J.M. Hernanz

Hospital Universitario Infanta Leonor, Madrid, Madrid, Spain

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Publications (57)54.92 Total impact


  • No preview · Article · Apr 2014 · Clinical and Experimental Dermatology
  • D Velázquez · I Casado · P de la Cueva · J M Hernanz

    No preview · Article · Jul 2013 · Clinical and Experimental Dermatology
  • M. Valdivielso-Ramos · J.M. Hernanz
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    ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that is very rare in childhood. Only 6% of these tumors present in children. Clinical diagnosis is very difficult in the early stages of disease, but to ensure appropriate treatment it is important to identify DFSP as early as possible and rule out benign conditions that are more common at this age. The clinical presentation and histopathologic and molecular characteristics of DFSP are similar in children and adults. Clinical diagnosis is, however, more difficult in children and requires a high degree of suspicion. The absence of characteristic features and the rarity of this tumor explain why diagnosis is often delayed. Complete surgical excision of the tumor is very important to reduce the risk of recurrence. This article presents a review of current knowledge about the management of DFSP in children and examines the latest treatment options.
    No preview · Article · Dec 2012 · Actas Dermo-Sifiliográficas
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    ABSTRACT: Background Evaluation of disease severity is considered essential in the optimal management of psoriasis.Objectives To describe the clinical characteristics and therapeutic profile of patients with moderate to severe psoriasis in Spain and to assess the impact of the disease on the patients’ quality of life.Materials and methodsThis was an observational, cross-sectional study carried out in 90 dermatology units in Spain in 2009. We included 442 patients diagnosed with moderate to severe psoriasis who had started treatment with systemic agents, phototherapy, and/or topical treatments between 2004 and 2006.ResultsMore severe psoriasis was significantly associated with the following: longer disease duration; higher prevalence of concomitant disease; greater involvement of the nails, scalp, flexures, palms, and soles; and poorer quality of life. In the 5 years before the start of the study, 68% of the patients had received conventional systemic treatments, 39.1% biologic agents, and 22.3% phototherapy. At present, 57.5% of the patients are being treated with biologic agents, 32.6% with conventional systemic treatments, and 11% with phototherapy.Conclusions Severity of psoriasis was associated with a marked impact on quality of life. Regardless of disease severity, psychiatric comorbidity was the strongest predictor of poor quality of life. On average, patients had received other treatments, such as conventional systemic treatments or phototherapy, for more than 2 years before switching to biologic agents for the first time.
    No preview · Article · Dec 2012 · Actas Dermo-Sifiliográficas
  • M. Valdivielso-Ramos · J.M. Hernanz
    [Show abstract] [Hide abstract]
    ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that is very rare in childhood. Only 6% of these tumors present in children. Clinical diagnosis is very difficult in the early stages of disease, but to ensure appropriate treatment it is important to identify DFSP as early as possible and rule out benign conditions that are more common at this age.The clinical presentation and histopathologic and molecular characteristics of DFSP are similar in children and adults. Clinical diagnosis is, however, more difficult in children and requires a high degree of suspicion. The absence of characteristic features and the rarity of this tumor explain why diagnosis is often delayed. Complete surgical excision of the tumor is very important to reduce the risk of recurrence.This article presents a review of current knowledge about the management of DFSP in children and examines the latest treatment options.
    No preview · Article · Dec 2012 · Actas Dermo-Sifiliográficas
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    ABSTRACT: BACKGROUND: Evaluation of disease severity is considered essential in the optimal management of psoriasis. OBJECTIVES: To describe the clinical characteristics and therapeutic profile of patients with moderate to severe psoriasis in Spain and to assess the impact of the disease on the patients' quality of life. MATERIALS AND METHODS: This was an observational, cross-sectional study carried out in 90 dermatology units in Spain in 2009. We included 442 patients diagnosed with moderate to severe psoriasis who had started treatment with systemic agents, phototherapy, and/or topical treatments between 2004 and 2006. RESULTS: More severe psoriasis was significantly associated with the following: longer disease duration; higher prevalence of concomitant disease; greater involvement of the nails, scalp, flexures, palms, and soles; and poorer quality of life. In the 5 years before the start of the study, 68% of the patients had received conventional systemic treatments, 39.1% biologic agents, and 22.3% phototherapy. At present, 57.5% of the patients are being treated with biologic agents, 32.6% with conventional systemic treatments, and 11% with phototherapy. CONCLUSIONS: Severity of psoriasis was associated with a marked impact on quality of life. Regardless of disease severity, psychiatric comorbidity was the strongest predictor of poor quality of life. On average, patients had received other treatments, such as conventional systemic treatments or phototherapy, for more than 2 years before switching to biologic agents for the first time.
    No preview · Article · Nov 2012 · Actas Dermo-Sifiliográficas
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    ABSTRACT: Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous keratotic dermatosis of unknown origin. It has a bimodal distribution of age onset concentrating in the first and fifth decades. Classification of PRP includes 5 types: types I and II represent the forms of adult PRP, respectively, whereas types III, IV, and V are seen in juveniles. In recent years, a type VI PRP associated with HIV infection has been proposed. A universal standard treatment for PRP is lacking. Topical therapy is the treatment of choice for patients with mild type III and type IV PRP, including corticoids, vitamin D analogue or topical retinoids. Systemic therapy is reserved for patients with severe type III and type V. Acitretin and isotretinoin are the treatment of choice.
    No preview · Article · Sep 2012 · Acta pediátrica española
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    ABSTRACT: Background Psoriatic Arthritis (PsA) is a complex disease with a substantial genetic risk component (first-degree relative risk ∼55). Recently, Genomewide Association Studies (GWAS) have expanded the number of risk loci for Psoriasis (Ps) in >20 new loci. Objectives We have studied the association of Ps risk loci in PsA and purely cutaneous Ps (PsC). We have also analyzed the genetic association with several subphenotypes of clinical relevance. Methods Loci showing the strongest statistical evidence of association to Ps were selected (n=32). The SNP having the highest statistical evidence was genotyped using Taqman technology in a cohort of n=955 PsA, 1,050 PsC and 1,497 hypernormal controls of the Spanish population. According to each subphenotype variable, the genetic association was performed using the chi-square test, logistic regression or linear regression. Results We have replicated the association to COG6 and SERPINB8 loci with Ps for the first time in a Caucasian population. We have identified, for the first time, an association of PsA with variation at IFIH1, DPP6 and COG6. Analyzing the association with other clinically relevant subphenotypes we have identified a strong association of LCE3D locus with the severity of cutaneous affection. We have also found a significant association of IL1RN gene with nail disease. Conclusions Our findings show that common genetic variants associated to a complex phenotype like PsV influence PsA as well as different subphenotypes of high clinical relevance. Disclosure of Interest None Declared
    No preview · Article · Jun 2012 · Annals of the Rheumatic Diseases
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    ABSTRACT: Background Psoriatic Arthritis (PsA) is a complex disease and is present in ∼11% in patients with Psoriasis (Ps). Recently, Genomewide Association Studies (GWAS) have expanded the number of risk loci for Ps in >20 new loci. Two of these genes, ERAP-1 and HLA-C, have been shown to interact epistatically in the risk to develop Ps. Objectives We have studied the presence of new genetic interactions between Ps risk lci with the HLA region in Purely cutaneous Psoriasis (PsC) and PsA. Methods Within each Ps risk locus (n=31), the SNP having the highest statistical evidence was selected. The 31 SNPs were genotyped using Taqman technology in a cohort of n=955 PsA, 1,050 PsC and 1,497 hypernormal controls of the Spanish population. The presence of statistically significant gene-gene interactions was performed using a logistic regression model with three parameters to determine the presence of interaction at the allelic level. Results We replicated the previously described interaction of HLA-C and ERAP1 in the PsC cohort but not in the PsA cohort. We identified, for the first time, a significative epistatic association between HLA-C and SERPINB8. Microarray gene expression studies on cutaneous biopsies corroborate the presence of this interaction at a functional level. In PsA, no statistically significant interactions where identified with variation at HLA-C. However, 6 of the studied genes showed a significant (P<0.05) association with HLA-B27 positivity. Conclusions The present study has identified new genetic interactions associated with the risk to develop PsC and PsA. The functional study of these interactions will improve our knowledge of the biological basis of these complex diseases. Disclosure of Interest None Declared
    No preview · Article · Jun 2012 · Annals of the Rheumatic Diseases
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    ABSTRACT: We report the case of a 6-year-old child with hyperpigmented lesions in perioral region. His father had been diagnosed of Peutz-Jeghers syndrome (PJS) by genetic testing. PJS is a rare entity characterized by the presence of hyperpigmented periorificial lesions and gastrointestinal polyps. Histologically, these polyps are hamartomas that can become malignant. Moreover, PJS is associated with the development of nongastrointestinal cancer (breast, endometrium, ovary, testicle, pancreas...). It is therefore necessary to make an early diagnosis and periodic monitoring of the patients with this syndrome and their families.
    No preview · Article · May 2012 · Acta pediátrica española
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    ABSTRACT: Molluscum contagiosum is a common skin infection, caused by a poxvirus, that affect mainly children. The disease can be transmitted by direct contact, fomites, or auto-inoculation. The infection will usually resolve within months or years in people with normal immunity. There has been a continous discussion about whether physicians should treat Molluscum contagiosum actively or not.
    No preview · Article · Apr 2012 · Acta pediátrica española
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    ABSTRACT: Tinea corporis is a superficial infection caused by dermatophytes. It is the second most common tinea in childhood after the ringworm of the scalp. It may spread and cause distant lesions if not treated on time. Pets are usually on the origin of the infection. Topical treatment is sufficient except when multiple lesions appear or in case of involvement of hairy areas. In those circumstances, oral treatment is appropiate. Griseofulvin and terbinafine are the most commonly used systemic treatments.
    No preview · Article · Mar 2012 · Acta pediátrica española
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    ABSTRACT: Uncombable hair syndrome is a rare anomaly of the hair shaft that results in a disorganized, unruly hair pattern that it is impossible to comb flat. This condition has a characteristic longitudinal groove along the hair shaft and a triangular cross-section.
    No preview · Article · Feb 2012 · Acta pediátrica española
  • Article: Piebaldism
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    ABSTRACT: Piebaldism is an autosomal dominant rare disease characterized by the presence since birth of poliosis and congenital depigmentation white patches on the mid-forehead, chest, abdomen and extremities, where no melanocytes are found. It has been linked to inactivating mutations or deletions of the c-Kit gene. These mutations result in a decrease of the receptor tyrosine kinase signalling. These mutations do not allow the correct development of the melanoblast and the posterior migration from the neural crest to a definitive site.
    No preview · Article · Nov 2011

  • No preview · Article · Oct 2011 · Actas Dermo-Sifiliográficas
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    ABSTRACT: Bullous pemphigoid is an acquired autoimmune blistering disorder extremely uncommon in children, characterized by circulating IgG antibodies to antigens of the epidermal basement membrane zone. In general, the clinical course of this condition is good and relapses are rare. The early diagnosis and treatment are fundamental.We present a 3-month-old girl with a blistering eruption on her palms and soles, and urticarial plaques on trunk, and face, 3 weeks after vaccine at two months (hepatitis B, diphtheria, tetanus, pertussis, polio, Haemophilus influenzae B, meningococcal C, pneumococcus). The clinical course worsened with vaccinations at 4 and 6 months. The control of lesions was achieved with oral deflazacort 1mg/kg/day, with a gradual decrease until 3 months of therapy. The patient is still in remission after 8 months of follow-up.Bullous pemphigoid has been connected with some drugs and vaccinations, 1 day to 4 weeks after receiving immunization. Although the exact mechanism of induction is unclear, this case report has a visible relationship with vaccinations.
    No preview · Article · Sep 2011 · Anales de Pediatría

  • No preview · Article · Sep 2011 · Actas Dermo-Sifiliográficas
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    ABSTRACT: The precalcaneal congenital fibrolipomatous hamartoma is a benign condition of infancy present at birth or appearing a few months later, as a solitary nodule, unilateral or most commonly bilateral and symmetrical in the medial posterior region of the heels. They are usually asymptomatic and not associated with any other alterations. Usually no treatment is required.
    No preview · Article · Jul 2011 · Acta pediátrica española
  • [Show abstract] [Hide abstract]
    ABSTRACT: Bullous pemphigoid is an acquired autoimmune blistering disorder extremely uncommon in children, characterized by circulating IgG antibodies to antigens of the epidermal basement membrane zone. In general, the clinical course of this condition is good and relapses are rare. The early diagnosis and treatment are fundamental. We present a 3-month-old girl with a blistering eruption on her palms and soles, and urticarial plaques on trunk, and face, 3 weeks after vaccine at two months (hepatitis B, diphtheria, tetanus, pertussis, polio, Haemophilus influenzae B, meningococcal C, pneumococcus). The clinical course worsened with vaccinations at 4 and 6 months. The control of lesions was achieved with oral deflazacort 1 mg/kg/day, with a gradual decrease until 3 months of therapy. The patient is still in remission after 8 months of follow-up. Bullous pemphigoid has been connected with some drugs and vaccinations, 1 day to 4 weeks after receiving immunization. Although the exact mechanism of induction is unclear, this case report has a visible relationship with vaccinations.
    No preview · Article · Jun 2011 · Anales de Pediatría

  • No preview · Article · Apr 2011 · Actas Dermo-Sifiliográficas

Publication Stats

345 Citations
54.92 Total Impact Points

Institutions

  • 2009-2014
    • Hospital Universitario Infanta Leonor
      Madrid, Madrid, Spain
  • 2010
    • Hospital Universitario de La Princesa
      • Servicio de Dermatología
      Madrid, Madrid, Spain
  • 2007-2009
    • Hospital General Universitario Gregorio Marañón
      • • Department of Dermatology
      • • Servicio de Dermatología
      Madrid, Madrid, Spain