tretinoin and Basal-Cell-Nevus-Syndrome

We describe a 9 year old boy with skin changes, minor facial signs, jaw cysts and cryptorchism, compatible with Gorlin syndrome. His 7 year old sister has similar skin changes, while their father has radiographic signs of the syndrome. Gorlin syndrome is rarely described in childhood when it occurs without major manifestations or associated neoplasia. The monitoring for prevention of complications is the greatest problem at this age.

Topics: Basal Cell Nevus Syndrome; Child; Chromosomes, Human, Pair 9; Family; Female; Humans; Male; Tretinoin

The case of a child with basal cell nevus syndrome whose condition was successfully managed for 10 years with a combination of topical 5-fluorouracil and tretinoin is reported. The concurrent use of these two agents prevented the development of new tumors, inhibited the growth of existing tumors, and caused the regression of superficially invasive basal cell carcinomas.

Topics: Basal Cell Nevus Syndrome; Child, Preschool; Drug Synergism; Drug Therapy, Combination; Female; Fluorouracil; Humans; Skin Neoplasms; Time Factors; Tretinoin

Two patients with multiple basal cell carcinomas, due either to the nevoid basal cell carcinoma syndrome (NBCCS) or arsenical insecticide exposure, were treated with oral isotretinoin for 7 or 8 years, respectively. Gradually decreasing dosage levels were employed. During the initial courses of therapy, high doses (2.0-3.0 mg/kg/day) were intended as chemotherapy. In these patients only 6 of 40 (15%) lesions underwent complete clinical regression. In subsequent courses aimed at chemoprevention, the dose was progressively reduced from 1.5 to 0.25 mg/kg/day. During therapy, no new lesions were observed in the patient with the arsenical exposure. The NBCCS patient developed 1 new lesion during therapy at 1.0 mg/kg/day, 1 new lesion at 0.5 mg/kg/day and 5 new lesions at 0.25 mg/kg/day. Treatment was discontinued and the patient with the arsenic exposure developed his first new tumor 17 months afterwards; in contrast, the NBCCS patient developed 29 tumors within 13 months. These findings suggest that long-term therapy with isotretinoin is needed for the continuation of the cancer chemopreventive effect. However, the need for continuous rather than intermittent maintenance therapy, and the determination of the optimal dose for this purpose may depend on the etiology of the multiple carcinomas and on the tolerability of the lowest effective dose by the individual patient. With these encouraging data, it now appears appropriate to expand this pilot study and perform larger trials to determine the usefulness of isotretinoin in the chemoprevention of basal cell carcinoma in patients with multiple tumors.

Topics: Basal Cell Nevus Syndrome; Carcinoma, Basal Cell; Humans; Isotretinoin; Male; Middle Aged; Neoplasms, Multiple Primary; Prognosis; Skin Neoplasms; Tretinoin

A histologically confirmed, clinically inapparent and reversible hepatitis occurred in 2 patients (1 psoriasis, 1 basal cell nevus syndrome) within the first months after introduction of etretinate therapy. Causes of hepatitis other than etretinate were not found. Reintroduction of etretinate resulted in reactivation and/or persistence of the hepatitis in both patients. These data strongly suggest that the hepatitis in both patients was caused by etretinate. Later the basal cell nevus syndrome patient was given 13-cis-retinoic acid, which caused no liver test disturbances during a follow-up period of 6 months.

Topics: Aged; Basal Cell Nevus Syndrome; Biopsy; Carcinoma, Basal Cell; Chemical and Drug Induced Liver Injury; Etretinate; Female; Humans; Liver; Middle Aged; Psoriasis; Tretinoin

Topics: Adult; Basal Cell Nevus Syndrome; Carcinoma, Basal Cell; Cell Transformation, Neoplastic; Etretinate; Facial Neoplasms; Humans; Male; Tretinoin

Beneficial effects of oral retinoids in prophylaxis of epithelial neoplasias have been demonstrated by experimental works. In this study oral retinoid (RO 10-9359) was used in human dermatosis with high frequency of cutaneous malignancies: xeroderma pigmentosum with or without malignant neoplasias, Mibelli's porokeratosis with multifocal malignant degeneration, basal cell naevus syndrome with basal cell carcinomas, familial epithelioma of Ferguson-Smith and actinic keratosis. This work started in december 1977. Visible epitheliomas have been treated before trial. Initial dose of retinoid was 1 mg/kg daily, decreased depending on individual tolerance. Results were appreciated by comparising number of epitheliomas observed in years preceding retinoid therapy and number of them appearing during treatment; in two familial cases (basal cell naevus syndrom in twins and xeroderma pigmentosum in two brothers) comparison was made between treated and untreated patients. First results are very promising: an excellent response on solar keratosis is noted; epitheliomas occurrence seems actually to be prevented or delayed by oral retinoid therapy. Of course more numerous cases, a longer time, periods without treatment are necessary to confirm these interesting first results. On the other hand drug is not with this dose active on already constituted carcinomas.

Topics: Adolescent; Basal Cell Nevus Syndrome; Carcinoma, Basal Cell; Carcinoma, Squamous Cell; Child; Etretinate; Female; Humans; Keratosis; Male; Precancerous Conditions; Skin Diseases; Skin Neoplasms; Tretinoin; Xeroderma Pigmentosum