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Biomedical subjects

A España

Publications and source records attributed to A España.

At least 19 recordsLinked to original sources
PubMed2005

[Surgical treatment of penile lymphedema associated with hidradenitis suppurativa].

Penoscrotal lymphedema is a rare disease in the developed countries, although it is relatively frequent in tropical countries. The most common cause is filariasis, although in our practice usually is associate to neoplasic and inflammatory processes, surgery, radiotherapy, hidroelectrolitic disbalances and idiopathic. We present a 22 years old patient with penoscrotal lymphedema due to hidradenitis suppurativa. After unsuccessful medical treatment, was performed a total excision of the penile skin and subcutaneous tissue to Buck's fascia. Split thickness skin grafts were used to cover the defect. Even medical management of penoscrotal lymphedema is not effective for most patients, surgery is a safe and effective procedure that gives excellent functional and cosmetic results.

Adult↗
PubMed2005

Successful treatment of lichen planus with sulfasalazine in 20 patients.

BACKGROUND: Lichen planus (LP) is a disturbing pruritic cutaneous disease that may have an spontaneous resolution or exhibit a more chronic course during some weeks or months. OBJECTIVE: Our objective was to demonstrate that sulfasalazine is effective in the treatment of LP. METHODS: Twenty patients were diagnosed in our department with LP of the skin and/or mucosa between 1985 and 2001 on the basis of clinical and histologic findings. RESULTS: All patients were treated with sulfasalzine at initial doses of 1.5 g/day, increasing by 0.5 g/week to 3 g/day for 4-16 weeks. Some patients also received descendent doses for 2-12 months. Complete responses were observed in 13 patients and partial responses in seven patients. All patients reported an early resolution of the pruritus. No changes were detected in mucosal LP. Most of the patients tolerated the treatment well and only eight patients presented some minor side-effects. CONCLUSION: Sulfasalazine is a successful therapeutic option for cutaneous LP, constituting an alternative to corticosteroids and retinoids.

Adolescent↗
PubMed2004

Pemphigus vulgaris autoantibodies induce apoptosis in HaCaT keratinocytes.

Pemphigus vulgaris (PV) is an autoimmune disease characterized by binding of IgG autoantibodies to epidermal keratinocyte desmosomes. IgG autoantibodies obtained from a patient with mucocutaneous PV reacted with plakoglobin (Plkg) in addition to desmoglein-3 (Dsg3) and Dsg1. Immunofluorescence analysis confirmed that IgG autoantibodies, unlike antibodies from a healthy volunteer, caused disruption of cell-cell contacts in HaCaT keratinocytes. Moreover, apoptosis was enhanced in cells treated with autoantibodies compared to those treated with normal antibodies. The apoptotic process induced by IgG autoantibodies was characterized by caspase-3 activation, Bcl-2 depletion and Bax expression. The present report demonstrates that PV IgG autoantibodies promote apoptosis in HaCaT keratinocytes.

Apoptosis↗
PubMed2004

In vivo blockade of pemphigus vulgaris acantholysis by inhibition of intracellular signal transduction cascades.

BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune disease characterized by mucocutaneous intraepithelial blisters and pathogenic autoantibodies against desmoglein 3. The mechanism of blister formation in pemphigus has not been defined; however, in vitro data suggest a role for activation of intracellular signalling cascades. OBJECTIVES: To investigate the contribution of these signalling pathways to the mechanism of PV IgG-induced acantholysis in vivo. METHODS: We used the passive transfer mouse model. Mice were injected with IgG fractions of sera from a patient with PV, with or without pretreatment with inhibitors of proteins that mediate intracellular signalling cascades. RESULTS: Inhibitors of tyrosine kinases, phospholipase C, calmodulin and the serine/threonine kinase protein kinase C prevented PV IgG-induced acantholysis in vivo. CONCLUSIONS: These observations strongly support the role of intracellular signalling cascades in the molecular mechanism of PV IgG-induced acantholysis.

Acantholysis↗
PubMed2004

Acneiform lesions secondary to ZD1839, an inhibitor of the epidermal growth factor receptor.

Drugs that inhibit the epidermal growth factor receptor, such as ZD1839 or C225, are being used increasingly in the treatment of solid tumours. This has led to the appearance of new secondary effects. We describe the case of a patient who presented with an acneiform eruption secondary to the administration of ZD1839. These lesions healed in a few days after stopping the drug.

Acneiform Eruptions↗
PubMed2003

Systemic lupus erythematosus-associated anetoderma and anti-phospholipid antibodies.

Anetoderma is characterized by a loss of normal elastic tissue that presents clinically as localized areas of wrinkled or flaccid skin. We describe the case of a 30-year-old woman with systemic lupus erythematosus-associated anetoderma and positive anti-phospholipid antibodies. We discuss the possible role of these antibodies in the pathogenesis of anetoderma, and, when detected, the need to check for an associated anti-phospholipid syndrome in such patients.

Adult↗
PubMed2001

Bleomycin in the treatment of keloids and hypertrophic scars by multiple needle punctures.

BACKGROUND: The treatment of keloids and hypertrophic scars has been difficult and a recent French study showed that bleomycin has been useful in the treatment of these lesions. OBJECTIVE: To determine the effectiveness and safety of bleomycin in the treatment of hypertrophic scars and keloids when this drug is administered through multiple superficial punctures. METHODS: We applied bleomycin to keloids and hypertrophic scars in 13 patients using a multiple-puncture method on the surface of the skin. All patients were given bleomycin at a concentration of 1.5 IU/ml. Clinical response after treatment was classified according to the following scale: complete flattening (100%), highly significant flattening (>90%), or significant flattening (75-90%). RESULTS: The clinical response was very positive in all cases: complete flattening in six cases, highly significant flattening in six cases, and significant flattening in one case. Two patients presented a recurrence as a small nodule 10 and 12 months after the last infiltration. CONCLUSIONS: These clinical findings show that administration of bleomycin in keloids and hypertrophic scars shows promise and needs further investigation.

Adolescent↗
PubMed2000

Langerhans' cell histiocytosis on the vulva.

BACKGROUND: Langerhans' cell histiocytosis (LCH) affecting the vulva alone is rare. At present, there are 51 published cases of LCH affecting the genital area. Of these, only 7 had LCH located in the genital area alone; in these cases, there was no subsequent systemic spread of the disease. A report of the 8th such case is presented with a review of the previous 7 cases. CASE: A 40-year-old white woman presented with a several-month history of pruritic and ulcerous lesions on the vagina which had then spread to the vulva. No tiredness, general malaise, or associated temperature was observed. Physical examination disclosed an erythematous plaque on the labia minora, which was bilateral and slightly infiltrated. The rest of the physical examination brought no other disorders of interest to light. Histological findings were characteristic of LCH. Four cycles of iv vincristine (2 mg/m cycle) 15 days apart did not obtain objective or symptomatic improvement in the vulvar lesions. Therefore, a local and partial extirpation of both labia minora was performed. Eighteen months after surgery, the patient has no symptoms or signs of local recurrence or systemic spread. CONCLUSIONS: Although the occurrence of LCH on the vulva is very unusual, we must bear this possibility in mind when a woman presents atypical chronic lesions on the genital mucosa. In such cases, it is necessary to perform a biopsy on the mucosa, rule out the possibility of systemic disease, and review the patient periodically in order to forestall a possible spread of the disease at any time.

Adult↗
PubMed2000

Congenital infiltrating lipoma of the upper limb in a patient with von Willebrand disease.

Infiltrating lipoma is a rare variety of lipoma, characterized by an infiltration of the adipose tissue of the muscles. Infiltrating lipomas are usually classified in two groups: intermuscular infiltrating lipoma and intramuscular infiltrating lipoma. Most are acquired, and they usually appear in middle-aged individuals. Exceptionally, they are congenital. In such cases they are not related to other diseases. We report an 8-year-old boy with a congenital infiltrating lipoma of the upper limb and von Willebrand disease. Both diseases are linked to an alteration in chromosome 12, but this clinical association seems to be random rather than causal.

Arm↗
PubMed2000

Correlation between profile of circulating mononuclear cells and clinical manifestations in patients with pemphigus vulgaris.

Phenotypes of 38 samples of mononuclear (PBMC) cells from 11 different patients with pemphigus vulgaris (PV) at different stages of the disease were explored looking for a possible relationship between cell immunity, mucocutaneous or mucosal lesion intensity and capacity of serum autoantibodies to elicit the disease in mice. PBMC from 5 patients with mucocutaneous lesions and sera with IgG capable of inducing the disease in neonatal mice had a high proportion of mature monocytes with CD14low DRhigh, and co-expressing CD16 and CD11b. In addition, a high proportion of CD19+CD5+ activated B cells and a very low proportion of naive CD4+CD45RA+ and CD8+CD11b+ T lymphocytes was observed. Monocytes from these patients expressed inducible nitric oxide synthase (iNOS). In contrast, PBMC from 6 patients, with lesions restricted to mucosal membranes and IgG lacking the capacity to induce the disease in mice, contained a high proportion of CD14high DRlow co-expressing CD16 circulating macrophages, CD8+CD11b+ T cells, and a low proportion of activated B lymphocytes. The results suggest a possible association between proportion of different antigen presenting cells (monocytes with high HLA-DR and low CD14 expression and activated B lymphocytes, or differentiated monocytes/macrophages), type of PV and capacity of serum autoantibodies to elicit the disease in mice.

Acantholysis↗
PubMed1999

Wells' syndrome (eosinophilic cellulitis): correlation between clinical activity, eosinophil levels, eosinophil cation protein and interleukin-5.

Wells' syndrome (WS) (eosinophilic cellulitis) is characterized by the presence of oedematous skin lesions associated with eosinophilia of the tissues. It has recently been observed that in patients with this disease, increased eosinophil cation protein (ECP) and interleukin (IL) -5 can be detected in peripheral blood, with T lymphocytes that have mRNA for this lymphokine. We present a patient with WS in whom we found a close correlation between clinical activity, eosinophils in blood and bone marrow, and ECP and IL-5 levels in peripheral blood and tissues. We underline the major part played by IL-5 in this disease.

Adult↗
PubMed1999

Disseminated epidermolytic acanthoma probably related to trauma.

Epidermolytic acanthoma is a rare benign tumour, which may occur in both isolated and disseminated forms. Only seven cases of disseminated epidermolytic acanthoma (DEA) have been described. This entity should be distinguished from other hereditary or acquired conditions which involve epidermolytic hyperkeratosis and other benign acanthomas. On the basis of the clinical history and the histological findings, we diagnosed a case of DEA which was probably secondary to repeated trauma.

Adult↗
PubMed1997

Mechanisms of acantholysis in pemphigus foliaceus.

Pemphigus foliaceus (PF) is a dermatosis characterized by subcorneal vesicles and pathogenic IgG autoantibodies against desmoglein 1. PF IgG passively transferred into neonatal mice induces a blistering disease that duplicates the key findings of PF. In this study we have used this animal model to investigate the role of complement and IgG valence in triggering blister formation. In the passive transfer experiments, we found that PF IgG, as well as the F(ab')2 and Fab fragments, was capable of inducing the typical subcorneal blistering disease in both complement-deficient and complement-sufficient mice. Moreover, the disease activity in these mice correlated well with the dose of IgG or its proteolytic fragments injected in the animals. We conclude that neither complement activation nor IgG-mediated cell surface antigen crosslinking is required for the induction of acantholysis in the experimental PF model.

Acantholysis↗
PubMed1997

Mechanisms of acantholysis in pemphigus vulgaris: role of IgG valence.

Pemphigus vulgaris (PV) is a dermatosis mediated by autoantibodies against desmoglein 3 (Dsg3). It was known that intraperitoneal (i.p.) injections of PV IgG and PV F(ab')2, but not of PV Fab, into neonatal mice reproduced the key features of the disease in these animals. It was proposed that crosslinking of antigen by bivalent PV autoantibodies may trigger acantholysis in PV. In the present study, we have used subcutaneous (s.c.) injections of PV IgG and its proteolytic fragments into neonatal mice to test equimolar amounts of these autoantibody fractions. Mice developed clinical and histological features of PV in a dose-dependent manner following a similar time course. PV IgG and Fab fractions induced acantholysis as early as 2 hr after the injection. It was also demonstrated that sc injections of PV Fab were more effective in inducing disease than ip injections. PV autoantibodies may bind an "adhesive site" of Dsg3 and impair its function, thus triggering acantholysis.

Acantholysis↗