Phakomatosis pigmentovascularis: Clinical findings in 15 patients and review of the literature.

INTRODUCTION: Phakomatosis pigmentovascularis (PPV) is a rare syndrome characterized by the association of a vascular nevus with an extensive pigmentary nevus. OBJECTIVE: We sought to study and evaluate clinical findings in patients with PPV referred to the laser department of our hospital. METHODS: We revised the clinical findings of 15 patients with PPV and reclassified them according to Happle's new classification. RESULTS: We studied 11 female patients and 4 male patients with a mean age of 21 years. Thirteen had phakomatosis cesioflammea, one cesiomarmorata, and one an unclassifiable form. Of 15 patients, 12 had nevus of Ota. The vascular involvement was extensive in our PPV population and 14 patients were affected in two or more areas. The mosaicism pattern in 13 patients was patchy and without a midline separation. The most frequent associations found were Sturge-Weber syndrome, Klippel-Trénaunay syndrome, and melanosis oculi. LIMITATIONS: Limitations include the methods of case collection, that this is a retrospective study, and that there were a relatively small number of patients. CONCLUSIONS: PPV are rare syndromes with a wide variability in their clinical expression. Most of the publications in the literature have only reported isolated cases.

Experience with photodynamic therapy in Hailey-Hailey disease.

Hailey-Hailey disease (HHD) is a rare genodermatosis that is often difficult to treat. This paper reports three patients with HHD treated with one session of photodynamic therapy (PDT) using topical methyl aminolevulinic acid applied under occlusion for 3 hours and red light at 37 J/cm2 for 7.5 minutes. Our results are not successful: all of the patients suffered discomfort during the 3-4 weeks following PDT and only one patient experienced clinical improvement. None of the patients would like to repeat the treatment. PDT is at an exploratory stage; further studies are necessary to determine whether PDT is useful in the treatment of HHD.

[Gefitinib-induced perforating dermatosis]. / Dermatosis perforante por gefitinib.

Gefitinib (Iressa) is a new antineoplastic agent that acts by selectively inhibiting epidermal growth factor receptor tyrosine kinase (EGFR-TK). It has shown activity against several solid tumors. Because of their action mechanism, gefitinib and other tyrosine kinase inhibitors have been associated with multiple cutaneous effects, most of which are mild and well tolerated. We present a case of perforating dermatosis after treatment with gefitinib.

[Treatment of acneiform rash by epidermal growth factor inhibitors with oral tetracyclines]. / Tratamiento de la erupción acneiforme por inhibidores del factor de crecimiento epidérmico con tetraciclinas orales.

INTRODUCTION: Epidermal growth factor inhibitors (EGFR) are new antineoplastic agents that are increasingly being developed. They are basically used as second line treatment in advanced stage tumors. Appearance of a facial acneiform rash in patients treated with these drugs is common and characteristic. The literature proposes multiple topical and systemic treatment options. Up to now, there is no clear evidence on any of them. PATIENTS AND METHODS: A descriptive study of 6 patients who were treated with 100 mg daily dose of doxycycline for 3 weeks was conducted. Clinical characteristics of the patients and treatment efficacy were analyzed. RESULTS: Five of the six patients achieved total resolution of the acneiform rash with this treatment. One patient achieved partial response. After long follow-up periods and in spite of following treatment with the EGFR inhibitors, no relapse was observed. CONCLUSIONS: Doxycycline is suggested as an effective treatment in this disease. Even though it is a short series, the results in our patients support this efficacy.

Dermatosis perforante por gefitinib / Gefitinib-induced perforating dermatosis

El gefitinib (Iressa®) es un nuevo agente antineoplásico que actúa inhibiendo selectivamente la tirosina cinasa del factor de crecimiento epidérmico (EGFR-TK). Ha demostrado actividad frente a varios tumores sólidos. Por su mecanismo de acción, el gefitinib y otros inhibidores de tirosina cinasa se han asociado a múltiples efectos cutáneos dermatológicos, la mayoría de ellos leves y bien tolerados. Se presenta un caso de dermatosis perforante tras tratamiento con gefitinib

Gefitinib (Iressa®) is a new antineoplastic agent that acts by selectively inhibiting epidermal growth factor receptor tyrosine kinase (EGFR-TK). It has shown activity against several solid tumors. Because of their action mechanism, gefitinib and other tyrosine kinase inhibitors have been associated with multiple cutaneous effects, most of which are mild and well tolerated. We present a case of perforating dermatosis after treatment with gefitinib

Tratamiento de la erupción acneiforme por inhibidores del factor de crecimiento epidérmico con tetraciclinas orales / Treatment of acneiform rash by epidermal growth factor inhibitors with oral tetracyclines

Introducción. Los inhibidores del factor de crecimiento epidérmico (EGFR) son unos nuevos agentes antineoplásicos en creciente desarrollo. Se utilizan fundamentalmente como tratamiento de segunda línea de tumores en estadios avanzados. La aparición de una erupción acneiforme facial en pacientes tratados con estos fármacos es frecuente y característica. La literatura propone múltiples opciones de tratamiento, tanto tópico como sistémico. Hasta el momento, no hay una evidencia clara sobre ninguno de ellos. Pacientes y métodos. Realizamos un estudio descriptivo de 6 pacientes que han sido tratados con doxiciclina en dosis de 100 mg diarios durante 3 semanas. Analizamos las características clínicas de los pacientes y la eficacia del tratamiento. Resultados. Cinco de los seis pacientes alcanzan la curación total de la erupción acneiforme con este tratamiento. Un paciente alcanza una respuesta parcial. Tras largos períodos de seguimiento, y a pesar de continuar el tratamiento con los inhibidores del EGFR, no se observa recaída. Conclusiones. La doxicilina se plantea como un tratamiento eficaz en esta patología. A pesar de ser una serie corta, los resultados en nuestros pacientes avalan dicha eficacia

Introduction. Epidermal growth factor inhibitors (EGFR) are new antineoplastic agents that are increasingly being developed. They are basically used as second line treatment in advanced stage tumors. Appearance of a facial acneiform rash in patients treated with these drugs is common and characteristic. The literature proposes multiple topical and systemic treatment options. Up to now, there is no clear evidence on any of them. Patients and methods. A descriptive study of 6 patients who were treated with 100 mg daily dose of doxyclycline for 3 weeks was conducted. Clinical characteristics of the patients and treatment efficacy were analyzed. Results. Five of the six patients achieved total resolution of the acneiform rash with this treatment. One patient achieved partial response. After long follow-up periods and in spite of following treatment with the EGFR inhibitors, no relapse was observed. Conclusions. Doxycycline is suggested as an effective treatment in this disease. Even though it is a short series, the results in our patients support this efficacy