Paraneoplastic pemphigus with eosinophilic spongiosis and autoantibodies against desmocollins 2 and 3.

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.

Macular lymphocytic arteritis: first clinical presentation with ulcers.

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.

Prevalence of antiphospholipid antibodies in patients with subacute and chronic cutaneous lupus erythematosus.

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-ß2-glycoprotein i (anti-ß2-GPI) antibodies. In the case of ACAs and anti-ß2-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.

Ectodermal dysplasias: a clinical and molecular review.

The ectodermal dysplasias are a large group of hereditary disorders characterized by alterations of structures of ectodermal origin. Although some syndromes can have specific features, many of them share common clinical characteristics. Two main groups of ectodermal dysplasias can be distinguished. One group is characterized by aplasia or hypoplasia of ectodermal tissues, which fail to develop and differentiate because of a lack of reciprocal signaling between ectoderm and mesoderm, the other has palmoplantar keratoderma as its most striking feature, with additional manifestations when other highly specialized epithelia are also involved. In recent decades, the genes responsible for at least 30 different types of ectodermal dysplasia have been identified, throwing light on the pathogenic mechanisms involved and their correlation with clinical findings.

Victims of violence in an emergency department.

The aim of this study was to identify the risk profile of the victims of violent accidents. During 1997, we studied 1100 patients suffering from injuries produced by violent actions and treated in the emergency department of the largest teaching hospital in the Canary Islands, Spain. Seventy per cent of the patients were male and 30% were female. The mean age was 28 and 30 years, respectively. In the distribution by age, the main group was that between 18 and 36 years. Seventy-five per cent of violent accidents were public, 20% domestic and 5% remain unknown. The face-neck was the anatomical location with the highest number of severe injuries, followed by the upper extremities (42% vs. 26%, p<0.001). Eight per cent of the injured men presented severe injuries, in contrast with 1% of the women (p<0.001). Most patients (94%) were discharged from hospital after being treated. None of the victims died as a result of violence. The admission of victims occurred predominantly during the night hours and 35% of the patients were treated during the weekend. It is concluded that the typical victim of violent accidents in an emergency department of a Spanish teaching hospital is a young male with severe injuries, which occurred during late hours at weekends.

[Quality of life in relation to health and obesity in a primary care center]. / Calidad de vida relacionada con la salud y obesidad en un centro de atención primaria.

BACKGROUND: To evaluate the association between obesity and two aspects of health-related quality of life: self-perception of health status and psychological well-being in patients assigned to a primary care center. METHODS: 167 patients between 20 and 70 years old with a body mass index > or = were included in the study. A clinical interview was carried out to get sociodemographic and clinical data and to administer two questionnaires: 1) Nottingham Health Profile (NHP) and 2) Psychological General Well-being (PGWB) index. RESULTS: The mean scores of the NHP indicated a moderate overall impairment of perceived health in females. Pain, emotional reactions and physical mobility were the affected dimensions. Females, age group over 50, low level of education and the presence of psychiatric pathology or osteoarthritis were significantly associated with worse scores. The mean scores of PGWB indicates a small impairment of psychological well-being. In this case, worse scores were associated with females, anxiety and low level of education. CONCLUSIONS: In the study population, we identify a profile of obese patients with worse quality of life: women with age over 50, low level of education and the presence of psychiatric pathology or osteoarthritis. We think it would be a good idea to achieve a prior performance towards them.