Descripción basal de la cohorte nacional prospectiva de hemangiomas infantiles de la Academia Española de Dermatología y Venereología. Comparación de los pacientes tratados con propranolol en la práctica clínica rutinaria y los datos del ensayo clínico pivotal previo / Baseline Description of the Spanish Academy of Dermatology Infantile Haemangioma Nationwide Prospective Cohort. Comparison of Patients Treated with Propranolol in Routine Clinical Practice with Previous Pivotal Clinical Trial Data

Introducción Existen diversas opciones terapéuticas para los hemangiomas infantiles (HI). El propranolol se utiliza con base en un ensayo pivotal. Nuestro objetivo fue describir las características del HI en la práctica clínica, incluyendo las terapias utilizadas, así como comparar las características de los pacientes tratados con propranolol y las de los pacientes del ensayo, para valorar su validez externa. Métodos Se incluyó consecutivamente a los pacientes que acudieron a doce hospitales españoles desde junio de 2016 a octubre de 2019 (n=601). Resultados La edad media fue de 3,9 (DE:1,9) meses, con una ratio mujer-varón de 2:1. La mayoría de los HI fueron de tipo localizado (82%, 495), superficial (64%, 383) y ubicados en cara (25%, 157) y tronco (31%, 188). El tamaño mediano fue de 17 (RI: 10-30) x 12 (RI: 7-20) mm. Se encontraron complicaciones en 16 (3%) pacientes. Se inició tratamiento en el 52% (311) de los casos. La mayoría de los pacientes recibió timolol (76%, 237), reservándose propranolol para las complicaciones o los HI de alto riesgo. El compromiso estético fue el principal motivo de iniciar la terapia (64%, 199). Las diversas características de los pacientes y de los HI tratados con propranolol fueron similares a las del ensayo clínico pivotal, aunque 1/3 de los HI no alcanzó el diámetro mínimo para cumplir los criterios de inclusión, y no se comunicó información pronóstica importante. Conclusiones Dado que muchos pacientes reciben tratamiento debido al compromiso estético, existe una necesidad de conocer mejor los resultados estéticos de las terapias e incrementar la evidencia sobre el uso de timolol, que actualmente es la terapia más común. El propranolol está siendo utilizado en una población generalmente similar a la del ensayo; sin embargo, esta afirmación no puede confirmarse de manera definitiva (AU)

Background There are several therapeutic options for infantile haemangiomas (IH). Propranolol is used according to a pivotal trial. We aimed to describe the characteristics of IH in clinical practice, including the therapies used, and to compare the characteristics of patients treated with propranolol with those of the trial to assess its external validity. Methods Consecutive patients attending 12 Spanish hospitals from June 2016 to October 2019 were included (n=601). Results The mean age was 3.9 (SD:1.9) months, with a 2:1 female-to-male ratio. Most IHs were localized (82%, 495), superficial (64%, 383) and located in the face (25%, 157) and trunk (31%, 188). Median size was 17 (IR: 10-30) x 12 (IR: 7-20) mm. Complications were found in 16 (3%) patients. Treatment was initiated for 52% (311). Most patients received timolol (76%, 237); propranolol was reserved for complications or high-risk IHs. Aesthetic impairment was the main reason for starting therapy (64%, 199). Several characteristics of the patients and IHs treated with propranolol are similar to those of the pivotal clinical trial, but 1/3 of IHs did not reach the minimum diameter to meet the inclusion criteria, and important prognostic information was not reported. Conclusions As most patients receive treatment for aesthetic impairment, there is a need to better understand the aesthetic results of therapies and to increase evidence on the use of timolol, which is currently the most common therapy. Propranolol is being used in a population generally similar to that of the trial; however, this statement cannot be definitely confirmed (AU)

Baseline description of the Spanish Academy of Dermatology infantile haemangioma nationwide prospective cohort. Comparison of patients treated with propranolol in routine clinical practice with previous pivotal clinical trial data. / Descripción basal de la cohorte prospectiva de hemangioma infantil a nivel nacional de la Asociación Española de Dermatología. Comparación de los pacientes tratados con propranolol en la práctica clínica rutinaria y los datos de un ensayo clínico pivotal previo.

BACKGROUND: There are several therapeutic options for infantile haemangiomas (IH). Propranolol is used according to a pivotal trial. We aimed to describe the characteristics of IH in clinical practice, including the therapies used, and to compare the characteristics of patients treated with propranolol with those of the trial to assess its external validity. METHODS: Consecutive patients attending 12 Spanish hospitals from June 2016 to October 2019 were included (n=601). RESULTS: The mean age was 3.9 (SD:1.9) months, with a 2:1 female-to-male ratio. Most IHs were localized (82%, 495), superficial (64%, 383) and located in the face (25%, 157) and trunk (31%, 188). Median size was 17 (IR: 10-30) x 12 (IR: 7-20) mm. Complications were found in 16 (3%) patients. Treatment was initiated for 52% (311). Most patients received timolol (76%, 237); propranolol was reserved for complications or high-risk IHs. Aesthetic impairment was the main reason for starting therapy (64%, 199). Several characteristics of the patients and IHs treated with propranolol are similar to those of the pivotal clinical trial, but 1/3 of IHs did not reach the minimum diameter to meet the inclusion criteria, and important prognostic information was not reported. CONCLUSIONS: As most patients receive treatment for aesthetic impairment, there is a need to better understand the aesthetic results of therapies and to increase evidence on the use of timolol, which is currently the most common therapy. Propranolol is being used in a population generally similar to that of the trial; however, this statement cannot be definitely confirmed.

Cutaneous infection caused by Aspergillus ustus, an emerging opportunistic fungus in immunosuppressed patients.

We report a case of primary cutaneous infection by the emerging fungus Aspergillus ustus in an immunosuppressed patient after a domestic accident. The patient failed to respond to itraconazole and died before receiving a new treatment with amphotericin B. There have been eight other cases reported since 1973, and only two patients survived the infection. In vitro susceptibility testing of seven antifungal drugs showed that terbinafine and the new azole derivative UR-9825 were the most active against this fungus.

Ofuji's papuloerythroderma following choledocholithiasis with secondary sepsis: complete resolution with surgery.

We describe a case of Ofuji's papuloerythroderma (PE) in a 72-year-old man with biliary sepsis induced by choledocholithiasis. The PE disappeared completely after surgery with no relapse. This aetiology for PE does not appear to have been described previously, while its resolution after treatment of the primary process supports the idea that it may be a reactive disorder of multifactorial origin.

Loose anagen hair in hypohidrotic ectodermal dysplasia.

We report a 4 year-old boy affected with hypohidrotic ectodermal dysplasia in whom loose anagen hair syndrome (LAHS) was suspected clinically. The diagnosis was confirmed by examination of hair by optic and scanning electron microscopy. Loose anagen hairs have not been previously described in the ectodermal dysplasias. It is possible that our patient had real LAHS or an ectodermal dysplasia with loose anagen hair as an epiphenomenon.

Papuloerythroderma of Ofuji: a report of 2 cases including the first European case associated with visceral carcinoma.

Papuloerythroderma (PE) is a rare type of erythroderma of the elderly which results from the coalescence of sheets of papules that spare skin folds, with peripheral eosinophilia in most cases. Skin biopsy shows a nonspecific eczematous pattern with a mature T cell lymphocytic and eosinophilic infiltrate with Langerhans cells in the dermis. We report 2 cases of PE. The 1st case, in which no underlying malignancy could be found, responded partially to oral etretinate, topical steroid creams and tar derivates. The 2nd case did not respond to high-dose oral steroids and was associated with adenocarcinoma of the colon, with a fatal outcome. PE has been associated with lymphoma and carcinoma. We report the first European case of PE associated with visceral carcinoma and review the cases reported in the western literature.

Cutaneous phaeohyphomycosis caused by Alternaria longipes in an immunosuppressed patient.

Alternaria longipes was reported as the agent of a cutaneous infection in a patient with a neoplastic disease. The fungus has not been reported previously as causing disease in humans. It was distinguished by its rather small conidia with smooth or slightly verruculose walls and a pale brown beak which rarely extended into a secondary conidiophore. In vitro inhibitory activities of amphotericin B, ketoconazole, itraconazole, and miconazole were shown.

Fluconazole-induced toxic epidermal necrolysis in a patient with human immunodeficiency virus infection.

Patients with human immunodeficiency virus (HIV) infection are prone to severe drug reactions, mainly from sulfonamides. We report the case of a 33-year-old male patient with HIV infection (group IV C-2 of CDC staging system) that developed a toxic epidermal necrolysis (TEN) affecting more than 70% of the body surface area and severe mucosal involvement after starting fluconazole for a recurrent oral thrush with dysphagia. This is to our knowledge the first reported case of TEN due to fluconazole.

[Kaposi's sarcoma associated with the acquired immunodeficiency syndrome. An analysis of 67 cases with a study of the prognostic factors]. / Sarcoma de Kaposi asociado a síndrome de inmunodeficiencia adquirida. Análisis de 67 casos con estudio de factores pronósticos.

The features and prognostic factors of 67 cases of Kaposi's sarcoma (KS) associated to the acquired immunodeficiency syndrome (AIDS) diagnosed at the Hospital Clinic, Barcelona, are analyzed. All the patients were male; mean age was 39.7 years, ranging from 22 and 62 years. 64 were homosexuals (95.5%), two were homosexual-drug addicts (3%) and one was drug addict (1.5%). Prevalence of cytomegalovirus and herpes virus infections were 91.1% and 89.5% respectively. In 42 cases (62.7%) KS was the initial AIDS presentation. The most common localization was the skin (89.5%), followed by the digestive tract (52.2%) and the lymph nodes (22.4%). Staging distribution was: 20 patients (29.8%) were in stage I, 11 patients (16.4%) in stage II, 7 patients (10.4%) in stage III, and 29 patients (43.2%) in stage IV. Constitutional symptoms associated to KS were found in 37 patients (55%). Overall 39 patients have already died, and the actuarial survival possibility of these 67 cases was 55% after 12 months. Univariant statistical analysis showed the presence of six variables with prognostic significance (p less than 0.05): staging, symptomatology, total white blood count, total lymphocyte count, T helper lymphocyte count and hemoglobin. Multivariant statistical analysis only chose the staging and symptomatology variables as independent (p less than 0.01 and p less than 0.001, respectively).

Solitary congenital nodular calcification of Winer located on the ear: report of two cases.

Solitary congenital nodular calcification of the skin is a form of cutaneous calcinosis. It appears as a small, firm nodule on the head and extremities of young infants. The histogenesis of this condition is controversial. We cared for two patients with the nodule on the ear.