RESUMEN
The association of familial totalis leukonychia with multiple pilar cysts is a rare condition that could represent a separate syndromic entity. Since Bauer described a family with totalis leukonychia and sebaceous cysts in 1920, only four new affected families have been reported. We report a five-generation family with a total leukonychia and multiple pilar cysts on the scalp. The hypothesis of a deficiency of a gene regulating the structure of keratin has been postulated but the exact genetic mechanism has not been yet determined. In our family, no other keratinizing structures were involved.
Asunto(s)
Blefaritis/diagnóstico , Quiste Epidérmico/diagnóstico , Enfermedades del Cabello/diagnóstico , Hipopigmentación/diagnóstico , Enfermedades de la Uña/congénito , Trastornos de la Pigmentación/diagnóstico , Enfermedades de la Retina/diagnóstico , Adulto , Blefaritis/genética , Quiste Epidérmico/genética , Femenino , Predisposición Genética a la Enfermedad , Enfermedades del Cabello/genética , Humanos , Hipopigmentación/genética , Lactante , Masculino , Persona de Mediana Edad , Enfermedades de la Uña/diagnóstico , Enfermedades de la Uña/genética , Trastornos de la Pigmentación/genética , Enfermedades de la Retina/genéticaRESUMEN
BACKGROUND: Keratosis pilaris atrophicans (KPA) is a group of disorders characterized by erythematous keratotic papules followed by atrophy on the face. The treatment is often unsatisfactory. METHODS: Four white women, with ages ranging from 14 to 20 years, were treated with an intense pulsed light (IPL) system with a cut filter of 570 nm. The power density was between 40 and 47 J/cm(2), divided into two pulses of 3 ms, with a delay between both of 20 ms. Patients received five to nine sessions. RESULTS: Clinical improvement was noted in all patients, with a reduction of erythema in treated areas of between 75% and 100%. Treatment was well tolerated and no adverse reactions were observed. After a follow-up of 10 months no recurrence was observed. In addition, in parallel mode to erythema improvement, a reduction of roughness was observed. CONCLUSION: Our results suggest IPL should be considered as a safe treatment option in patients with KPA.
Asunto(s)
Dermatosis Facial/terapia , Queratosis/terapia , Fototerapia , Adolescente , Eritema/terapia , Femenino , Humanos , Adulto JovenRESUMEN
BACKGROUND: Arteriovenous haemangioma (AVH) is considered a rare, benign, acquired, cutaneous tumour of vascular origin. Recently, a variant associated with chronic hepatic disease has been described. The usual treatment is surgical resection but no other treatments have been reported. OBJECTIVE: To evaluate the results obtained in the treatment of AVH with carbon dioxide laser vaporization. METHODS: Every patient with AVH related to chronic hepatic disease treated in the laser unit was reviewed. Five patients were treated with carbon dioxide laser vaporization. The first pass of treatment was performed in defocused mode at 2 W/cm(2). After this, several passes were performed in order to clear the entire lesion. One session of treatment was necessary for three patients, and the other two patients needed two sessions. RESULTS: The tumoral mass as well as the pulse disappeared in all lesions; total clearance was obtained in four of the five cases. In the postoperative time, no bleeding or haemorrhage were observed. No significant secondary effects of treatment were present. The cosmetic outcome was excellent in all cases. CONCLUSION: Carbon dioxide laser vaporization may be an alternative treatment for cases of AVH in chronic hepatic disease: the procedure is easy and with good cosmetic outcome.