RESUMEN
Pellagra is a nutrient deficiency disease caused by insufficient niacin levels. Recent studies have shown that numbers of epidermal Langerhans cells decreased in other diseases caused by nutritional deficiencies, including necrolytic migratory erythema and acrodermatitis enteropathica. Epidermal Langerhans cells are capable of modulating or even halting the inflammatory reaction. The aim of this study was to examine changes in the number of Langerhans cells and other dendritic cells, and maturation of epidermal Langerhans cells in the lesional and adjacent non-lesional skin in pellagra patients. Seven pellagra patients and 10 healthy individuals who served as controls were included. The number and distribution of dendritic cells and other cutaneous cells were examined by immunohistochemistry. Epidermal Langerhans cells decreased considerably in the skin lesions of pellagra patients, whereas other dendritic cells did not change. The decrease in the number of Langerhans cells was positively correlated with the histological severity of skin lesions. As the number of Langerhans cells was not reduced in the undisturbed neighboring skin, the depletion of epidermal Langerhans cells did not precede skin damage but was a cause of prolonged severe inflammation.
Asunto(s)
Epidermis/patología , Células de Langerhans/patología , Pelagra/patología , Adulto , Anciano , Biopsia , Estudios de Casos y Controles , Recuento de Células , Epidermis/química , Femenino , Humanos , Inmunohistoquímica , Japón , Células de Langerhans/química , Masculino , Persona de Mediana Edad , Índice de Severidad de la EnfermedadRESUMEN
BACKGROUND: Patients with steroid-resistant bullous pemphigoid (BP) require an appropriate treatment option. OBJECTIVE: A multicenter, randomized, placebo-controlled, double-blind trial was conducted to investigate the therapeutic effect of high-dose intravenous immunoglobulin (IVIG; 400mg/kg/day for 5days) in BP patients who showed no symptomatic improvement with prednisolone (≥0.4mg/kg/day) administered. METHODS: We evaluated the efficacy using the disease activity score on day15 (DAS15) as a primary endpoint, and changes in the DAS over time, the anti-BP180 antibody titer, and safety for a period of 57days as secondary endpoints. RESULTS: We enrolled 56 patients in this study. The DAS15 was 12.5 points lower in the IVIG group than in the placebo group (p=0.089). The mean DAS of the IVIG group was constantly lower than that of the placebo group throughout the course of observation, and a post hoc analysis of covariance revealed a significant difference (p=0.041). Furthermore, when analyzed only in severe cases (DAS≥40), the DAS15 differed significantly (p=0.046). The anti-BP180 antibody titers showed no difference between the two groups. CONCLUSION: IVIG provides a beneficial therapeutic outcome for patients with BP who are resistant to steroid therapy.
Asunto(s)
Resistencia a Medicamentos , Glucocorticoides/farmacología , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Penfigoide Ampolloso/terapia , Prednisolona/farmacología , Anciano , Anciano de 80 o más Años , Autoanticuerpos/sangre , Autoantígenos/inmunología , Método Doble Ciego , Femenino , Glucocorticoides/administración & dosificación , Glucocorticoides/uso terapéutico , Humanos , Inmunoglobulinas Intravenosas/administración & dosificación , Inmunoglobulinas Intravenosas/efectos adversos , Factores Inmunológicos/administración & dosificación , Factores Inmunológicos/efectos adversos , Masculino , Persona de Mediana Edad , Colágenos no Fibrilares/inmunología , Penfigoide Ampolloso/inmunología , Prednisolona/administración & dosificación , Prednisolona/uso terapéutico , Resultado del Tratamiento , Colágeno Tipo XVIIAsunto(s)
Carcinoma de Células Escamosas/patología , Neoplasias Primarias Secundarias/patología , Nevo Sebáceo de Jadassohn/patología , Neoplasias Cutáneas/patología , Encéfalo/diagnóstico por imagen , Carcinoma de Células Escamosas/cirugía , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neoplasias Primarias Secundarias/cirugía , Nevo Sebáceo de Jadassohn/cirugía , Neoplasias Cutáneas/cirugía , TorsoAsunto(s)
Dermatitis Atópica/inmunología , Dermatitis Atópica/fisiopatología , Interleucina-13/biosíntesis , Interleucinas/biosíntesis , Subgrupos de Linfocitos T/inmunología , Adulto , Linfocitos T CD4-Positivos/inmunología , Linfocitos T CD4-Positivos/metabolismo , Linfocitos T CD8-positivos/inmunología , Linfocitos T CD8-positivos/metabolismo , Humanos , Inmunoglobulina E/sangre , Piel/inmunología , Subgrupos de Linfocitos T/metabolismo , Interleucina-22Asunto(s)
Síndrome de Stevens-Johnson/inmunología , Células Th17/inmunología , Antiinflamatorios/uso terapéutico , Vesícula/inmunología , Citocinas/sangre , Citocinas/inmunología , Humanos , Prednisolona/uso terapéutico , Síndrome de Stevens-Johnson/sangre , Síndrome de Stevens-Johnson/tratamiento farmacológicoAsunto(s)
Dermatomiositis/complicaciones , Dermatosis de la Mano/complicaciones , Neoplasias/complicaciones , Adulto , Anciano , Creatina Quinasa/sangre , Dermatomiositis/fisiopatología , Eritema/complicaciones , Femenino , Dermatosis de la Mano/fisiopatología , Humanos , Enfermedades Pulmonares Intersticiales/complicaciones , Masculino , Persona de Mediana Edad , Debilidad Muscular/complicaciones , Músculo Esquelético/fisiopatología , Estudios Retrospectivos , Factores SexualesRESUMEN
BACKGROUND: Recently, reports have indicated that the continuous use of topical calcineurin inhibitors such as tacrolimus may induce rosacea-like dermatitis (RD). OBJECTIVE AND METHODS: To assess clinical features of RD associated with tacrolimus, 44 cases of patients diagnosed with RD between 2005 and 2010 at our hospital were retrospectively reviewed. RESULTS: In total, 22 cases were caused by topical steroid use, 8 by topical tacrolimus use, and 8 by consecutive treatment with topical steroids and tacrolimus. Clinical presentation was basically similar among the 3 groups, although the nose was less frequently affected and pustules were rarely observed in the latter 2 sets of cases. Demodex mites were often found in smears of skin lesions from patients with RD caused by steroids and tacrolimus. Treatment with topical metronidazole was effective in most RD patients. CONCLUSION: Topical tacrolimus is becoming an important cause of RD along with topical steroids.
Asunto(s)
Inmunosupresores/efectos adversos , Infestaciones por Ácaros/etiología , Pomadas/administración & dosificación , Rosácea/inducido químicamente , Esteroides/efectos adversos , Tacrolimus/efectos adversos , Administración Tópica , Adulto , Anciano , Femenino , Humanos , Inmunosupresores/administración & dosificación , Masculino , Persona de Mediana Edad , Pomadas/efectos adversos , Estudios Retrospectivos , Rosácea/complicaciones , Esteroides/administración & dosificación , Tacrolimus/administración & dosificaciónRESUMEN
BACKGROUND AND AIMS: Subcutaneous aseptic abscess is one phenotype of neutrophilic dermatitis. We were interested to see if a case of steroid refractory Crohn's disease (CD) complicated by subcutaneous aseptic neutrophilic abscesses responds to intensive granulocyte/monocyte adsorptive apheresis (GMA). METHODS: The patient was a 21-year-old male with worsening severe CD while on oral prednisolone (30 mg/day). His symptoms included fever, bloody diarrhoea and multiple painful subcutaneous nodules throughout his body. Skin biopsy showed chronic panniculitis with neutrophilic infiltrates. Further, colonoscopy showed oedematous sigmoid colon, while colonic biopsy showed non-caseous granuloma. Because biologics were feared to increase the risk of bacteraemia as the result of germ culture on his pus was not known at the time, we decided to treat this case with GMA. Five GMA sessions with the Adacolumn over 5 consecutive days (daily GMA) were initiated. RESULTS: On admission, his CD activity index (CDAI) was 355, C-reactive protein (CRP) 11.2 mg/dL. After 5 GMA sessions, CDAI decreased to 170, and CRP fell to 5.0 mg/dL, with no fever. GMA was restarted at 2 sessions/week (total 10 sessions). The patient's CDAI fell to <150, and the skin lesions re-epithelialized. CONCLUSIONS: In this CD case complicated by subcutaneous aseptic neutrophilic abscesses, GMA appeared to be effective. Our impression is that when biopsy reveals neutrophil infiltrate is a major feature of the lesions, GMA should be considered. As GMA appears to have no safety concerns, a frequent GMA protocol, like daily followed by 2 to 3 times/week should be preferred over the routine weekly GMA.
Asunto(s)
Absceso/terapia , Enfermedad de Crohn/terapia , Granulocitos , Leucaféresis , Monocitos , Enfermedades de la Piel/terapia , Absceso/etiología , Adulto , Enfermedades del Colon/etiología , Enfermedad de Crohn/sangre , Enfermedad de Crohn/complicaciones , Edema/etiología , Humanos , Masculino , Enfermedades de la Piel/etiología , Adulto JovenAsunto(s)
Infecciones por Mycobacterium no Tuberculosas/patología , Enfermedades Cutáneas Bacterianas/patología , ADN Bacteriano/genética , Femenino , Humanos , Japón , Pruebas de Sensibilidad Microbiana , Persona de Mediana Edad , Datos de Secuencia Molecular , Infecciones por Mycobacterium no Tuberculosas/tratamiento farmacológico , Infecciones por Mycobacterium no Tuberculosas/microbiología , Micobacterias no Tuberculosas/efectos de los fármacos , Micobacterias no Tuberculosas/genética , Micobacterias no Tuberculosas/aislamiento & purificación , Enfermedades Cutáneas Bacterianas/tratamiento farmacológico , Enfermedades Cutáneas Bacterianas/microbiologíaRESUMEN
Leishmaniasis has been occasionally reported in returnees from endemic areas. Here, we report a case of cutaneous leishmaniasis in a 33-year-old Japanese man who presented with a skin nodule after returning from an 8-year stay in West Africa including Burkina Faso. He was successfully treated with liposomal amphotericin B with no significant adverse effects. This is the first Japanese case of cutaneous leishmaniasis treated successfully with liposomal amphotericin B.
Asunto(s)
Anfotericina B/administración & dosificación , Antiprotozoarios/administración & dosificación , Leishmania major , Leishmaniasis Cutánea/tratamiento farmacológico , Adulto , África Occidental/etnología , Burkina Faso/etnología , ADN Protozoario/genética , ADN Protozoario/aislamiento & purificación , Humanos , Japón , Leishmania major/genética , Leishmaniasis Cutánea/parasitología , Leishmaniasis Cutánea/patología , Liposomas , MasculinoAsunto(s)
Analgésicos no Narcóticos/efectos adversos , Carbamazepina/efectos adversos , Erupciones por Medicamentos/etiología , Infecciones por Herpesviridae/etiología , Herpesvirus Humano 6/fisiología , Activación Viral/efectos de los fármacos , Analgésicos no Narcóticos/farmacología , Carbamazepina/farmacología , Femenino , Infecciones por Herpesviridae/virología , Humanos , Persona de Mediana EdadRESUMEN
BACKGROUND: Ritodrine hydrochloride, a ß2-adrenergic agonist, has been used for the treatment of pre-term labor as a relatively safe agent, although tolerable side-effects have been occasionally reported. OBJECTIVE: The purpose of this study was to assess our clinical experience of skin eruptions caused by ritodrine. METHODS: Fourteen pregnant women with pruritic skin eruptions associated with the administration of ritodrine for pre-term labor were examined in Saitama Medical Center Hospital between 2005 and 2008. RESULTS: Patients included both primigravidas and multigravidas. Their mean age was 33.7 years (range: 27-41 years). Almost all subjects were in the third trimester of pregnancy. Skin eruptions occurred 7-27 days (mean: 14.9 days) after the start of intravenous or oral ritodrine. In eight patients, the eruption occurred after an increase in the dose of the drug. The reaction was characterized by a pruritic, erythematous, papular eruption, mainly distributed on the abdomen and upper extremities. Lymphocyte transformation tests for ritodrine were positive in five of the eight patients. CONCLUSIONS: Ritodrine-induced, erythematous, papular eruption probably occurs more frequently than has been previously estimated. An immunologic mechanism may play a role in the development of this eruption caused by ritodrine, although the reaction is somewhat dependent on the dose.
Asunto(s)
Agonistas de Receptores Adrenérgicos beta 2/administración & dosificación , Agonistas de Receptores Adrenérgicos beta 2/efectos adversos , Erupciones por Medicamentos/etiología , Ritodrina/efectos adversos , Agonistas de Receptores Adrenérgicos beta 2/uso terapéutico , Adulto , Relación Dosis-Respuesta a Droga , Erupciones por Medicamentos/inmunología , Femenino , Humanos , Activación de Linfocitos/inmunología , Trabajo de Parto Prematuro/tratamiento farmacológico , Embarazo , Ritodrina/administración & dosificación , Ritodrina/uso terapéuticoRESUMEN
BACKGROUND: Pemphigus is a rare life-threatening intractable autoimmune blistering disease caused by IgG autoantibodies to desmogleins. It has been difficult to conduct a double-blind clinical study for pemphigus partly because, in a placebo group, appropriate treatment often must be provided when the disease flares. OBJECTIVE: A multicenter, randomized, placebo-controlled, double-blind trial was conducted to investigate the therapeutic effect of a single cycle of high-dose intravenous immunoglobulin (400, 200, or 0 mg/kg/d) administered over 5 consecutive days in patients relatively resistant to systemic steroids. METHODS: We evaluated efficacy with time to escape from the protocol as a novel primary end point, and pemphigus activity score, antidesmoglein enzyme-linked immunosorbent assay scores, and safety as secondary end points. RESULTS: We enrolled 61 patients with pemphigus vulgaris or pemphigus foliaceus who did not respond to prednisolone (> or =20 mg/d). Time to escape from the protocol was significantly prolonged in the 400-mg group compared with the placebo group (P < .001), and a dose-response relationship among the 3 treatment groups was observed (P < .001). Disease activity and enzyme-linked immunosorbent assay scores were significantly lower in the 400-mg group than in the other groups (P < .05 on day 43, P < .01 on day 85). There was no significant difference in the safety end point among the 3 treatment groups. LIMITATION: Prednisolone at 20 mg/d or more may not be high enough to define steroid resistance. CONCLUSION: Intravenous immunoglobulin (400 mg/kg/d for 5 d) in a single cycle is an effective and safe treatment for patients with pemphigus who are relatively resistant to systemic steroids. Time to escape from the protocol is a useful indicator for evaluation in randomized, placebo-controlled, double-blind studies of rare and serious diseases.
Asunto(s)
Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Pénfigo/tratamiento farmacológico , Método Doble Ciego , Femenino , Humanos , Masculino , Persona de Mediana Edad , Factores de TiempoRESUMEN
BACKGROUND: Recently, human herpesvirus 6 (HHV-6) reactivation has been frequently observed in patients with drug-induced hypersensitivity syndrome or drug rash with eosinophilia and systemic symptoms but not in patients with other types of drug eruptions, eg, Stevens-Johnson syndrome and toxic epidermal necrolysis (TEN). This finding suggests that there is a close relationship between HHV-6 reactivation and drug-induced hypersensitivity syndrome. OBSERVATIONS: A 71-year-old man who was not immunocompromised developed TEN because of zonisamide therapy. After the onset of the rash, significant increases in HHV-6 IgG titers and HHV-6 DNA levels were observed in the patient's whole blood samples, indicating that an HHV-6 reactivation had occurred. Furthermore, the patient's clinical manifestations of TEN appeared to recur concurrently with HHV-6 reactivation. Conclusion Our case suggests that HHV-6 reactivation may also occur in several types of drug eruptions, including Stevens-Johnson syndrome and TEN.
Asunto(s)
Anticonvulsivantes/efectos adversos , Herpesvirus Humano 6/fisiología , Isoxazoles/efectos adversos , Síndrome de Stevens-Johnson/etiología , Activación Viral , Anciano , ADN Viral/sangre , Herpesvirus Humano 6/genética , Herpesvirus Humano 6/inmunología , Humanos , Inmunoglobulina G/sangre , Masculino , Síndrome de Stevens-Johnson/sangre , Síndrome de Stevens-Johnson/patología , ZonisamidaAsunto(s)
Erupciones por Medicamentos/etiología , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Erupciones por Medicamentos/diagnóstico , Erupciones por Medicamentos/patología , Erupciones por Medicamentos/terapia , Glucocorticoides/administración & dosificación , Humanos , Inmunoglobulinas/administración & dosificación , Pruebas Inmunológicas , Activación de Linfocitos , Pruebas del Parche , Intercambio Plasmático , Quimioterapia por Pulso , Factores de TiempoRESUMEN
OBJECTIVE: To provide evidence that lymphomatoid keratosis should be categorized as an epidermotropic subtype of cutaneous lymphoid hyperplasia. DESIGN: Clinicopathological, immunohistochemical, and molecular biological studies of epidermotropic and dermal bandlike infiltrates of lymphocytes without necrotic keratinocytes, Civatte bodies, or Max-Joseph spaces and solar lentigo or seborrheic keratosis adjacent to the lesion, but with epidermal hyperplastic change (clinically scaly plaque) in cases of lymphomatoid keratosis. Conventional histopathologic study as well as immunohistochemical examinations for CD1a, CD3, CD4, CD8, CD20, and CD79a and S100 protein and genotypic examinations were performed. SETTING: University departments comprising 2 sections of dermatology and 1 section of pathology. MAIN OUTCOME MEASURES: Ratio of T to B cells and of CD4(+) to CD8(+) cells, and the phenotype of epidermotropic cells were evaluated. Gene rearrangement of the immunoglobulin heavy chain gene and T-cell receptor (TCR)-beta and TCRgamma genes was also investigated by the polymerase chain reaction method. RESULTS: Immunohistochemically, epidermotropic CD20(+) and/or CD79a(+) cells were present. In the upper dermal lymphocytic infiltrates, the CD3(+)/CD79a(+) cell ratio ranged from 5:5 to 8:2. The CD4(+)/CD8(+) cell ratio was within normal limits. Rearrangements of the TCRgamma gene were demonstrated in 2 cases and of the TCRbeta gene in 1 case. CONCLUSIONS: Our results indicate that lymphomatoid keratosis is a clinically benign keratotic lesion but histologically malignant, simulating mycosis fungoides. Immunohistochemical findings showed a reaction pattern in all cases, but genotypical examination showed some clonal dermatoses. Therefore, "lymphomatoid" keratosis should be classed as a pseudolymphoma, namely, a subtype of cutaneous lymphoid hyperplasia with epidermotropism.