Biomedical subjects
Amaro García-Díez
Publications and source records attributed to Amaro García-Díez.
The clinical and histologic spectrum of chronic graft-versus-host disease.
Explore the source record for details and available documents.
[Single blister in pretibial region].
Explore the source record for details and available documents.
Nodular herpes zoster with herpetic syringitis and no epidermal involvement in a patient with Burkitt lymphoma.
Herpes zoster (HZ) occurs with an increased incidence in immunosuppressed patients, in whom it frequently displays atypical clinical presentations. Herpetic syringitis, the involvement of the eccrine epithelium by herpes virus infection, is an infrequently described histologic pattern that has been rarely and almost exclusively reported in HIV-infected patients. We report the case of a woman with Burkitt lymphoma who developed 2 nodular, asymptomatic lesions while receiving treatment with chemotherapy and radiotherapy for her hematological disease. Histology showed viropathic changes in the epithelium of eccrine glands not in the epidermis. PCR was positive for varicella-zoster virus (VZV). Nodular herpes zoster seems to be an exceptional clinical presentation. We report another such case which is, as far as we know, the first report of herpetic syringitis with no concomitant epidermal involvement.
A case of Grover's disease with syringoma-like features and leukemia cutis.
UNLABELLED: Leukemia cutis used to be a late manifestation of leukemia. On the other hand, Grover's disease has been described in the setting of cancer. CASE REPORT: A patient diagnosed as having a chronic myelomonocytic leukemia presented with lichenoid, slightly infiltrated plaques on the anterior aspect of his thorax and abdomen and papulovesicles on his back. A skin biopsy showed the co-existence of leukemia cutis, Grover's disease, and syringoma-like features. DISCUSSION: We discuss and comment upon this unusual association.
Safety, efficacy, and dosage of 1% pimecrolimus cream for the treatment of atopic dermatitis in daily practice.
INTRODUCTION: Although several controlled clinical trials have demonstrated the efficacy and good tolerability of 1% pimecrolimus cream for the treatment of atopic dermatitis, the results of these trials may not apply to real-life usage. The objective of this study was to evaluate the safety and efficacy of a pimecrolimus-based regimen in daily practice. METHODS: This was a 6-month, open-label, multicenter study in 947 patients aged >or=3 months with atopic dermatitis of all severities. The investigators incorporated 1% pimecrolimus cream into patients' standard treatment protocols on the basis of their clinical diagnosis. Use of topical corticosteroids was allowed at the discretion of the physician. Safety and tolerability were evaluated by monitoring adverse events. Efficacy was evaluated by recording changes in the Investigators' Global Assessment scores and pruritus scores at each visit. RESULTS: No clinically unexpected adverse events were reported. The discontinuation rate for adverse events was 2.3%. The disease improvement rate was 53.7% at week 1 and 66.9% at week 24. The pimecrolimus-based regimen was particularly effective for the treatment of lesions involving the face (improvement rate: 61.9% at week 1 and 76.7% at week 24). The greatest therapeutic response was experienced by pediatric patients with mild or moderate disease. Nonetheless, 64% and 65% of infants and children, respectively, with severe/very severe facial disease at baseline were clear/almost clear of signs of atopic dermatitis on their face at week 24. In patients aged <18 years, most of the improvement occurred within the first week of treatment, while in adults a progressive improvement was observed over the entire study period. Worsening of disease by the end of the study occurred in 9.5% of patients and was most frequent in adults (12.6%). The discontinuation rate for unsatisfactory therapeutic effect was 4.8%. The mean number of treatment days was 135.6 (SD 53.2). The mean drug consumption (non-US centers only) was 4.2 g per treatment day. Drug consumption decreased over time as disease improved. In total, 47% of patients who completed the study never used topical corticosteroids over 6 months. CONCLUSION: In daily practice, incorporation of 1% pimecrolimus cream into patients' standard treatment regimen is well tolerated and improves atopic dermatitis in approximately two-thirds of patients. Disease improvement is particularly evident on the face. The greatest therapeutic response is experienced by pediatric patients with mild or moderate disease. In these patients, most of the improvement is observed within 1 week from the start of treatment.
Histiocytoid Sweet syndrome: a dermal infiltration of immature neutrophilic granulocytes.
OBJECTIVE: To describe a series of 41 patients with fresh lesions of Sweet syndrome in which the histopathologic study demonstrated an inflammatory infiltrate mostly composed of histiocytoid mononuclear cells. DESIGN: Histopathologic, immunohistochemical, and cytogenetic studies of the inflammatory infiltrate in a case series of histiocytoid Sweet syndrome. SETTING: University departments of dermatology and a private laboratory of dermatopathology. METHODS: Conventional histopathologic study as well as immunohistochemical investigations were performed using the alkaline phosphatase antialkaline phosphatase technique with a large panel of antibodies. In some cases, fluorescent in situ hybridization studies were performed to investigate the presence of the bcr/abl gene fusion. RESULTS: Immunohistochemical studies demonstrated that most cells of the infiltrate showed immunoreactivity for CD15, CD43, CD45, CD68, MAC-386, HAM56, and lysozyme, which is consistent with a monocytic-histiocytic immunoprofile. However, intense myeloperoxidase reactivity was detected in most of the cells with histiocytic appearance, which raised the possibility of specific cutaneous involvement by myelogenous leukemia. Nevertheless, cytologic peripheral blood examinations, fluorescent in situ hybridization studies to investigate the bcr/abl gene fusion, and follow-up of the patients, taken all together, ruled out this possibility. CONCLUSIONS: This case series demonstrates that some fresh cutaneous lesions of Sweet syndrome are histopathologically characterized by an infiltrate mostly composed of cells that may be misinterpreted as histiocytes, when in fact they are immature myeloid cells. We named this histopathologic variant histiocytoid Sweet syndrome, which should not be mistaken with leukemia cutis or other inflammatory dermatoses that are histopathologically characterized by histiocytes interstitially arranged between collagen bundles of the dermis.
[Follicular mycosis fungoides, comedo-like and cystic].
Follicular mycosis fungoides is an infrequent variant of mycosis fungoides. It has classically been defined by the presence of an atypical lymphoid infiltrate around and in the follicular epithelium with little or no epidermotropism, and no follicular mucin deposits. The fact that there are cases with epidermal involvement and/or follicular mucinosis means that some uniform diagnostic criteria are necessary. We describe two cases of follicular mycosis fungoides with follicular mucinosis and with varying degrees of associated epidermotropism.
[Cutaneous large B-cell lymphoma of the leg].
Large B-cell lymphoma of the leg (LBCLL) accounts for 2 % of primary cutaneous lymphomas, and the EORTC considers it to be of intermediate prognosis. It usually presents in elderly patients, and manifests clinically as erythematous nodules or tumors, often unilateral, on the lower third of the legs. On rare occasions, it presents with extracutaneous dissemination. We present the case of a 77-year-old male who consulted his physician because of an asymptomatic indurated erythematous papule which had appeared one month earlier on his left leg and had quickly and progressively increased in size. Concurrently, other similar lesions had been appearing on the thigh and back of the same leg. Histological analysis detected a large cell lymphoid infiltrate positive for CD20 and bcl-2 throughout the dermis. The extension study was normal. The patient was treated with an intravenous infusion of rituximab for four consecutive weeks, with full remission of the lesions.
[Occupational allergic contact dermatitis from color developers used in automatic developing].
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.
[Fast-growing frontal erythematous plaque].
Explore the source record for details and available documents.
[Cutaneous marginal zone B-cell lymphoma treated with rituximab].
Marginal zone B-cell lymphoma (MZL) is probably the most frequent of the primary cutaneous B-cell lymphomas, which are entities with indolent behavior. Clinically, it appears in middle-aged patients as papules, nodules or erythematous plaques, solitary or multiple, on the trunk and proximal part of the limbs. The prognosis is excellent despite frequent cutaneous recurrences. We present the case of a 40-year-old male who, after having several recurrences of MZL over a ten-year period, was treated with rituximab for multiple skin lesions. The patient showed full remission after four weeks of treatment, and developed cytokine-release syndrome after the first infusion of the drug.
[Lupus miliaris disseminatus faciei associated with epidermal cysts].
Lupus miliaris disseminatus faciei (LMDF) is a chronic granulomatous disease, which tends to be located on the central part of the face. Its typical histological image is one of epithelioid granulomas with caseous necrosis. Its association with epidermal cysts has been described on few occasions in literature. We present a case of LMDF associated with epidermal cysts in a female Caucasian patient. This association suggests that the rupture of the epidermal cysts may be related to the appearance of LMDF. However, there are probably other factors that may also contribute to its pathogenesis.
Solar-simulated ultraviolet radiation induces abnormal maturation and defective chemotaxis of dendritic cells.
Exposure to ultraviolet (UV) light induces immunosuppression. Different evidences indicate that this phenomenon is mainly a consequence of the effect of UV light on skin dendritic cells (DC). To investigate the cellular and molecular basis of this type of immunosuppression, we assessed in vitro the effect of solar-simulated UV radiation on the phenotypic and functional characteristics of human monocyte-derived DC and Langerhans-like DC. UV radiation induced a decreased expression of molecules involved in antigen capture as DC-SIGN and the mannose receptor. This effect was accompanied by a diminished endocytic capacity, an enhanced expression of molecules involved in antigen presentation such as major histocompatibility complex-II and CD86, and a significant increase in their capability to stimulate T cells. Furthermore, irradiated DC failed to acquire a full mature phenotype upon treatment with lipopolysaccharide. On the other hand, solar-simulated radiation induced the secretion of tumor necrosis factor-alpha and interleukin (IL)-10 by DC, but no IL-12. Interestingly, solar-simulated UV radiation also caused an altered migratory phenotype, with an increased expression of CXCR4, and a lack of induction of CCR7, thus correlating with a high chemotactic response to stromal cell-derived factor 1(SDF-1) (CXCL12), but not to secondary lymphoid tissue chemokine (SLC) (CCL21). These data indicate that solar-simulated UV radiation induces a defective maturation and an anomalous migratory phenotype of DC.
Role of tetraspanins CD9 and CD151 in primary melanocyte motility.
Tetraspanins CD9 and CD151 have been implicated in cellular motility and intercellular adhesion in several cellular types. Here, we have studied the subcellular localization and the functional role of these molecules in primary melanocytes. We found that endogenous tetraspanins preferentially clustered in areas of melanocyte homotypic intercellular contacts and at the tips of dendrites. These observations were further confirmed using time-lapse fluorescence confocal microscopy of melanocytes transfected with CD9- and CD151-GFP (green fluorescent protein) constructs, suggesting an involvement of these proteins in cellular contacts and migration. Cell adhesion and migration assays performed using blocking monoclonal antibodies against CD9 and CD151 showed no significant effect on cell-extracellular matrix adhesion, whereas the migration of melanocytes was significantly enhanced. The regulation of the migratory capacity of melanocytes by CD9 and CD151 was further confirmed knocking down the endogenous expression of these tetraspanins with small interference RNA oligonucleotides. Therefore, tetraspanin molecules are localized at motile structures in primary human melanocytes regulating the migratory capacity of these cells.
Dermal amyloid deposits in disseminated superficial porokeratosis.
Explore the source record for details and available documents.
Hepatocellular carcinoma risk in patients with porphyria cutanea tarda.
AIM: It has been suggested that patients with porphyria cutanea tarda (PCT) are at high risk of developing hepatocellular carcinoma (HCC); however, this has not been confirmed by other workers. The aim of our study was to evaluate the incidence of HCC in patients with PCT, and to assess the possible co-factors associated with cancer development. METHODS: Thirty-nine consecutive patients with a diagnosis of PCT were included. Hepatitis B virus and hepatitis C virus (HCV) infection was investigated, and a percutaneous liver biopsy was performed. Patients were treated with phlebotomies, which resulted in a clinical remission in all. These patients were included in a surveillance programme for the detection of HCC, with ultrasonography and serum alpha-fetoprotein every 6 months. RESULTS: Thirty-nine patients (92% male; mean age, 55 +/- 16 years) with PCT were included. Alcohol abuse was reported in 87% of the cases. The mean follow-up time since the initial diagnosis of PCT was 9.7 years (378 patient-years of follow-up). Serological markers of past infection with hepatitis B virus were found in 20% of the patients, while HCV infection was diagnosed in 56%. The stage of fibrosis in patients having liver biopsy was: 0 (32%), 1 (32%), 2 (9%), 3 (18%), and 4 (9%). HCC was diagnosed in 1/39 patients with PCT (cumulative incidence, 2.6%), giving a yearly incidence of 0.26% per patient-year. This patient was a 69-year-old male, alcohol abuser, with HCV infection, with a 12-year period between diagnosis of PCT and HCC, and with liver biopsy (3 years before) showing fibrosis stage 3. CONCLUSION: The risk of developing HCC in patients with PCT in our area is relatively low (a yearly incidence of less than 1% per patient-year of follow-up), and perhaps attributable, at least in part, to concomitant HCV infection. Patients presenting with PCT should undergo both HCV infection determination and liver biopsy, and those with concomitant HCV infection or advanced fibrosis/cirrhosis should probably be included in a standard surveillance programme in order to achieve early diagnosis of HCC.
Takayasu's disease with cutaneous involvement.
Takayasu's arteritis (TA) is a chronic inflammatory and fibrosing arteriopathy that can also involve cutaneous vessels. The disease typically presents with a prepulseless phase that overlaps or is followed by the characteristic pulseless stage. In both phases of TA, cutaneous manifestations may be present. Lesions considered to be 'specifically' associated with TA have been described most frequently simulating erythema nodosum, erythema induratum and pyoderma gangrenosum. We report 2 Caucasian patients with TA and nodular cutaneous lesions. Nine skin biopsies from these patients were studied. A necrotizing vasculitis was present in 5 biopsies. We review those patients with TA and well-documented cutaneous manifestations in the English literature, with special interest in nodular lesions, the most frequent cutaneous manifestation of TA in Caucasian patients. Biopsies from lesions with similar morphology frequently show different histological findings.