Congenital cutaneous Langerhans cell histiocytosis and cutaneous mastocytoma in a child.

Langerhans cell histiocytosis and mastocytoma are clonal disorders of bone-marrow-derived cells, most commonly seen in the pediatric age. Infiltration of mast cells and Langerhans cells in the same lesion has been published before, but, to our knowledge, this is the first time that the occurrence of two mastocytomas and Langerhans cell histiocytosis is reported. It could be hypothesized that both clonal disorders of bone-marrow-derived cells could have a common origin.

Phototherapy in atopic dermatitis: a systematic review of the literature.

BACKGROUND: Phototherapy is a treatment option for atopic dermatitis recommended by several guidelines. OBJECTIVE: To perform a systematic review of the efficacy of different modalities of phototherapy and photochemotherapy in moderate to severe atopic dermatitis. MATERIAL AND METHODS: We considered all randomized clinical trials (RCTs) performed in patients with atopic dermatitis, and accepted all outcome measures. Articles were identified via an online search of the MEDLINE (via Ovid) and Embase databases and the Cochrane Central Register of Controlled Trials. We also searched for clinical trials registered in Current Controlled Trials and in the World Health Organization's International Clinical Trials Registry Platform. RESULTS: Twenty-one RCTs (961 patients) were included in the qualitative analysis. Two of the trials included children and adolescents (32 patients). The efficacy of narrow-band UV-B and UV-A1 phototherapy was similar for the different outcome measures contemplated. Two RCTs assessed the efficacy of psoralen plus UV-A therapy (PUVA). No serious adverse events were described. In general, the publications reviewed were characterized by a high risk of bias and poor reporting of methodology and results. CONCLUSIONS: There is evidence for the use of narrow-band UV-B and UV-A1 phototherapy in moderate to severe atopic dermatitis. Evidence supporting the use of PUVA in atopic dermatitis is scarce and there is little information on the use of phototherapy in childhood. For the purpose of future studies, it would be advisable to use comparable criteria and scales for the evaluation of disease severity and patients, to standardize radiation methods, and to establish a minimum follow-up time.

Skin infections caused by community-acquired methicillin-resistant Staphylococcus aureus: clinical and microbiological characteristics of 11 cases.

INTRODUCTION: Community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) is an emerging pathogen that causes skin and soft-tissue infections. OBJECTIVE: To describe the clinical characteristics of skin infections caused by CA-MRSA and correlations with the available demographic and microbiological data. MATERIAL AND METHODS: This was a descriptive study of patients with a microbiologically confirmed diagnosis of CA-MRSA infection treated in a dermatology department between June 2009 and December 2011. We recorded demographic details, the clinical characteristics of lesions, and the treatments used. RESULTS: We studied 11 patients (5 men and 6 women); 91% were under 40 years of age and had no relevant past medical history. The most common presentation was a skin abscess (with or without cellulitis). In all such cases, marked tissue necrosis and little or no purulent exudate was observed when the abscess was drained. Fifty percent of these abscesses had been treated previously with ß-lactam antibiotics, and in all cases the lesions resolved after surgical drainage, which was combined in 63% of cases with quinolones or cotrimoxazole. CONCLUSIONS: Today, skin infections due to CA-MRSA affect healthy young athletes who have no contact with healthcare settings. The most common presentation is a skin abscess characterized by marked tissue necrosis and little or no purulent exudate. In cases with these characteristics in susceptible patients, the involvement of CA-MRSA as the causative agent should be suspected. The abscesses should be drained whenever possible and, if necessary, antibiotic treatment should be prescribed; empirical use of ß-lactam antibiotics should be avoided.

[Dermatomyositis: assessment and follow-up of 20 patients]. / Dermatomiositis: estudio y seguimiento de 20 pacientes.

BACKGROUND: Dermatomyositis is an idiopathic inflammatory myopathy that mainly affects the skin and skeletal muscle. An estimated 15% to 25% of patients have underlying tumors and some forms are exclusively cutaneous. The factors that predict disease course and prognosis in these patients have not been clearly identified. Here we report our experience through the description and analysis of a series of patients. MATERIAL AND METHODS: This was a retrospective study of 20 patients with a diagnosis of dermatomyositis undergoing follow-up in the Department of Dermatology at Hospital General Universitario Gregorio Marañón in Madrid, Spain between February 2007 and February 2010. Clinical and histopathological characteristics were assessed alongside the results of laboratory tests and the treatments used. RESULTS: Nineteen of the 20 patients included in the study were women. The mean age was 61 years (median, 60 years). We identified 11 patients with classic, 3 with amyopathic, 2 with paraneoplastic, 1 with drug-associated, and 1 with juvenile dermatomyositis, and 2 patients had dermatomyositis associated with connective tissue disease. Heliotrope erythema, Gottron papules, and periungual erythema were the most frequent skin lesions. Cutaneous necrosis was present in 2 patients with paraneoplastic dermatomyositis. None of the patients had myositis-specific antibodies. Initial treatment was with systemic corticosteroids in 85% of cases. Eighty percent of patients required 2 or more drugs to achieve disease control. CONCLUSIONS: Dermatomyositis is a potentially serious disease. Dermatologists can facilitate diagnosis and contribute to the early detection of associated tumors and systemic complications. In most patients, the disease has a good prognosis, although extended periods of treatment may be required. Complications occur most commonly in patients with associated tumors or cardiopulmonary disease.

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.

[Erythrodermic lichen planus pemphigoides]. / Liquen plano penfigoide eritrodérmico.

Lichen planus pemphigoides is a clinico-histological subtype of lichen planus in which there are bullous lesions similar to those of bullous pemphigoid. Lichen planus is included among the rare causes of erythroderma. We present a case of erythrodermic lichen planus pemphigoides in a 49-year-old female patient who satisfactorily responded to treatment with cyclosporine. A detailed physical examination and immunofluorescence study are key to the diagnosis of lichen planus pemphigoides.