Quality Indicators in Care of Patients With Atopic Dermatitis: Consensus Document of the Spanish Academy of Dermatology and Venereology. / Indicadores de calidad en la atención dermatológica a pacientes con dermatitis atópica. Documento de consenso de la Academia Española de Dermatología y Venereología.

No information is currently available on whether the available clinical practice guidelines on the management of atopic dermatitis are known or being applied in Spain. The aim of this study was to improve the care of patients with atopic dermatitis by developing a set of quality indicators based on existing clinical practice guidelines. Relevant clinical practice guidelines identified through a literature search were submitted to a panel of 11 specialists, who selected the highest quality guidelines using the AGREE (Appraisal of Guidelines for Research & Evaluation) II instrument. The panel then defined a subset of the recommendations supported by a high level of evidence and proposed a health care quality indicator for each one together with a standard for measuring degree of adherence. Consensus was achieved on 21 of the 150 proposed indicators using the modified Delphi method. The aim of implementing the indicators that achieved consensus in this study is to standardize the actions of health professionals providing care for patients with atopic dermatitis and ultimately to improve the quality of the care delivered.

Safe and effective use of alitretinoin in children with recalcitrant hand eczema and other dermatoses - a retrospective analysis.

BACKGROUND: Alitretinoin is a systemic retinoid licensed for use in adult patients suffering from chronic hand eczema recalcitrant to potent topical steroids. Experience with its use in childhood is lacking. OBJECTIVES: To report on the efficacy and safety of alitretinoin treatment in a cohort of children and adolescents with chronic hand eczema (CHE) and other inflammatory skin diseases. METHODS: We performed a retrospective chart review of all consecutive patients under the age of 18 years treated with alitretinoin at our paediatric skin centre. Physician's Global Assessment (PGA) was used as the primary outcome measure. RESULTS: Thirteen children (9 girls and 4 boys) were enrolled in this study. The median age at start of treatment with alitretinoin was 11.5 years (range 5.8-15.8 years). Nine children were diagnosed with CHE, two with severe atopic dermatitis (AD), and two with inherited ichthyosis [netherton syndrome (NS), autosomal recessive congenital ichthyosis (ARCI)]. Moderate to excellent response (PGA decrease of ≥1 point) was observed in 7 (78%) of the nine patients with CHE, one of the two patients with extensive AD and in the one patient with ARCI. In the remaining four subjects, no convincing effect was documented. Tolerability was overall very good. The most common adverse event was headache in 10 patients (77%) during the initiation of treatment, leading to interruption of therapy in one subject. CONCLUSIONS: Alitretinoin seems to be highly effective and safe for the treatment of paediatric CHE and should thus be considered in children with refractory disease under topical therapy. Larger studies are required to corroborate these findings.

A Retrospective Study of Systemic Treatment of Severe Atopic Dermatitis With Azathioprine: Effectiveness and Tolerance in 11 Pediatric Patients. / Estudio retrospectivo del tratamiento sistémico de la dermatitis atópica grave con azatioprina. Eficacia y tolerancia en 11 pacientes pediátricos.

BACKGROUND: Atopic dermatitis (AD) is a chronic inflammatory skin disease that typically affects children. Severe forms may have a profound effect on patients' quality of life. Some forms are resistant to conventional treatment and require the use of systemic immunosuppressants such as azathioprine (AZA) to adequately manage the disease. OBJECTIVE: To evaluate the effectiveness and tolerance of AZA in children with severe AD. PATIENTS AND METHODS: We performed a retrospective study of children with severe AD treated with AZA between January 2007 and May 2017. RESULTS: We reviewed the cases of 11 patients (6 boys and 5 girls) with a mean age of 13 years (range, 8-18 years). The mean (SD) age at start of treatment was 10.9 (2.2) years (95% CI 8.6-13.1). The mean initial dosage of AZA was 1.8 (0.2) mg/kg/d. We evaluated treatment response after 4 weeks, 12 to 16 weeks, and 6 months. Mean treatment duration was 10.8 (5.7) months. Treatment had to be suspended in 2 patients because of adverse effects. Seven of the 9 remaining patients presented complete or almost complete clearance of the AD after 6 months of treatment. CONCLUSION: In our experience, AZA is well tolerated and may be considered as a treatment option in children with severe AD resistant to conventional treatment.

Amelanotic naevoid melanoma in a 16-month-old albino infant.

Melanoma in individuals with oculocutaneous albinism has been reported in the literature to be rare compared with the more common occurrence of squamous cell carcinoma and basal cell carcinoma. We present a singular case of amelanotic naevoid melanoma arising from a small congenital naevus in a 16-month old albino boy, the youngest reported to date.

Extensive Acquired Telangiectasias: Comparison of Generalized Essential Telangiectasia and Cutaneous Collagenous Vasculopathy. / Telangiectasias extensas adquiridas: comparación entre telangiectasia esencial generalizada y vasculopatía colágena cutánea.

The late development of symmetrical, ascending telangiectasias over an extensive area of the skin with no associated systemic manifestations is a common presentation of generalized essential telangiectasia (GET). It was recently suggested that cutaneous collagenous vasculopathy (CCV) is clinically identical to GET but that the 2 conditions can be distinguished by their distinctive histopathologic findings. We present 2 patients, both women, with multiple telangiectasias and describe the histopathologic findings that led to the diagnoses of GET and CCV. Dermoscopic findings in both cases were similar, except that the older telangiectasias in the patient with CCV were violaceous and distributed in a tortuous, serpentine pattern. During follow-up 12 years for the woman with GET and 42 years for the woman with CCV we saw that in GET the lesions remained stable in appearance whereas in CCV there was progressive darkening and morphological changes eventually resulting in superficial varicose veins.

Dermoscopic Findings of Jellyfish Stings Caused by Pelagia noctiluca.

BACKGROUND AND OBJECTIVES: Jellyfish are free-living members of the phylum Cnidaria who share a specialized stinging cell, the cnidocyte. Pelagia noctiluca is the most frequent and toxic jellyfish species found in the Balearic beaches and cnidocytes are arranged in pigmented clusters called "warts". Dermoscopy continues to expand its use much beyond the pigmentary lesions and to date, there is no data regarding dermoscopic findings in jellyfish stings. The aim of the present work was to study the dermoscopic findings of jellyfish stings in the island of Mallorca. PATIENTS AND METHODS: We retrospectively reviewed the clinical and dermoscopic images of 25 episodes of jellyfish stings caused by P. noctiluca that occurred between 2009 and 2015. RESULTS: Overall, the following dermoscopic features were found: brown dots (84%), pinkish hue (56%), pinpoint brown crusts (44%), scale-crust (40%), brown "Chinese characters pattern" (32%), "serpentine" ulceration (28%), linear purpura (20%), and whitish-yellow crusts (15%). Vessels were mainly dotted (36%) or reticular (16%). Scale-crust, serpentine ulceration and pinkish hue were significantly more frequent in lesions older than 2 days. CONCLUSIONS AND LIMITATIONS: Our study identifies 4 dermoscopic features that may represent the contact with P. noctiluca cnidocytes: brown dots, brown "Chinese characters pattern", pinpoint brown crusts and whitish-yellow crusts. A peculiar finding of "serpentine ulceration" with brown dots would be very suggestive of P. noctiluca sting. We believe dermoscopy is a valuable tool in the diagnosis of jellyfish stings when a clear history of contact is lacking. Further studies are needed to validate our findings in other jellyfish species.

Otologic Manifestations of Autosomal Recessive Congenital Ichthyosis in Children.

BACKGROUND: Few studies have investigated ear involvement in nonsyndromic autosomal recessive congenital ichthyosis (ARCI). OBJECTIVES: To assess the type and frequency of otologic manifestations of ARCI in patients under follow-up at the pediatric dermatology department of our hospital. MATERIALS AND METHODS: We prospectively studied the presence of ear pain, ear itching, tinnitus, otitis, cerumen impaction, accumulation of epithelial debris, and hearing loss. Daily hygiene measures, topical treatments, medical-surgical interventions, and frequency of visits to an ear, nose, and throat (ENT) specialist were noted in the patients' medical records. Ear examination and hearing tests were performed in all cases. RESULTS: Ten patients were studied: 2 had a self-healing collodion baby phenotype and 8 had ichthyosis. There was mention of otologic manifestations in the records of all 8 patients with ichthyosis (100%); 6 of these patients (75%) had abnormalities in the external auditory canal examination and 2 (25%) had conductive hearing loss. Our findings are limited by the small number of patients studied, all of whom were younger than 19 years. CONCLUSIONS: The involvement of both dermatologists and ENT specialists in the management of patients with ichthyosis is crucial to ensure the application of the best therapeutic and preventive measures. More studies are needed to assess the prevalence and impact on quality of life of ear involvement in patients with ichthyosis and to determine the optimal interval between ENT visits for these patients.

Hypotrichosis associated with capillary malformation-arteriovenous malformation syndrome.

BACKGROUND: Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is associated with multifocal small CMs and a high risk for high-flow lesions. It is an autosomal dominant disorder, caused by RASA1 gene mutations. Recently, two novel clinical features have been identified: numerous small pale halos with central punctate red spots, and naevus anemicus. OBJECTIVES: To identify the prevalence of the new clinical manifestations in our patients with CM-AVM. The secondary objective was to investigate the presence of other skin lesions. METHODS: We retrospectively searched the picture database of our department for cases with a clinical diagnosis of CM-AVM, based on the identification of multiple cutaneous CMs and a negative history of epistaxis. We prospectively conducted a clinical and dermoscopic skin examination in all of these patients. RESULTS: Seven patients with multiple CMs were found, and only in one case was a cutaneous AVM present. Five patients had red punctate spots surrounded by pale halos on the upper limbs. Two adult patients also showed multiple telangiectasias on the neck and upper trunk. Naevus anemicus was not detected in any patient. A partial or total absence of vellous hair on the surface of CMs was observed in all patients. CONCLUSIONS: Red punctate spots with pale halos or small telangiectasias are frequent findings in CM-AVM syndrome. Hypotrichosis on the CMs suggests that RASA1 gene mutations could be involved in the hair follicle proliferation and cell cycle.