Amaro García-Díez

What Characterizes the Severity of Psoriasis

Background: Understanding the epidemiology of moderate-to-severe psoriasis is essential for its management. Objective:To assess the epidemiological characteristics of patients with moderate-to-severe psoriasis. Methods: Cross-sectional, observational epidemiological study conducted in Spain and Portugal. Data were collected by 332 dermatologists for ≧10 consecutive presenting patients. Results: Based on body surface area (BSA) and Psoriasis Area and Severity Index (PASI) criteria, moderate-to-severe psoriasis was confirmed in ≧79.3% of patients (n = 3,320). Preexisting comorbid conditions included psoriatic arthropathy (13%), dyslipidemia (14.1%) and hypertension (20.2%). The mean BSA involvement was 23% (95% confidence interval, CI: 22.2–23.3%), and the mean PASI score was 14.3 (95% CI: 13.9–14.6%). During the 2 years prior to assessment, 97.0% of patients had received topical treatments, whereas 31.3% had not received systemic treatment or phototherapy. The median annual cost of treatment was 825 EUR. Conclusion: Moderate-to-severe psoriasis is accurately diagnosed, but inadequately treated in many patients in Spain and Portugal.

HIV-Associated Eosinophilic Folliculitis and Follicular Mucinosis

The term HIV-associated eosinophilic folliculitis (EF) designates an idiopathic dermatitis that appears in HIV-infected patients with different clinical manifestations but with a distinctive histological feature characterized by a predominantly eosinophilic infiltrate in the follicular infundibula. On the other side, follicular mucinosis (FM) is a reaction pattern in the follicular epithelium, characterized by a mucinous degeneration of the outer sheath of follicles and sebaceous glands. It has been described in association with a variety of unrelated conditions. We report 2 HIV-infected patients with a pruritic papular eruption. Histopathological study revealed the coexistence of EF and FM. To our knowledge, this is the first report that describes this association. The possible relationship between these two entities is discussed.

Impacto de la psoriasis en la calidad de vida relacionada con la salud

Fundamento y objetivo: Diversos estudios han informado de las desventajas fisicas, sociales y psicologicas que presentan los pacientes con psoriasis. El objetivo del presente estudio es conocer la repercusion de la psoriasis moderada-grave en la calidad de vida relacionada con la salud, evaluada por medio del Indice de Discapacidad de la Psoriasis (Psoriasis Disability Index, PDI). Pacientes y metodo: Se ha realizado un estudio observacional, transversal e internacional en consultas de dermatologia de Espana y Portugal. Participaron 332 investigadores, cada uno de los cuales incluyo a 10 pacientes consecutivos diagnosticados de psoriasis moderada-grave. Se recogieron, entre otras, las siguientes variables: calidad de vida relacionada con la salud, medida por el PDI, variables objetivas de gravedad como la superficie corporal afectada (body surface area, BSA) y el indice de gravedad y de area de la psoriasis xadPASI (Psoriasis Area and Severity Index)xad, prurito, costes y disposicion a pagar. Resultados: Se incluyo a un total de 3.320 pacientes. La mayoria eran varones (57%) y la edad media era de 47 anos. El PDI presento un valor medio de 8,93 (intervalo de confianza del 95%, 7,83-9,21), lo que representa un porcentaje de discapacidad global inferior al 20%. El efecto suelo oscilo entre el 8,3 y el 61,2% y el efecto techo, entre el 0 y el 3%. Las correlaciones entre el PDI y las variables objetivas de gravedad (PASI y superficie corporal afectada) fueron debiles, aunque estadisticamente significativas (p < 0,001). Todas indican que cuanto mayor es la afeccion o la gravedad de la psoriasis, mayor es el impacto negativo en la calidad de vida relacionada con la salud. Conclusiones: La psoriasis tiene un impacto negativo en la calidad de vida relacionada con la salud, aunque las magnitudes halladas son debiles e indican algunos problemas con el cuestionario. Posiblemente el PDI refleje mas la discapacidad que la forma en que los individuos perciben y reaccionan ante su estado de salud, y algunos items parecen tener una escasa aplicabilidad a la poblacion espanola y portuguesa. El PDI no parece el instrumento idoneo para este tipo de pacientes en nuestro entorno sociocultural.

Identification of Genes Responsive to Solar Simulated UV Radiation in Human Monocyte-Derived Dendritic Cells

Ultraviolet (UV) irradiation has profound effects on the skin and the systemic immune system. Several effects of UV radiation on Dendritic cells (DCs) functions have been described. However, gene expression changes induced by UV radiation in DCs have not been addressed before. In this report, we irradiated human monocyte-derived DCs with solar-simulated UVA/UVB and analyzed regulated genes on human whole genome arrays. Results were validated by RT-PCR and further analyzed by Gene Set Enrichment Analysis (GSEA). Solar-simulated UV radiation up-regulated expression of genes involved in cellular stress and inflammation, and down-regulated genes involved in chemotaxis, vesicular transport and RNA processing. Twenty four genes were selected for comparison by RT-PCR with similarly treated human primary keratinocytes and human melanocytes. Several genes involved in the regulation of the immune response were differentially regulated in UVA/UVB irradiated human monocyte-derived DCs, such as protein tyrosine phosphatase, receptor type E (PTPRE), thrombospondin-1 (THBS1), inducible costimulator ligand (ICOSL), galectins, Src-like adapter protein (SLA), IL-10 and CCR7. These results indicate that UV-exposure triggers the regulation of a complex gene repertoire involved in human-DC–mediated immune responses.

Reticular Erythematous Mucinosis Associated with Human Immunodeficiency Virus Infection

We report the case of a 56-year-old woman with reticular erythematous mucinosis (REM). During her workup infection with the human immunodeficiency virus (HIV) was detected. She developed a cerebral toxoplasmosis, salmonella sp. bacteremia and oral ulcerations with the presence of type I herpes simplex virus and cytomegalovirus. The relation of REM with the deposition of mucin in AIDS patients bone marrow and HIV infection is discussed. To our knowledge, this is the first report where REM is associated with HIV disease.

Leukocytoclastic vasculitis in subacute cutaneous lupus erythematosus: clinicopathologic study of three cases and review of the literature.

BACKGROUNDnLeukocytoclastic vasculitis associated with subacute cutaneous lupus erythematosus (SCLE) was observed by Sontheimer et al. in their first clinical series of patients with SCLE, although recent reports have suggested that its frequency was rare.nnnOBJECTIVEnTo evaluate the prevalence of cutaneous leukocytoclastic vasculitis (CLV) in patients with SCLE and to describe the clinicopathologic manifestations, response to treatment and prognosis of this subgroup of patients.nnnMETHODSnWe reviewed all cases of SCLE seen at the Hospital de la Princesa from 1980 to 1995.nnnRESULTSnThree (9%) of our 27 patients with SCLE exhibited CLV and SCLE. Purpura, maculoerythematous lesions, urticaria, nodules and necrotic lesions on the leg, trunk and palmoplantar regions were present in our patients with CLV and SCLE. Histologically, SCLE-associated CLV was a small-sized vessel leukocytoclastic vasculitis with intravascular thrombosis in the deep dermis and coexistence of lesions of CLV and SCLE.nnnCONCLUSIONnIn our patients, CLV was self-limited and not associated with a worsened prognosis, with only cutaneous involvement in their further relapses of SCLE.

Refractory generalized pustular psoriasis responsive to a combination of adalimumab and acitretin

combination of adalimumab and acitretin Editor, Generalized pustular psoriasis is a distinctive rare variant of psoriasis which may become complicated by systemic alterations. Its management causes some difficulties, despite the availability of new modes of treatment with biologic therapy. The combination of biologics with acitretin has already been described for the treatment of moderate-to-severe plaque psoriasis but not for pustular psoriasis. We report a patient with generalized pustular psoriasis resistant to systemic conventional and biologic therapy, treated successfully with adalimumab in combination with acitretin. A 21-year-old woman presented with a 14-year history of pustular eruptions arising on an erythematous skin associated with scaly erythematous plaques; this was diagnosed clinically and histologically as pustular psoriasis. The patient had suffered an icthyosiform dermatitis during early childhood but was otherwise healthy. She had initially been treated with topical agents and intermittent courses of oral acitretin (0.5–1.0 mg/kg/d) since October 1994. In January 2004, the patient developed an acute severe thrombocytopenia that led to acitretin interruption, although the causal relationship with the drug was unclear. The patient then experienced a flare in psoriasis accompanied by fever and general malaise. Over the following four years, she was treated with different drugs as monotherapy or in combination. Firstly, treatment with oral intermittent cycles of cyclosporin (3 mg/kg/d) was ineffective. Then, acitretin monotherapy was restarted (50 mg/d), but achieved only a partial improvement with no side effects. Psoralen combined with ultraviolet A (PUVA) therapy was administered with acitretin for 12 weeks for a total dose of 37.5 J/cm and achieved a partial response. During a second cycle of PUVA therapy, after two doses of 2.0 J/cm and 2.5 J/cm, the patient developed a severe generalized pustular flare of psoriasis and required hospitalization (Fig. 1). Oral methotrexate (20 mg/week) was started, but new episodes of malaise and disseminated cutaneous lesions proved this to be unsuccessful. Intravenous infusions of infliximab (6 mg/kg) were added to methotrexate. The patient presented an increase in transaminase levels, and methotrexate was substituted by acitretin. This combination facilitated a remarkable improvement in the patient’s psoriasis, achieving almost complete clearance of the lesions, but the effectiveness of the therapy decreased progressively and relapses occurred. Infliximab was then substituted by subcutaneous adalimumab (80 mg at baseline, 40 mg at week 1, and then 40 mg every two weeks), which, in combination with acitretin (50 mg/d), achieved a dramatic improvement in the skin lesions (Fig. 2). After 11 months of continuous treatment, the patient’s psoriasis remains well controlled despite the progressive reduction of the acitretin dose (to 30 mg/d). The combination of acitretin with a biologic agent represents, in some cases, a unique approach in the Figure 1 Severe pustular generalized flare requiring hospitalization

Association between psoriasis and polymorphisms in the TNF , IL12B , and IL23R genes in Spanish patients

BACKGROUND & OBJECTIVESnSusceptibility to psoriasis has been associated with the HLA-C*0602 allele, although it may be affected by other polymorphisms.nnnMATERIALS & METHODSnWe genotyped 142 patients and 160 healthy volunteers to evaluate the possible relationship between susceptibility to psoriasis and the HLA-C*0602 allele and polymorphisms in the TNF, IL12B, and IL23R genes.nnnRESULTSnThe frequency of the wild-type TNF-238, TNF-308, and TNF-1031 genotypes was greater in patients with psoriasis than in healthy volunteers, although that of the mutant TNF-857 genotype was higher. The only difference between psoriasis and psoriatic arthritis was TNF-857. The frequency of the HLAC*0602 allele was higher in psoriatic patients than in healthy volunteers. No differences were observed for IL12B and IL23R. Multivariate logistic regression analysis only confirmed these associations for TNF-238, TNF-857, and HLA-C*0602.nnnCONCLUSIONnOur results support an association between susceptibility to psoriasis and TNF polymorphisms in the Spanish population.

Dermatitis de contacto alérgica profesional por reveladores de color del proceso de revelado automático

The color developers CD2, CD3 and CD4 and the black-and-white developer TSS are well known contact allergens that affect those who work in photographic processing. The frequency of occupational allergic contact dermatitis from color developers has decreased in recent years because of the fact that most photo developing takes place automatically, reducing exposure to different chemicals used in developing. We present a case of occupational allergic contact dermatitis in a non-atopic male who had worked for the last 5 years in a photography shop equipped with an automatic developing machine. The clinical morphology of the lesions was eczematous, and the patch tests showed contact sensitization to CD2, CD3, CD4 and TSS. The clinical symptoms cleared up when direct contact with the developing product containing CD3 and CD4 was avoided.