Enfermedad de Darier tratada con acitretina oral / Dariers disease treated with oral acitretin

La enfermedad de Darier (ED) descrita por Darier y White en 1889, es un trastorno autosómico dominante de la queratinización, causada por una mutación del gen ATP2A2, localizado en el cromosoma 12 que codifica para la bomba de calcio ATP-asa de tipo 2 del retículo sarco-endoplásmico (SERCA-2), que conduce a una queratinización anormal (disqueratosis) y pérdida de la adhesión intercelular de los queratinocitos (acantolisis), con la consecuente formación de hendiduras suprabasales. Se caracteriza por alteraciones de la queratinización de la epidermis, uñas y mucosas. Presentamos el caso de un paciente masculino de 21 años, con lesiones cutáneas e histológicas características de (ED), quien realizó tratamiento con acitretina oral a una dosis de 30 mg al día, con respuesta favorable. Se plantea una alternativa terapéutica con retinoides orales para las lesiones crónicas y resistentes.(AU)

Dariers disease (DD) described by Darier and White in 1889 is an autosomic dominant disorder of keratinization, caused by a mutation of gen ATP2A2, located in the chromosome 12 encoding for calcium ATPase pump of sarcoplasmic reticulum type 2 (SERCA-2) leading to abnormal keratinization (dyskeratosis) and loss of intercellular adhesion of keratinocytes (acantholysis), with the consequent formation of suprabasal clefts. Characterized for abnormal keratinization in the epidermis, nails and mucosae. We present the case of a 21-year-old, male patient, with clinically and histologically confirmed (DD) treated with oral acitretin 30 mg for day with good response. We recommend an optional therapeutic treatment for chronic, itchy, recalcitrant lesions with oral retinoids.(AU)

A Case of Dariers Disease Treated with Adapalene / 대한피부과학회지

Dariers disease is inherited as an autosomal dominant trait and characterized by follicular pruritic hyperkeratotic papules. The onset is usually in late childhood and the course of the disease is chronic and slowly progressive in most cases. Dariers disease is refractory to treatment with most conventional keratolytics and anti-inflammatory agents. To date, oral and topical retinoid products have been the mainstay therapy for this population, but local as well as systemic side effects often limit their effectiveness. We present a case of localized Dariers disease which responded promptly to the topical application of adapalene 0.1% gel without side effects. A review of treatment for Dariers disease also is presented.

A Case of Flexural Darier' s Disease / 대한피부과학회지

Dariers disease is a domirantly inherited skin disease that is characterized by keratotic papules predominantly on the sebornieic areas, palmar pits, and a nail dystrophy. The diagnosis is difficult in the small group with flexural involvement because the features of flexural Dariers disease overlap clinically and histolopica.lly with those of Hailey-Hailey disease. The diagnosis of Dariers disease may be confirmed if the patient has the pathognomonic nail dystrophy. We presents a case of flexured. Dariers disease in a 65 year old woman. She has suffered from skin lesions which have some resemblance to those of Hailey-Hailey disease. We could diagnose her skin lesions as Dariers disease because she had the eharacteristic nail dystrophy.

A Case of Darier' s Disease Treated by the Combined Treatment with Oral Etretinate and Topical Tretinoin Cream / 대한피부과학회지

We report a case of Dariers disease which showed a more effective result using a combined treatment with oral etretinate and topical tretinoin cream than treatment with oral etretinate alone in a 17-year old male patient. Oral etretinate 40mg was given daily and topical therapy with tretinoin cream was applied to lesions of neck and face. Two weeks later, lesions of neck and face showed good improvement compared with the not applied lesions. Topical therapy was then extended to other lesions. Six weeks after treatment, there was a marked improvement. Three months after treatment, most of lesions were nearly gon.

Two cases of Darier's disease treated with etretinate / 대한피부과학회지

We report two cases of Darier's disease improved with Etretmate in the 44-year-old father and his 18-year-old son. Clinically, multiple dirty brownish, verrucous, discrete or confluent papules and plaques were noted on their back, abdomen, and face. Histopathologically, both of them demonstrated hyperkeratosis, suprabasal acantholysis, acantholytic cells in the lacunae, corpsronds, grains, and dyskeratotic cells. Oral Etretinate 30mg daily was given in both cases with remakable improvement 3 weeks after treatment. Six months later, they showed almost total clearin, of their skin lesions. No specific adverse effect was noted. There has been no evidence of recurrence for 6 months follow up period.

Eczema herpeticum in a patient with Darier;s disease duringtreatment with etretinate / 대한피부과학회지

We report a case of eczema herpeticum associated with secondry staphylococcal infection in a 19 year-old male patient with Dariers disease during tratment with etretinate. Diagnosis was made by the history, clinical feature and histologic examination with light microscope & electron microscope. This patients condition was improved by the treatment with systemic and topical antibiotics within 1 week of admission.