Estudio retrospectivo de pacientes diagnosticados de síndrome de Sweet en el área de un hospital terciario de la Comunidad Valenciana / Retrospective Study of Patients Diagnosed With Sweet Syndrome in the Health Area of a Tertiary Hospital in the Autonomous Community of Valencia

El síndrome de Sweet (SS) es la más característica de todas las dermatosis neutrofílicas. Para definir el perfil de los pacientes diagnosticados de SS en nuestro Departamento y evaluar las diferencias clínico-epidemiológicas entre subgrupos, hemos realizado un estudio retrospectivo desde 2001 a 2009, ambos inclusive. Han sido incluidos 24 pacientes (13 mujeres y 11 hombres). La distribución por edades es similar en todos los grupos con dos picos: entre los 30-39 y los 70-79 años. Respecto a la etiología predomina el grupo que incluye los casos infecciosos e inflamatorios, seguido del grupo de etiología idiopática. De los 4 casos paraneoplásicos dos correspondían a neoplasias de órganos sólidos. Hay un caso asociado a la administración de infliximab. En cuanto a la evolución existe una mayor duración de la sintomatología en los casos paraneoplásicos e idiopáticos (AU)

Sweet syndrome is the most characteristic of the neutrophilic dermatoses. We performed a retrospective study of patients with Sweet syndrome seen in our department between 2001 and 2009, inclusive; the aims were to define the patient profile and to evaluate the clinical and epidemiological differences between subgroups. There were 24 patients (13 women and 11 men). The age distribution was similar in both sexes and showed 2 peaks, one in the fourth decade and the other in the eighth decade. The etiology was predominantly infectious or inflammatory, followed by the idiopathic form. There were 4 cases of paraneoplastic disease, 2 of which involved solid-organ tumors. One case was associated with the administration of infliximab. Symptoms persisted longer in cases that were idiopathic or that developed in the context of neoplastic disease (AU)

[Retrospective study of patients diagnosed with Sweet syndrome in the health area of a tertiary hospital in the autonomous community of Valencia]. / Estudio retrospectivo de pacientes diagnosticados de síndrome de Sweet en el área de un hospital terciario de la Comunidad Valenciana.

Sweet syndrome is the most characteristic of the neutrophilic dermatoses. We performed a retrospective study of patients with Sweet syndrome seen in our department between 2001 and 2009, inclusive; the aims were to define the patient profile and to evaluate the clinical and epidemiological differences between subgroups. There were 24 patients (13 women and 11 men). The age distribution was similar in both sexes and showed 2 peaks, one in the fourth decade and the other in the eighth decade. The etiology was predominantly infectious or inflammatory, followed by the idiopathic form. There were 4 cases of paraneoplastic disease, 2 of which involved solid-organ tumors. One case was associated with the administration of infliximab. Symptoms persisted longer in cases that were idiopathic or that developed in the context of neoplastic disease.

[Vulvar lymphedema: unusual manifestation of metastatic Crohn's disease]. / Linfedema vulvar: una manifestación infrecuente de la enfermedad de Crohn metastásica.

Cutaneous-mucosal lesions constitute one of the most frequent extraintestinal manifestations of Crohn's disease and in some cases may be the first symptom of intestinal disease. We describe the case of a 45-year-old female patient who sought medical help for genital tumefaction of 20 years' evolution. For the previous 15 years, she had been experiencing digestive symptomatology attributed to irritable bowel syndrome. Two months before the consultation, and coinciding with aggravation of the condition, the patient had been diagnosed with colonic Crohn's disease. Skin biopsy of the labia minora revealed sarcoid granulomas. The results of microbiological studies (staining for microorganisms and cultures) were negative. A diagnosis of metastatic vulvar Crohn's disease was made and, treatment with metronidazole was started, which improved the genital edema after 2 months. Genital lymphedema is an exceptionally rare manifestation of metastatic Crohn's disease that may appear several years before intestinal symptomatology develops. Treatment with metronidazole seems to be a good therapeutic option.

Síndrome de Stevens-Johnson por nevirapina

No disponible

Esclerodermatomiositis infantil / Infantile sclerodermatomyositis

La esclerodermatomiositis (EDM) es un síndrome de solapamiento, caracterizado por la presencia de manifestaciones clínicas de esclerosis sistémica y de dermatomiositis, cuyo principal marcador serológico es el anticuerpo anti-PM/Scl. Presentamos el caso clínico de una niña de 10 años diagnosticada de EDM (AU)

Identification of a novel keratinocyte mitogen derived from bovine pituitary glands.

Bovine pituitary glands contain one or more factors that are important for keratinocyte proliferation in serum-free culture medium. We used a tissue culture system in which the growth of keratinocytes in basal medium (KBM) containing insulin was dependent upon exogenous growth factors. Using this experimental system, we began to purify and characterize the pituitary factor(s) necessary for clonal growth of human keratinocytes in serum-free medium. Proteins of approximately 150 kDa and 95 kDa bound specifically to living keratinocytes, and we suggest that the 95 kDa protein is a likely novel mitogen. Although prolactin has been previously identified as a pituitary hormone that may act as an in vitro mitogen for keratinocytes, imunoblots indicated that the 95 kDa protein was unrelated to prolactin. Furthermore, the 95 kDa protein showed high homology with a bovine 90 kDa heat shock protein in the limited sequencing of an internal peptide.

Generalized eruptive histiocytoma evolving into xanthoma disseminatum in a 4-year-old boy.

We report a 4-year-old boy who developed a generalized and symmetrical eruption of brownish papules over a period of 8 months, with spontaneous regression of some lesions. Clinical, histopathological and ultrastructural studies were suggestive of the diagnosis of generalized eruptive histiocytoma (GEH). The clinical features subsequently changed to a generalized eruption of confluent, yellowish papules, and diabetes insipidus developed. The clinical, histopathological and ultrastructural features of the new lesions were those of xanthoma disseminatum with cerebral involvement. This evolution suggests that GEH and xanthoma disseminatum may be variants of a continuous spectrum of histiocytic diseases.

Perianal pseudoverrucous papules and nodules after surgery for Hirschsprung disease.

We report two patients with Hirschsprung disease in whom severe diarrhea and a distinctive diaper dermatitis developed after delayed ileoanal anastomosis. The perianal papulonodular lesions cleared in months or years without specific treatment after resolution of the diarrhea. These perianal pseudoverrucous papules and nodules represent a peculiar form of primary irritant diaper dermatitis, distinct from Jacquet erosive diaper dermatitis and granuloma gluteale infantum.

Langerhans' cells and lymphocytic infiltrate in AIDS-associated Kaposi's sarcoma. An immunohistochemical study.

The epidermal Langerhans' cells are dendritic cells of the skin capable of triggering cutaneous immune responses. They possess the membrane antigens required to this effect: class II histocompatibility antigen, CD1a and CD4; the latter acts as receptor for the human immunodeficiency virus. The skin is the organ primarily affected by Kaposi's sarcoma (KS). In epidemic KS, the local immunologic conditions of the skin are little known. We therefore studied 12 patients with AIDS-associated KS, evaluating the density and phenotypic expression in KS-affected and unaffected skin of the following antigens: CD1a, HLA-DR, CD4 in dendritic epidermal cells and dermis, and CD3, CD4 and CD8 in cells of the inflammatory infiltrate, using monoclonal antibodies applied to frozen sections with the avidin-biotin-peroxidase technique. Langerhans' cells in the AIDS-KS skin lesions were found to be decreased in number. This decrease was even more pronounced in the case of cells expressing HLA-DR antigen. A number of them were also revealed with CD4. The tumour lymphocytic infiltrate was almost exclusively composed of CD3+ CD8+ phenotype lymphocytes. The dermis also revealed CD4+ dendritic cells. The basal keratinocytes focally expressed HLA-DR. These phenotypical alterations of the Langerhans' cells and the local immunological imbalance observed may contribute to the growth and continuity of the KS lesions.

Pyoderma-gangraenosum-like ulcers associated with lupus anticoagulant.

A 45-year-old women presented with a deep ulcerative lesion of the left breast. Two months later, a new cutaneous necrotic lesion located in the right pretibial areas was observed. The only serum abnormality was the presence of circulating lupus anticoagulant. Clinical features suggested the diagnosis of pyoderma gangraenosum and histologic examination showed microthrombosis in the dermal capillary vessels adjacent to a dense, mixed inflammatory infiltrate with some degree of vascular damage. Although thrombosis may be the result of vasculitis, it is likely that in our patient the presence of a lupus anticoagulant activity played a role in the pathogenesis of the cutaneous ulcers.

[Immunology of leprosy. Review of data from the 1980-1990 time period]. / Immunologie der Lepra. Ubersicht zu Daten des Zeitraumes 1980-1990.

The classification used for leprosy so far distinguishes between two polar forms (pauci-bacillary and multi-bacillary), with different influences on the immune cells. The identification of specific antigens of the germ surface with monoclonal antibodies allows finer differentiation in classification, which now extends to transitional forms of clinical relevance. The current status of knowledge about immunological phenomena triggered by Mycobacterium leprae is complex, and controversy is rife at present. The bacillus is comparable to other mycobacteria in antigenicity. Owing to the complicated composition of the cellular envelope, few epitopes are accessible for immunological reactions. Investigation on the behaviour of T-lymphocytes in combination with analysis of the bacterial surface structures led to the development of a new pathogenetic concept. In spite of different immunological and inflammatory reactions in patients (reflecting the individual pathology), the discrimination between a partial and a total immunological defect is made. So far, no histocompatibility-antigen type has been found that allows recognition of the predisposition to infection. Immunity against the bacteria is achieved after BCG in some cases. All this suggests that the development of a vaccine via biotechnical synthesis of idiotypes and anti-idiotypes may be possible.