IntroducciónLos pacientes con psoriasis y hepatopatías son un reto terapéutico dado el mayor riesgo de efectos adversos que presentan con el tratamiento sistémico clásico y a que, aunque los fármacos anti-TNF-α son considerados más seguros, también se han descrito reactivaciones de hepatitis vírica y hepatitis tóxica en relación con los mismos. El ustekinumab (UTK) tiene un mecanismo de acción diferente y se sabe poco de sus efectos en el hígado, sobre todo a partir de los estudios pivotales. Nuestro objetivo ha sido estimar la incidencia de toxicidad hepática en los pacientes tratados con UTK en la práctica clínica habitual, y examinar los cambios hepáticos en aquellos pacientes tratados que tenían hepatopatía de base.MétodoSe incluyeron todos los pacientes tratados con UTK según pauta habitual. Se analizaron la edad, el género, el tipo de psoriasis, la afectación ungueal, la artritis, los tratamientos previos, la hepatopatía previa, las serologías virales y el PASI (basal y a las 12, 16 y 52 semanas). Se analizaron también las transaminasas, los signos y síntomas de hepatopatía y los factores como el índice de masa corporal, el hábito enólico, la ferritina y la ecografía hepática.ResultadosSe observaron hipertransaminasemias de grado 1 en solo 6 pacientes. Ninguno presentó hipertransaminasemias graves. Ninguno de los pacientes con hipertransaminasemia basal tuvo problemas durante el tratamiento.ConclusionesLa hepatotoxicidad asociada a UTK es poco frecuente y grave, y parece seguro a nivel hepático, incluso en pacientes con hepatopatía basal o que habían presentado con anterioridad alteraciones hepáticas con otros fármacos.

INTRODUCTION:The therapy of patients with psoriasis and liver disease can be a challenge due to the increased risk of adverse effects from traditional systemic treatments; in addition, although the anti-tumor necrosis factor agents are considered safer, they have also been associated with drug-induced liver injury and reactivation of viral hepatitis. Ustekinumab has a different mechanism of action and the little that is known of its effects on the liver comes from pivotal studies. The objectives of this study were to estimate the incidence of drug-induced liver injury in patients treated with ustekinumab in daily clinical practice and to analyze liver alterations in those patients with pre-existing liver disease.METHOD:All patients treated with the standard regimen of ustekinumab were included in the study. Variables gathered included age, sex, type of psoriasis, nail involvement, arthritis, previous treatments, history of liver disease, viral serology, Psoriasis Area Severity Index (at baseline and at 12, 16, and 52 weeks), transaminase levels, manifestations of liver disease, liver ultrasound, and factors such as body mass index, alcohol consumption, and ferritin levels.RESULTS:Grade 1 elevation of the transaminases was only observed in 6 patients; no cases of severe hypertransaminasemia were observed. None of the patients with elevation of the transaminases at baseline developed problems during treatment.CONCLUSIONS:Ustekinumab-related liver injury is uncommon and mild. From a hepatic point of view, the drug appears safe, even in patients with pre-existing liver disease and those who have developed altered liver function previously with other drugs.

Journal Article Brunsting–Perry‐type cicatricial pemphigoid with IgG autoantibodies to LAD‐1 Get access P. García‐Martín, P. García‐Martín Department of Dermatology Hospital Universitario de La Princesa C/Diego de León 62 28006 Madrid Spain Search for other works by this author on: Oxford Academic Google Scholar J. Fraga, J. Fraga Department of Pathology Hospital Universitario de La Princesa C/Diego de León 62 28006 Madrid Spain Search for other works by this author on: Oxford Academic Google Scholar T. Hashimoto, T. Hashimoto Department of Dermatology Kurume University School of Medicine Fukuoka Japan Search for other works by this author on: Oxford Academic Google Scholar A. García‐Diez A. García‐Diez Department of Dermatology Hospital Universitario de La Princesa C/Diego de León 62 28006 Madrid Spain Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 170, Issue 3, 1 March 2014, Pages 743–745, https://doi.org/10.1111/bjd.12677 Published: 01 March 2014

OBJECTIVE:To define and characterize the progression of the spontaneous autoimmune disease that develops in mice in the absence of the leukocyte adhesion receptor P-selectin glycoprotein ligand 1 (PSGL-1).METHODS:Skin-resident immune cells from PSGL-1-deficient mice and C57BL/6 control mice of different ages were isolated and analyzed by flow cytometry. Biochemical parameters were analyzed in mouse serum and urine, and the presence of serum autoantibodies was investigated. Skin and internal organs were extracted, and their structure was analyzed histologically.RESULTS:Skin-resident innate and adaptive immune cells from PSGL-1(-/-) mice had a proinflammatory phenotype with an imbalanced T effector cell:Treg cell ratio. Sera from PSGL-1(-/-) mice had circulating autoantibodies commonly detected in connective tissue-related human autoimmune diseases. Biochemical and histologic analysis of skin and internal organs revealed skin fibrosis and structural and functional abnormalities in the lungs and kidneys. Furthermore, PSGL-1(-/-) mice exhibited vascular alterations, showing loss of dermal vessels, small vessel medial layer remodeling in the lungs and kidneys, and ischemic processes in the kidney that promote renal infarcts.CONCLUSION:Our study demonstrates that immune system overactivation due to PSGL-1 deficiency triggers an autoimmune syndrome with characteristics similar to systemic sclerosis, including skin fibrosis, vascular alterations, and systemic organ involvement. These results suggest that PSGL-1 expression contributes to the maintenance of the homeostasis of the immune system and could act as a barrier for autoimmunity in mice.

Photodermatoses are skin conditions that are induced or exacerbated by electromagnetic radiation (including visible light, UV light, and infrared radiation) from the sun or artificial light sources. In Part 1 of this series we review current understanding of the pathophysiology of these processes and their classification. We also discuss technical aspects and the basic physics of photobiology and describe the equipment required for photobiologic testing and calibration (light sources and measurement instruments). (C) 2012 Elsevier Espana, S.L. and AEDV. All rights reserved.

Journal of the European Academy of Dermatology and VenereologyVolume 28, Issue 12 p. 1830-1832 Letter to the Editor Neutrophilic eccrine hidradenitis in a patient with Crohn's disease and azathioprine hypersensitivity syndrome P. García-Martín, Corresponding Author P. García-Martín Dermatology Department, Hospital Universitario De La Princesa, Madrid, SpainCorrespondence: P. García-Martín. E-mail: [email protected]Search for more papers by this authorJ. Sánchez-Pérez, J. Sánchez-Pérez Dermatology Department, Hospital Universitario De La Princesa, Madrid, SpainSearch for more papers by this authorJ. Fraga, J. Fraga Pathology Department, Hospital Universitario De La Princesa, Madrid, SpainSearch for more papers by this authorA. García-Diez, A. García-Diez Dermatology Department, Hospital Universitario De La Princesa, Madrid, SpainSearch for more papers by this author P. García-Martín, Corresponding Author P. García-Martín Dermatology Department, Hospital Universitario De La Princesa, Madrid, SpainCorrespondence: P. García-Martín. E-mail: [email protected]Search for more papers by this authorJ. Sánchez-Pérez, J. Sánchez-Pérez Dermatology Department, Hospital Universitario De La Princesa, Madrid, SpainSearch for more papers by this authorJ. Fraga, J. Fraga Pathology Department, Hospital Universitario De La Princesa, Madrid, SpainSearch for more papers by this authorA. García-Diez, A. García-Diez Dermatology Department, Hospital Universitario De La Princesa, Madrid, SpainSearch for more papers by this author First published: 03 February 2014 https://doi.org/10.1111/jdv.12380Citations: 7Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. References 1Bachmeyer C, Aractingi S. Neutrophilic eccrine hidradenitis. Clin Dermatol 2000; 18: 319–330. 2Mendoza JL, García-Paredes J, Peña AS, Cruz-Santamaría DM, Iglesias C. Díaz Rubio M. A continuous spectrum of neutrophilic dermatoses in Crohn's disease. Rev Esp Enferm Dig 2003; 95: 229–232. 3Bidinger JJ, Sky K, Battafarano DF, Henning JS. The cutaneous and systemic manifestations of azathioprine hypersensitivity syndrome. J Am Acad Dermatol 2011; 65: 184–191. 4Choonhakarn C, Chaowattanapanit S. Azathioprine-induced Sweet's syndrome and published work review. J Dermatol 2013; 40: 267–271. 5El-Azhary RA, Brunner KL, Gibson LE. Sweet syndrome as a manifestation of azathioprine hypersensitivity. Mayo Clin Proc 2008; 83: 1026–1030. Citing Literature Volume28, Issue12December 2014Pages 1830-1832 ReferencesRelatedInformation

The second of this series describes the characteristics of 3 types of photobiologic studies: the light test, the photochallenge test, and the photopatch test. We explain how the tests are carried out, the expected results, and their clinical usefulness in various photodermatoses. These tests are needed before attempting to induce adaptation (skin hardening or light tolerance) in the most debilitating cases.

Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms, typically lymphoproliferative disorders. PNP is characterized clinically by painful erosive stomatitis and polymorphous skin lesions. Histopathological findings are also very varied, and include lichen planus-like and pemphigus-like changes. These polymorphic clinicopathological findings are probably due to the complex pathogenic mechanism, in which both cellular and humoral immunity are implicated. Eosinophilic spongiosis, although infrequent, can be found with pemphigus herpetiformis and bullous pemphigoid, although this association has not been established in PNP. The presence of autoantibodies against envoplakin and periplakin in PNP has been reported, but autoantibodies against desmocollins (Dscs) have been found in only a very few cases of PNP, probably due to the lack of studies on such associations. We report the first case, to our knowledge, of PNP with eosinophilic spongiosis as the initial histopathological finding, and presence of autoantibodies to Dsc2 and Dsc3.

Journal of the European Academy of Dermatology and VenereologyVolume 28, Issue 7 p. 979-981 Letter to the Editor Multiple eruptive dermatofibromas related to imatinib treatment M. Llamas-Velasco, Corresponding Author M. Llamas-Velasco Department of Dermatology, Hospital Universitario de La Princesa, Madrid, SpainCorrespondence: M. Llamas Velasco. E-mail: [email protected]Search for more papers by this authorJ. Fraga, J. Fraga Department of Pathology, Hospital Universitario de La Princesa, Madrid, SpainSearch for more papers by this authorG.E. Solano-López, G.E. Solano-López Department of Dermatology, Hospital Universitario de La Princesa, Madrid, SpainSearch for more papers by this authorJ.L. Steegmann, J.L. Steegmann Department of Hematology, Hospital Universitario de La Princesa, Madrid, SpainSearch for more papers by this authorA. García Diez, A. García Diez Department of Dermatology, Hospital Universitario de La Princesa, Madrid, SpainSearch for more papers by this authorL. Requena, L. Requena Department of Dermatology, Fundación Jimenez Diaz, Madrid, SpainSearch for more papers by this author M. Llamas-Velasco, Corresponding Author M. Llamas-Velasco Department of Dermatology, Hospital Universitario de La Princesa, Madrid, SpainCorrespondence: M. Llamas Velasco. E-mail: [email protected]Search for more papers by this authorJ. Fraga, J. Fraga Department of Pathology, Hospital Universitario de La Princesa, Madrid, SpainSearch for more papers by this authorG.E. Solano-López, G.E. Solano-López Department of Dermatology, Hospital Universitario de La Princesa, Madrid, SpainSearch for more papers by this authorJ.L. Steegmann, J.L. Steegmann Department of Hematology, Hospital Universitario de La Princesa, Madrid, SpainSearch for more papers by this authorA. García Diez, A. García Diez Department of Dermatology, Hospital Universitario de La Princesa, Madrid, SpainSearch for more papers by this authorL. Requena, L. Requena Department of Dermatology, Fundación Jimenez Diaz, Madrid, SpainSearch for more papers by this author First published: 10 December 2013 https://doi.org/10.1111/jdv.12328Citations: 13Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. References 1Ammirati CT, Mann C, Hornstra IK. Multiple eruptive dermatofibromas in three men with HIV infection. Dermatology 1997; 195: 344–348. 10.1159/000245985 CASPubMedWeb of Science®Google Scholar 2Tsunemi Y, Tada Y, Saeki H, Ihn H, Tamaki K. Multiple dermatofibromas in a patient with systemic lupus erythematosus and Sjogren's syndrome. Clin Exp Dermatol 2004; 29: 483–485. 10.1111/j.1365-2230.2004.01574.x CASPubMedWeb of Science®Google Scholar 3Huang PY, Chu CY, Hsiao CH. Multiple eruptive dermatofibromas in a patient with dermatomyositis taking prednisolone and methotrexate. J Am Acad Dermatol 2007; 57: S81–S84. 10.1016/j.jaad.2006.05.070 PubMedWeb of Science®Google Scholar 4Santos-Juanes J, Coto-Segura P, Mallo S, Galache C, Soto J. Multiple eruptive dermatofibromas in a patient receiving efalizumab. Dermatology 2008; 216: 363. 10.1159/000117708 PubMedWeb of Science®Google Scholar 5Bachmeyer C, Cordier F, Blum L, Cazier A, Verola O, Aractingi S. Multiple eruptive dermatofibromas after highly active antiretroviral therapy. Br J Dermatol 2000; 143: 1336–1337. 10.1046/j.1365-2133.2000.03924.x CASPubMedWeb of Science®Google Scholar 6Rojas JM, Wang L, Owen S, Knight K, Watmough SJ, Clark RE. Naturally occurring CD4+ CD25+ FOXP3+ T-regulatory cells are increased in chronic myeloid leukemia patients not in complete cytogenetic remission and can be immunosuppressive. Exp Hematol 2010; 38: 1209–1218. Google Scholar 7Nestle FO, Nickoloff BJ, Burg G. Dermatofibroma: an abortive immunoreactive process mediated by dermal dendritic cells? Dermatology 1995; 190: 265–268. 10.1159/000246714 PubMedWeb of Science®Google Scholar 8Alexandrescu DT, Wiernik PH. Multiple eruptive dermatofibromas occurring in a patient with chronic myelogenous leukemia. Arch Dermatol 2005; 141: 397–398. 10.1001/archderm.141.3.397 PubMedWeb of Science®Google Scholar Citing Literature Volume28, Issue7July 2014Pages 979-981 ReferencesRelatedInformation

BACKGROUND AND OBJECTIVES:The prevalence of antiphospholipid antibodies (APLAs) has been extensively studied in patients with systemic lupus erythematosus (SLE) but not in those with cutaneous lupus erythematosus (CLE). We determined the prevalence of APLAs among our patients with CLE, and analyzed their clinical and serologic characteristics.MATERIALS AND METHODS:This retrospective study analyzed 182 patients with subacute or chronic CLE who had been in follow-up for 5 years. We selected those positive for 1 or more of the following APLAs in 2 measurements at least 12 weeks apart: lupus anticoagulant (LA), anticardiolipin antibodies (ACAs), and anti-β2-glycoprotein i (anti-β2-GPI) antibodies. In the case of ACAs and anti-β2-GPI antibodies, only patients with titers greater than or equal to 40 U/mL were selected.RESULTS:We obtained a series of 13 patients (4 with subacute disease and 9 with chronic disease). Seven met the diagnostic criteria for SLE and only 1 met the diagnostic criteria for antiphospholipid syndrome (APS). The prevalence of APLAs was 38% among patients with SLE and 3.65% among those without SLE. The most prevalent APLA was LA, present in 10 patients. Antinuclear antibodies (ANAs) were detected in 12 patients and anti-double-stranded DNA antibodies in 11.CONCLUSIONS:The prevalence of APLAs among our patients with CLE who did not meet the diagnostic criteria for SLE was similar to that reported in the general population. This, along with the strong assocation between the presence of ANAs and the presence of APLAs, would bring into question the value of determining APLAs in patients with CLE who are negative for ANAs. We also note that there was a high prevalence of discoid lesions but a low prevalence of APS among our patients with CLE who were positive for APLAs.

The coexistence of non-Hodgkin lymphoma (NHL) and Hodgkin disease (HD) in the same patient, although previously reported, is very unusual. This situation is extremely rare when the first diagnosis is a cutaneous B NHL, and exceptional if there is no personal background of cytostatic treatment. We report a 44-year-old man who developed cutaneous nodules over a period of two years. A marginal zone cutaneous B-cell lymphoma was diagnosed. On staging investigation a mass in the lingual tonsil was found and excision biopsy showed a classical Hodgkin lymphoma.

BACKGROUND:Antitumour necrosis factor (anti-TNF)-α agents can be used successfully to treat patients with psoriasis and other inflammatory diseases. However, very few studies have examined the relationship between TNF-α polymorphisms and the response to anti-TNF-α agents.OBJECTIVES:To study the association of single nucleotide polymorphisms (SNPs) of the TNF-α promoter and IL12B/IL23R genes with the response to anti-TNF-α in patients with psoriasis.METHODS:SNPs for the TNF-α promoter and IL12B/IL23R genes, and the presence of the HLA-Cw6 haplotype were genotyped for 109 patients. We studied the association between these SNPs and the efficacy of treatment at 3 and 6 months [Psoriasis Area and Severity Index (PASI) and body surface area (BSA)].RESULTS:Patients with the TNF-α-238GG genotype more frequently achieved a PASI75 at 6 months (82·5% vs. 58·8%, P = 0·049). At 6 months, patients with the TNF-α-857CT/TT genotypes showed greater improvements in PASI score and BSA (83·1% vs. 92·7%, P = 0·004; 82·7% vs. 92·6%, P = 0·009) and more frequently achieved PASI75 (71·4% vs. 96·3%, P = 0·006). More patients with the TNF-α-1031TT genotype achieved PASI75 at 3 months (90·8 vs. 75·7, P = 0·047) and 6 months (85·5% vs. 65·7%, P = 0·038) and demonstrated superior improvements in PASI at 6 months (89·9% vs. 78·7%, P = 0·041). Patients with the IL23R-GG genotype (rs11209026) achieved PASI90 at 6 months more frequently (66·3% vs. 0, P = 0·006) and the improvement of the PASI score was also greater (86·8% vs. 67·8%, P = 0·013). Patients with the HLA-Cw6 haplotype showed poorer response than those without this haplotype.CONCLUSION:This study identified a relationship between certain TNF-α and IL12B/IL23R polymorphisms and the short-term response to anti-TNF-α drugs. If these results are confirmed, this information will allow for stratified consent with more accurate prediction of response/personalized choice of treatment hierarchy for the patient.

La lipodistrofia adquirida generalizada (LAG) es un trastorno raro caracterizado por un patrón característico de pérdida de tejido adiposo y frecuentemente asociado con trastornos metabólicos. El inicio tardío en mayores de 65 años es excepcional y se ha reportado solo en un único paciente.Además, no se han publicado casos de LAG asociada a afectación muscular. Presentamos a una mujer de 78 años con pérdida prácticamente completa de su tejido adiposo subcutáneo a lo largo de los últimos 6 años. Además durante ese período fue sucesivamente diagnosticada de hipertensión, hipertrigliceridemia, hipotiroidismo, esteatosis hepática y diabetes mellitus. Enfermedades que pueden causar caquexia como infecciones, neoplasias o alteraciones gastrointestinales fueron descartadas. Sus niveles de creatinin kinasa fueron repetidamente elevados y su electromiograma mostró un patrón miopático aunque tanto la biopsia como las pruebas de fuerza fueron normales.

Macular lymphocytic arteritis describes a recently reported entity, clinically characterized by asymptomatic hyperpigmented macules on the lower limbs, without association of systemic diseases. Histopathologically it is characterized by a lymphocytic arteritis with a hyalinized fibrin ring. We report a new case presenting with ulceration, a finding not previously described. A 25‐year‐old Hispanic woman was evaluated for a 1‐year history of a gradually progressive, asymptomatic eruption that begins at level of both knees and progressively affects both legs and feet. She also referred recently appeared ulcers on inner right ankle without previous traumatism. Physical examination revealed multiple fairly well‐defined light brown and faint pink patches with petechiae on as well as retiform crusts and livedoid lesions on inner right ankle. Both types of lesions were biopsied showing lymphocytic arteritis with fibrinoid necrosis and thrombus. There were no relevant laboratory alterations. The clinical peculiarity of our case is the clinical image of the lesions mimicking a pigmented purpuric dermatosis and the presence of a non‐traumatic ulcer which could be explained because chronic lymphocytic damage may cause ischemic damage. Ulceration in our case supports consideration of macular arteritis as a latent form of cutaneous polyarteritis nodosa.

Background  Both the safety and efficacy of biologic therapy may be affected in the presence of highly prevalent chronic viral hepatitis.