Disseminated Porokeratosis Mibelli Treated with RO 10-9359

Abstract

2 patients with widespread porokeratosis Mibelli (PM) were treated orally with RO 10-9359. The dose was 75 mg/day for 10 days, then 50 mg/day for 3 weeks. The drug produced a good improvement of condition with no serious side effect. Ultrastructural examination of a healed lesion showed the presence of a fine granular substance in the intercellular space of the spinous layer, most likely produced by keratinocytes; ultrastructural cellular alterations of PM were still evident and the lesion recurred after suspension of treatment.

Oral retinoids-present status

“…Finally, a number of miscellaneous other skin conditions have been reported to improve markedly after oral therapy with aromatic retinoid: verrucous epidermal naevus (Happle, Kastrup & Macher, 1977)5 subcorneal pustular dermatosis of Sneddon-Wilkinson (Folkers & Tafelkruger, 1978), Netherton's syndrome (Duperrat et al, 1977), epidermolytic hereditary palmoplantar keratoderma , plantar warts (Rust & Rufii, 1979), superficial actinic type of porokeratosis (Kariniemi, Stubb & Lassus, 1980), and disseminated porokeratosis of Mibelli (Bundino & Zina, 1980). So far it seems that two oral retinoids may have considerable chances to become established as routine therapy in the next few years: (a) the aromatic retinoid Ro 10-9359 for a broad spectrum dermatotherapy, particularly in psoriasis, lichen planus, keratotic genodermatoses in general (inclu-ding Darier's disease), and in precancerous lesions of the oral mucosa, and (b) the 13-cis-retinoic acid particularly for severe nodulocystic acne.…”

Porokeratosis of Mibelli: Successful Treatment with Cryosurgery

“…Reports of oral therapy with etretinate (16, 17) have demonstrated variable success without long‐term remission. Topical 5‐FU may require treatment of long duration and cause some difficulties such as irritation, pigmentary changes and recurrence (8, 18). Other therapies including carbon dioxide laser (10, 19) and dermabrasion (20) have also been tried; but scarring and hypopigmentation ensued.…”

Porokeratoses—A Comprehensive Review on the Genetics and Metabolomics, Imaging Methods and Management of Common Clinical Variants

“…On the other hand, topical tretinoin did not provide satisfactory outcomes in one case [347], whereas in another, it led to a reduction in hyperkeratosis without any impact on lesion size (unknown regimen; 1-year treatment) [357]. Systemic (0.5-0.7 mg/kg per day) and topical etretinate once daily for 4 weeks provided an unsatisfactory effect in one case of the hyperkeratotic PM subtype [291], whereas in another six cases, systemic etretinate was effective [320,[358][359][360][361]. A severe verrucous PM case associated with HIV responded well to oral acitretin (25 mg daily), antiretroviral therapy (efavirenz/tenofovir/emtricitabine) and oral trimethoprim/sulfamethoxazole by week 16, and achieved complete clearance after 1 year of treatment [289].…”