Indications and Dosage

The efficacy of systemic retinoid therapy in a variety of dermatologic diseases, such as acne, psoriasis (pustular and erythrodermic types), and disorders of keratiniza-tion (ichthyoses, symmetric progressive erythrokeratoderma, Darier disease, pityriasis rubra pilaris, and palmoplantar hyperkeratosis), is well known. There are also reports of successful treatment of other dermatologic conditions, including disorders of epidermal differentiation (epidermodysplasia verruciformis, confluent and reticulated papillomatosis, and axillar granular parakeratosis) and inflammatory and immunodermatoses (atrophoderma vermiculatum, lichen planus, sarcoidosis, and granuloma annulare). Various synthetic retinoids have also been tried in the treatment of patients with different forms of cutaneous LE (CLE), and there are numerous reports of good responses to etretinate, acitretin, and isotretinoin (Duna and Cash 1995, Furner 1990b). Etretinate has been shown to be effective in the treatment of DLE, particularly the hyperkeratotic variant, and SCLE (Grupper and Berretti 1984, Lubach and Wagner 1984, Ruzicka et al. 1985). Subsequently, acitretin, the major metabolite of etretinate, given in oral doses of 50 mg/day initially, has also been reported to be effective in patients with DLE and SCLE (Fig. 27.4). The dose was individually adjusted according to clinical efficiency and side effects. The main advantage of using acitretin in the treatment of CLE compared with etretinate is the much shorter elimination half-life of the drug (although there is retroconversion to etretinate in some patients). The interesting observation that hydroxychloroquine leads to a decrease in serum triglyceride levels suggests that this effect may offset the acitretin-induced hypertriglyceridemia, a side effect frequently limiting the use of this drug. Therefore, the combination of low doses of acitretin with hydroxychloroquine should be considered (Ruzicka et al. 1988,1992). Finally, there are also reports describing therapeutic responses of LE to isotretinoin. Isotretinoin given in doses from 0.5 to 1.0mg/kg per day showed clinical improvement in patients with DLE and SCLE, particularly in those with erythematous, scaly lesions. The therapy was associ-

Fig. 27.4. a 29-year-old patient with subacute cutaneous lupus erythematosus before therapy. b Complete clearing of skin lesions with extensive depigmentation two weeks after therapy with acitretin

ated with the resolution of cutaneous histopathologic abnormalities, conversion of abnormal lesional direct immunofluorescence to normal, normalization of the epidermis on electron microscopy, and reduction in the number of T cells near the der-moepidermal junction without change in the ratio of T-helper (inducer) cells to T-suppressor (cytotoxic) cells. Retinoids may be useful in establishing rapid control of resistant lesions or in treating intermittent acute flare-ups, whether used as monotherapy or in combination with antimalarials or corticosteroids and in patients with CLE refractory to other drug regimens. Because of the recurrence of skin lesions after discontinuing medication use, long-term treatment may be needed to maintain control (Brecher and Orlow 2003, Duna and Cash 1995, Ellis and Krach 2001, Furner 1990a, Newton et al. 1986, Shornik et al. 1991).

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