tretinoin and Pityriasis-Rubra-Pilaris

Topics: Acne Vulgaris; Adolescent; Child; Child, Preschool; Darier Disease; Etretinate; Female; Humans; Ichthyosis; Infant; Isomerism; Isotretinoin; Keratins; Keratoderma, Palmoplantar; Male; Pityriasis Rubra Pilaris; Psoriasis; Skin Diseases; Skin Diseases, Vesiculobullous; Tretinoin

Topics: Acne Vulgaris; Administration, Oral; Adolescent; Dermatitis, Exfoliative; Half-Life; Humans; Ichthyosis; Isotretinoin; Neoplasms; Pityriasis Rubra Pilaris; Psoriasis; Tretinoin

Topics: Clinical Trials as Topic; Humans; Ichthyosis; Isotretinoin; Keratosis; Pityriasis Rubra Pilaris; Skin Diseases; Tretinoin

Forty-five patients with pityriasis rubra pilaris were treated with oral 13-cis-retinoic acid (isotretinoin). There was marked improvement in the degree of erythema, in duration, and scaling noted within 4 weeks. Remission or maintained improvement persisted after stopping therapy in many of the patients. The drug is a significant addition to the therapeutic armamentarium in this difficult-to-treat disease.

Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Clinical Trials as Topic; Female; Humans; Infant; Isomerism; Isotretinoin; Male; Middle Aged; Pityriasis Rubra Pilaris; Tretinoin

Pityriasis rubra pilaris (PRP) is an uncommon cutaneous disease with disorder of keratinisation. Up to now, systemic retinoids like acitretin or isotretinoin seem to be the most effective therapeutic agents. However, no large trials on this rare disease have been published and no standardised treatment has been established so far. Recently, single case reports demonstrate beneficial effects of alitretinoin (9-cis retinoic acid) in patients with PRP. We performed a retrospective observational analysis of type I adult-onset patients with PRP (n = 5) treated with systemic alitretinoin in our department. Alitretinoin was highly effective in the treatment of PRP in 4 of 5 cases. PASI score was reduced significantly in the alitretinoin responders. We assume that alitretinoin could serve as an additional effective systemic treatment option for type I adult-onset PRP.

Topics: Aged; Aged, 80 and over; Alitretinoin; Female; Humans; Keratolytic Agents; Male; Middle Aged; Pityriasis Rubra Pilaris; Remission Induction; Retrospective Studies; Treatment Outcome; Tretinoin

Topics: Administration, Oral; Aged; Alitretinoin; Antineoplastic Agents; Biopsy, Needle; Dose-Response Relationship, Drug; Drug Administration Schedule; Follow-Up Studies; Hand Dermatoses; Humans; Immunohistochemistry; Male; Pityriasis Rubra Pilaris; Severity of Illness Index; Treatment Outcome; Tretinoin

Topics: Administration, Oral; Aged; Alitretinoin; Anti-Inflammatory Agents; Female; Humans; Pityriasis Rubra Pilaris; Treatment Outcome; Tretinoin

Topics: Adult; Biopsy; Female; Humans; Keratolytic Agents; Pityriasis Rubra Pilaris; Skin; Treatment Outcome; Tretinoin

We describe a patient with severe pityriasis rubra pilaris in whom extensive extraspinal hyperostoses developed after 13 years of oral retinoid treatment. The most prominent abnormality was a bridging exostosis between the left acetabulum and collum. X-ray examinations of the spine during retinoid therapy showed no abnormalities. During oral retinoid treatment, it is important to ask the patient on a regular basis about any skeletal pains or mobility restriction. Normal spinal x-ray results are no guarantee that a patient is free of hyperostoses. Discontinuation of acitretin therapy resulted in a severe exacerbation of the patient's pityriasis rubra pilaris after 2 weeks. The clinical response to administration of azathioprine was clearly inferior to that of acitretin. However, low-dose oral methotrexate therapy appeared to be a good alternative in this patient, with a clinical result comparable to acitretin and no side effects after 6 months of therapy.

Topics: Acetabulum; Acitretin; Adolescent; Adult; Azathioprine; Child; Etretinate; Exostoses; Femur Neck; Follow-Up Studies; Humans; Hyperostosis; Male; Methotrexate; Pityriasis Rubra Pilaris; Retinoids; Tretinoin

Five patients with pityriasis rubra pilaris were treated with isotretinoin from September 1982 through 1985. Isotretinoin at an average dose of 1.16 mg/kg/day for 16 to 24 weeks caused complete or almost complete clearing in four of the five patients.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Female; Humans; Isotretinoin; Male; Middle Aged; Pityriasis Rubra Pilaris; Tretinoin; Triamcinolone; Triglycerides

Topics: Adolescent; Adult; Aged; Darier Disease; Female; Humans; Isotretinoin; Male; Middle Aged; Pityriasis Rubra Pilaris; Skin; Skin Diseases, Vesiculobullous; Tretinoin

The light and electron microscopic structure of pityriasis rubra pilaris (PRP) is described in five patients. Hyperkeratosis, hypergranulosis, keratotic plugs in the follicular openings, acanthosis and focal parakeratosis were observed. A moderate perivascular infiltrate was seen in the upper dermis. Electron microscopy revealed moderately activated keratinocytes, a decreased number of tonofilaments and desmosomes, enlarged intercellular spaces, parakeratosis with lipid-like vacuoles and a large number of keratinosomes. Lymphoid cells were present in the epidermis in moderate numbers. At the dermo-epidermal junction, the basal lamina was focally split, containing gaps. Etretinate therapy produced moderate to marked clinical improvement. The histological picture improved but the typical signs of PRP, including follicular plugging, persisted. Ultrastructurally the cellular activity and the amount of hyperkeratosis and parakeratosis decreased, while increases in keratinosomes, intercellular substance, microvilli and desmosomes were observed during treatment.

Topics: Adolescent; Adult; Capillaries; Epidermis; Etretinate; Humans; Keratins; Langerhans Cells; Male; Microscopy, Electron; Middle Aged; Pityriasis Rubra Pilaris; Skin; Time Factors; Tretinoin

Topics: Etretinate; Female; Humans; Middle Aged; Pityriasis Rubra Pilaris; Tretinoin

Topics: Child, Preschool; Humans; Male; Pityriasis Rubra Pilaris; Skin; Tretinoin; Vitamin A

Twenty patients with disabling disorders of keratinization were treated with Tigason (etretinate, Ro 10-9359), an oral aromatic retinoid, and the clinical responses and the effects on the skin were monitored. Most patients showed a considerable clinical improvement within 4 weeks. Side effects, such as cheilitis, were common but mostly transient or minor. In the skin there was a decrease in glucose-6-phosphate dehydrogenase activity within the granular cell layer of the epidermis, and an increase in mean epidermal thickness and mean corneocyte area. However, there was little apparent effect on epidermal proliferation or on histological and ultrastructural appearances. These findings suggest that the drug acts at a late stage of epidermal differentiation.

Topics: Drug Administration Schedule; Etretinate; Humans; Keratosis; Pityriasis Rubra Pilaris; Tretinoin

Topics: Child; Child, Preschool; Etretinate; Female; Humans; Male; Pityriasis Rubra Pilaris; Tretinoin

A patient with widespread PRP was first treated with an oral aromatic retinoid (RO 10--9359) and PUVA in successive periods. Retinoid treatment resulted in marked improvement but during PUVA treatment the disease became re-activated. The patient was finally treated with aromatic retinoid alone, which brought the disease to remission in 6 weeks.

Topics: Administration, Oral; Etretinate; Humans; Male; Middle Aged; Pityriasis Rubra Pilaris; Tretinoin

A new synthetic oral retinoid, 13-cis retinoic acid, is fairly well tolerated in patients and appears to be effective in those with Darier's disease and lamellar ichthyosis. It is less effective in those with pityriasis rubra pilaris. The mechanism of action of 13-cis retinoic acid in disorders of keratinization is unknown at the present time; however, it does not appear to cause lysosomal proliferation in therapeutic doses.

Topics: Adolescent; Adult; Cheilitis; Child; Conjunctivitis; Darier Disease; Dose-Response Relationship, Drug; Evaluation Studies as Topic; Female; Humans; Ichthyosis; Isotretinoin; Male; Middle Aged; Pityriasis Rubra Pilaris; Tretinoin

Five patients with Darier's disease and 6 patients with pityriasis rubra pilaris were treated with 13-cis-retinoic acid. Extracts of separated epidermis were assayed for extractable protein, lactic dehydrogenase, Cathepsin D, beta glucuronidase and neutral proteinase before beginning therapy and 2, 4 and 8 weeks after therapy had begun. The epidermal extracts from patients with pityriasis rubra pilaris before beginning therapy were similar to extracts from normal control patients. During the course of therapy with 13-cis-retinoic acid, protein extractability, lactic dehydrogenase and neutral proteinase did not change; there was a highly significant decrease in the specific activity of the lysosomal hydrolases Cathepsin D and beta glucuronidase. A similar but less dramatic fall was noted in the Darier's patients taking 13-cis-retinoic acid. Darier's patients also had a decrease in neutral proteinase activity before beginning therapy; the specific activity of this enzyme increased during the course of therapy. 13-cis-retinoic acid does not induce clinical remission by increasing the intracellular concentration of lysosomal enzymes in epidermis in vivo.

Topics: Darier Disease; Endopeptidases; Epidermis; Humans; Hydrolases; Lysosomes; Pityriasis Rubra Pilaris; Tretinoin

Topics: Acne Vulgaris; Administration, Oral; Carcinoma, Basal Cell; Darier Disease; Drug Evaluation; Humans; Ichthyosis; Pityriasis Rubra Pilaris; Skin Neoplasms; Tretinoin; Vitamin A

Thirteen patients with keratinising dermatoses were treated for 2-17 weeks with oral 13-cis retinoic acid. There was near complete clearing of the skin lesions beginning within 2 weeks of starting treatment in all five patients with lamellar ichthyosis (including two cases of non-bullous congenital ichthyosiform erythroderma), in two of the three patients with Darier's disease, and in one patient with pityriasis rubra pilaris. The patients with psoriasis and naevus comedonicus did not improve. The main form of toxicity was cheilitis. These results indicate that 13-cis retinoic acid may be more effective and is less toxic than naturally occurring retinoic acid (all-trans vitamin A acid), and that the synthetic retinoids may represent a potent new class of drugs in the treatment of cutaneous disease.

Topics: Administration, Oral; Adult; Cheilitis; Child; Child, Preschool; Darier Disease; Drug Evaluation; Female; Follow-Up Studies; Humans; Ichthyosis; Male; Middle Aged; Pityriasis Rubra Pilaris; Skin Diseases; Tretinoin; Vitamin A

Topics: Administration, Oral; Adolescent; Adult; Aged; Child; Darier Disease; Female; Humans; Ichthyosis; Keratoderma, Palmoplantar; Keratosis; Leukoplakia; Male; Middle Aged; Pityriasis Rubra Pilaris; Psoriasis; Skin Neoplasms; Tretinoin; Vitamin A

Up to date, the treatment of palmar/plantar hyperkeratoses presents a therapeutic problem. The known therapeutic procedures result in short-term improvement only, if any at all. In these investigations involving 68 patients suffering from palmar/plantar hyperkeratoses of different etiology, small doses of vitamin A acid locally applied, produced a striking improvement in hypertrophic lichen planus of palms or soles: the regression was complete and in most cases permanent. The skin texture of patients with genetic keratoses and callosities became normal within a few weeks: but this condition remained free of symptoms only as long as vitamin A acid was used as a maintenance dose once or twice weekly. In hyperkeratotic eczema, pityriasis rubra pilaris, and verrucae plantaris vitamin A acid locally applied was found to be unsuitable for treatment. The possible side effects of this treatment are mentioned. Several possibilities regarding the way of action of vitamin A acid are discussed.

Topics: Callosities; Eczema; Humans; Keratoderma, Palmoplantar; Lichen Planus; Long-Term Care; Occlusive Dressings; Ointments; Pityriasis Rubra Pilaris; Tretinoin; Vitamin A; Vitamin A Deficiency; Warts