole (150 mg bid), an imidazole derivative, inhibits the cytochrome P450-dependent 4-hydroxylation of retinoic acid, resulting in increased tissue levels of retinoic acid and a reduction in epidermal proliferation and scaling.
Patients with epidermolytic hyperkeratosis may develop chronic bacterial infections of the skin necessitating long-term antibiotic therapy. In these cases, benzathine penicillin G 1.2 million U intramuscularly (IM) is given every 2-3 weeks until skin infection subsides. Oral erythromycin ethylsuccinate 800 mg qid may be substituted in penicillin-allergic patients.
N-acetylcysteine 10% emulsion, a nontoxic and hypoallergenic amino acid derivative, can be safely and efficaciously used in the topical treatment of neonatal ichthyosis.
Retinoids
These agents decrease cohesiveness of abnormal hyperproliferative keratinocytes and may reduce potential for malignant degeneration. They modulate keratinocyte differentiation and have been shown to reduce risk of skin cancer formation in renal transplant patients.
Isotretinoin (Accutane)
The synthetic 13-cis isomer of the naturally occurring tretinoin (trans -retinoic acid). Both agents are structurally related to vitamin A.
Adult
2 mg/kg/d PO
Pediatric
Not recommended due to bone metabolism defects
Toxicity may occur with vitamin A coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; isotretinoin may reduce plasma levels of carbamazepine
Documented hypersensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
May decrease night vision; inflammatory bowel disease may occur; may be associated with development of hepatitis; occasional exaggerated healing response of acne lesions; excessive granulation with crusting may occur; diabetic patients may experience problems in controlling their blood sugar while on isotretinoin; avoid exposure to UV light or sunlight until tolerance achieved; discontinue treatment if rectal bleeding, abdominal pain, or severe diarrhea occur; long-term vitamin A therapy in children can induce cortical hyperostosis, premature closure of the epiphyses of the long bones, periosteal calcification, and demineralization of the long bones and spine
Imidazole derivatives
These agents inhibit the cytochrome P450-dependent 4-hydroxylation of retinoic acid, resulting in increased tissue levels of retinoic acid and a reduction in epidermal proliferation and scaling.
Liarozole
Imidazole broad-spectrum antifungal agent; inhibits cytochrome P450 metabolic pathways, increasing levels of cytochrome P450 metabolized drugs. The US FDA has not approved this medication for use.
Adult
150 mg PO bid
Pediatric
Not established
Not established
Documented hypersensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Not established
Antibiotics
Patients with epidermolytic hyperkeratosis may develop chronic bacterial infections of the skin necessitating long-term antibiotic therapy.
Penicillin G benzathine (Bicillin LA)
Interferes with synthesis of cell wall mucopeptides during active multiplication, which results in bactericidal activity.
Adult
1.2 million U IM q2-3wk until skin infection subsides
Pediatric
<27.3 kg: 300,000-600,000 U IM
>27.3 kg: Administer as in adults
Probenecid can increase penicillin effectiveness by decreasing clearance; coadministration with tetracyclines can decrease effectiveness of penicillin
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in impaired renal function
Erythromycin (EES, E-Mycin, Ery-Tab)
This is for patients with epidermolytic hyperkeratosis who develop chronic bacterial infections of the skin necessitating long-term antibiotic therapy and are allergic to penicillin.
Adult
800 mg PO qid
Pediatric
40-50 mg/kg/d PO in divided doses
Coadministration may increase toxicity of theophylline, digoxin, carbamazepine, and cyclosporine; may potentiate anticoagulant effects of warfarin; coadministration with lovastatin and simvastatin, increases risk of rhabdomyolysis
Documented hypersensitivity; hepatic impairment; coadministration with astemizole (withdrawn from US market)
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in liver disease; estolate formulation may cause cholestatic jaundice; GI adverse effects are common (give doses pc); discontinue use if nausea, vomiting, malaise, abdominal colic, or fever occur
Keratolytic agents
These agents are used to prevent cicatricial ectropion in lamellar ichthyosis, a humidified atmosphere combined with the use of topical moisturizing agents is beneficial. Petrolatum ointment and 10% urea cream applied to the eyelid skin several times daily helps to prevent skin contracture. Salicylic acid 2% and retinoic acid 0.1% ointments are also effective, but local irritation may limit their frequency of use.
Urea (Ureacin, Ureaphil, Carmol)
Promotes hydration and removal of excess keratin in conditions of hyperkeratosis.
Adult
Topical cream (2%, 10%, and 20%) or lotion (2% and 10%) can be applied to affected skin areas tid
Pediatric
Apply as in adults
None reported
Documented hypersensitivity; viral skin disease
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Do not use near eyes; caution if applied to broken or swollen skin
Lubricants
In chronic ocular surface disorders associated with ichthyosis, nonpreserved artificial tears (carboxymethylcellulose sodium 0.5-1.0%) and ointment (white petrolatum 56.8%, mineral oil 41.5%) are preferred to prevent complications from dryness and exposure. Preservative-free lubricants may be used as often as needed while decreasing the incidence of preservative-related allergies. In cases where poor corneal epithelial adhesion is present, bandage contact lenses and temporary collagen shields may decrease symptoms and promote surface healing.
Carboxymethylcellulose 0.5 to 1% (Celluvisc)
Lubricates and relieves dry eyes and eye irritation associated with deficient tear production.
Adult
1 gtt to affected eye(s) q1h or qid prn
Pediatric
Administer as in adults
None reported
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Do not use with contact lenses; discontinue use if eye pain, irritation, continued redness, or vision changes occur
More on Ichthyosis |
| Overview: Ichthyosis |
| Differential Diagnoses & Workup: Ichthyosis |
 Treatment & Medication: Ichthyosis |
| Follow-up: Ichthyosis |
| Multimedia: Ichthyosis |
| References |
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Further Reading
Keywords
ichthyosis vulgaris, lamellar ichthyosis, epidermolytic hyperkeratosis, X-linked ichthyosis, acquired ichthyosis, congenital ichthyosiform erythroderma, CIE, KID syndrome, CHIME syndrome, Netherton syndrome, Sjögren-Larsson syndrome, CHILD syndrome, treatment, symptoms
