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1.
Pediatr Dermatol ; 2024 Feb 02.
Article En | MEDLINE | ID: mdl-38305078

Mast cells (MCs) can release a variety of biologically active mediators under different circumstances, such as fever or vaccination. Our aim was to evaluate the incidence and severity of MC activation symptoms induced by SARS-CoV-2 virus (COVID-19) infection and vaccination in a cohort of 92 pediatric patients with cutaneous mastocytosis. Our findings support previous evidence on the safety of COVID-19 infection and vaccination in patients with MC disorders.

6.
Pediatr Dermatol ; 39(4): 557-562, 2022 Jul.
Article En | MEDLINE | ID: mdl-35504688

BACKGROUND: Guidelines and expert recommendations on infantile hemangiomas (IH) are aimed at increasing homogeneity in clinical decisions based on the risk of sequelae. OBJECTIVE: The objective was to analyze the inter- and intra-observer agreement among pediatric dermatologists in the choice of treatment for IH. METHODS: We performed a cross-sectional inter-rater and intra-rater agreement study within the Spanish infantile hemangioma registry. Twenty-seven pediatric dermatologists were invited to participate in a survey with 50 clinical vignettes randomly selected within the registry. Each vignette contained a picture of an infantile hemangioma with a clinical description. Raters chose therapy among observation, topical timolol, or oral propranolol. The same survey reordered was completed 1 month later to assess intra-rater agreement. Vignettes were stratified into hemangioma risk categories following the Spanish consensus on IH. The agreement was measured using kappa statistics appropriate for the type of data (Gwet's AC1 coefficient and Gwet's paired t test). RESULTS: Twenty-four dermatologists completed the survey. Vignettes represented 7.8% of the Spanish hemangioma registry. The inter-rater agreement on the treatment decision was fair (AC1  = 0.39, 95% confidence interval [CI]: 0.30-0.47). When stratified by risk category, good agreement was reached for high-risk hemangiomas (AC1  = 0.77, 95% CI: 0.51-1.00), whereas for intermediate- and low-risk categories, the agreement was only fair (AC1 0.31, 95% CI: 0.16-0.46 and AC1  = 0.38, 95% CI: 0.27-0.48, respectively). Propranolol was the main option for high-risk hemangiomas (86.4%), timolol for intermediate-risk (36.8%), and observation for low-risk ones (55.9%). The intra-rater agreement was good. The inter-rater agreement between pediatric dermatologists on the treatment of IH is only fair. Variability was most significant with intermediate- and low-risk hemangiomas.


Hemangioma, Capillary , Hemangioma , Child , Cross-Sectional Studies , Dermatologists , Hemangioma/drug therapy , Humans , Observer Variation , Pediatrics , Propranolol/therapeutic use , Spain , Timolol/therapeutic use
8.
Pediatr Dermatol ; 36(6): 899-901, 2019 Nov.
Article En | MEDLINE | ID: mdl-31468572

Segmental infantile hemangiomas related to PHACE syndrome have recently been associated with enamel hypoplasia. We present two cases of solitary, localized upper lip infantile hemangioma with enamel hypoplasia of deciduous teeth.


Aortic Coarctation/complications , Dental Enamel Hypoplasia/etiology , Eye Abnormalities/complications , Hemangioma/etiology , Lip Neoplasms/etiology , Neurocutaneous Syndromes/complications , Female , Hemangioma/drug therapy , Humans , Infant , Lip Neoplasms/drug therapy
9.
J Am Acad Dermatol ; 80(2): 508-515, 2019 Feb.
Article En | MEDLINE | ID: mdl-30296533

BACKGROUND: The superficial lymphatic component of vascular malformations poses a significant treatment challenge. It is responsible for the majority of symptoms presented, and to date, there is no consensus regarding treatment. OBJECTIVE: To evaluate the effectiveness of topical rapamycin in treating superficial lymphatic malformations (LM). METHODS: A case series study was performed of patients with superficial LM, treated with topical rapamycin. The clinical characteristics of patients and the concentration and application mode of the drug were recorded. The changes in the signs and symptoms observed and associated adverse effects were noted and analyzed. RESULTS: The study population consisted of 11 patients of an average age of 10.5 years. All were treated with topical rapamycin: 6 patients with a 1% concentration, 1 with a 0.8% concentration, and 4 with a 0.4% concentration. Changes in the clinical appearance of the lesions were observed in all patients. The associated symptoms, present in 9 of 11 patients, improved in every case. The mean follow-up time was 16.1 months. LIMITATIONS: This study is retrospective, with a small sample size and considerable heterogeneity of lesions and treatment approaches. CONCLUSION: Treatment with topical rapamycin modifies the clinical appearance and alleviates symptoms of superficial LM.


Immunosuppressive Agents/therapeutic use , Lymphatic Abnormalities/diagnosis , Lymphatic Abnormalities/drug therapy , Sirolimus/therapeutic use , Administration, Topical , Adolescent , Child , Child, Preschool , Cohort Studies , Female , Humans , Male , Prognosis , Retrospective Studies , Severity of Illness Index , Treatment Outcome
13.
An. bras. dermatol ; 91(5,supl.1): 111-113, Sept.-Oct. 2016. graf
Article En | LILACS | ID: biblio-837914

Abstract Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus. It consists of small papules localized exclusively on the back of the hands, wrists and extensor aspects of distal forearms with no other clinical or laboratory manifestations. The lesions tend to persist and may increase slowly in number. Histologically, hematoxylin-eosin and Alcian blue staining demonstrate mucin accumulation in the upper reticular dermis with separation of collagen fibers as a result of hyaluronic acid deposition. Treatment is rarely necessary due to the absence of symptoms. We present a 27-year-old healthy woman with asymptomatic papules on her upper extremities, which adequately meet clinical and pathological criteria of acral papular mucinosis.


Humans , Female , Adult , Skin/pathology , Skin Diseases/pathology , Scleromyxedema/pathology , Biopsy , Upper Extremity , Rare Diseases , Mucins/analysis
14.
Rev. clín. med. fam ; 9(3): 246-249, oct. 2016. ilus
Article Es | IBECS | ID: ibc-159616

La psoriasis es una enfermedad cutánea crónica caracterizada más frecuentemente por placas eritematosas que aparecen normalmente con una distribución característica en superficie de extensión de extremidades, zonas de presión y cuero cabelludo. Aunque el diagnóstico diferencial de la psoriasis es amplio, el reconocimiento de esta entidad puede ser realizado mediante una correcta anamnesis y exploración física en la mayoría de los casos. La psoriasis folicular es una variante infradiagnosticada entre las diversas formas de psoriasis bien documentadas en la bibliografía (AU)


Psoriasis is a chronic skin disorder most commonly characterized by well-demarcated erythematous plaques with a typical distribution on extensor areas of limbs, pressure areas and scalp. Although the differential diagnosis of psoriasis is broad, its identification can be made by means of careful anamnesis and physical examination in the vast majority of cases. Follicular psoriasis is an under-diagnosed variant among the other well documented forms of psoriasis (AU)


Humans , Male , Female , Child , Psoriasis/diagnosis , Psoriasis/drug therapy , Skin Diseases/diagnosis , Skin Diseases/drug therapy , Pityriasis/diagnosis , Pityriasis/drug therapy , Pityriasis/physiopathology , Pityriasis Rubra Pilaris/complications , Pityriasis Rubra Pilaris/drug therapy , Adrenal Cortex Hormones/therapeutic use , Cyclosporine/therapeutic use , Primary Health Care/methods , Betamethasone/therapeutic use , Fluticasone/therapeutic use , Keratoderma, Palmoplantar/drug therapy , Diagnosis, Differential
15.
An Bras Dermatol ; 91(5 suppl 1): 111-113, 2016.
Article En | MEDLINE | ID: mdl-28300913

Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus. It consists of small papules localized exclusively on the back of the hands, wrists and extensor aspects of distal forearms with no other clinical or laboratory manifestations. The lesions tend to persist and may increase slowly in number. Histologically, hematoxylin-eosin and Alcian blue staining demonstrate mucin accumulation in the upper reticular dermis with separation of collagen fibers as a result of hyaluronic acid deposition. Treatment is rarely necessary due to the absence of symptoms. We present a 27-year-old healthy woman with asymptomatic papules on her upper extremities, which adequately meet clinical and pathological criteria of acral papular mucinosis.


Scleromyxedema/pathology , Skin Diseases/pathology , Skin/pathology , Adult , Biopsy , Female , Humans , Mucins/analysis , Rare Diseases , Upper Extremity
16.
Rev. clín. med. fam ; 8(2): 162-165, jun. 2015. ilus
Article Es | IBECS | ID: ibc-140657

Presentamos un paciente de 13 años referido a la consulta de Dermatología por una lesión indurada, pigmentada, lineal en miembro inferior derecho. Tras su valoración clínica y con sospecha diagnóstica de morfea, se realizaron las exploraciones pertinentes para confirmar el diagnóstico e indicar el tratamiento específico (AU)


We present a 13-year-old patient referred to the Dermatology consultation due to a lineal, indurated, pigmented lesion on the right lower limb. After clinical assessment and suspected diagnosis of morphea, relevant examinations were performed to confirm the diagnosis and to indicate the specific treatment (AU)


Adolescent , Humans , Male , Connective Tissue , Connective Tissue/pathology , Scleroderma, Localized/complications , Scleroderma, Localized/diagnosis , Adrenal Cortex Hormones/therapeutic use , Scleroderma, Localized/drug therapy , Scleroderma, Localized/physiopathology , Antibodies, Antinuclear , Bromocriptine/therapeutic use , Pentazocine/therapeutic use
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