RESUMO
Scleroderma is a heterogeneous group of fibrosing connective tissue disorders of unknown etiology. Morphea is a localized form of scleroderma that occasionally leads to chronic erosions and ulcerations of the skin. Fibrosis, inflammation and chronic ulcerations may eventually promote skin neoplasms; morphea is therefore a rare but established risk factor for cutaneous squamous cell carcinoma (cSCC). We present a review of 16 scleroderma patients: 15 case reports from the literature (identified by a PubMed search) and one case from our clinic of a patient who had developed cSCC, and we discuss potential underlying mechanisms. Statistical analysis revealed that the lower extremities were the body site most commonly affected by cSCC in these scleroderma patients. The mean time interval between the onset of scleroderma and the development of cSCC was ten to twenty years. In patients with morphea, we recommend checking for skin tumors during follow-up examinations as well as a careful risk-benefit analysis when considering the application of immunosuppressants or phototherapy in view of their potential carcinogenic side effects.
Assuntos
Carcinoma de Células Escamosas/etiologia , Esclerodermia Localizada/complicações , Neoplasias Cutâneas/etiologia , Adolescente , Adulto , Idade de Início , Idoso , Carcinoma de Células Escamosas/patologia , Feminino , Humanos , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Fototerapia/efeitos adversos , Fatores de Risco , Esclerodermia Localizada/patologia , Esclerodermia Localizada/terapia , Neoplasias Cutâneas/patologia , Adulto JovemRESUMO
Cutaneous lymphomas comprise different subgroups with distinct biological behavior. Mycosis fungoides, the most common cutaneous lymphoma, presents with patches, plaques, tumors and erythroderma. Therapeutic options depend on stage and comprise local skin-directed treatment in early stages, while later stages and Sézary syndrome require systemic therapies including bexarotene, interferon or brentuximab vedotin. While the rare CD4-positive lymphoproliferation and acral CD8-positive lymphoma present with an invariably indolent course, cutaneous peripheral Tcell lymphomas exhibit an aggressive clinical behavior. Among the subgroup of cutaneous Bcell lymphomas, primary cutaneous marginal zone lymphoma and follicle center cell lymphoma belong to indolent entities with almost unrestricted overall survival, whereas cutaneous large Bcell lymphoma presents with a significant risk of systemic dissemination and is associated with high lethality.
Assuntos
Linfoma de Células B/terapia , Linfoma não Hodgkin/terapia , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/terapia , Micose Fungoide/diagnóstico , Micose Fungoide/terapia , Síndrome de Sézary/diagnóstico , Síndrome de Sézary/terapia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Doença de Hodgkin/mortalidade , Doença de Hodgkin/terapia , Humanos , Linfoma , Linfoma de Células B/mortalidade , Linfoma de Células B/patologia , Linfoma de Zona Marginal Tipo Células B/mortalidade , Linfoma de Zona Marginal Tipo Células B/patologia , Linfoma de Zona Marginal Tipo Células B/terapia , Linfoma não Hodgkin/diagnóstico , Linfoma não Hodgkin/mortalidade , Linfoma Cutâneo de Células T/mortalidade , Micose Fungoide/mortalidade , Síndrome de Sézary/mortalidade , Neoplasias Cutâneas/mortalidade , Taxa de SobrevidaRESUMO
The essential trace element zinc (Zn) plays a key role in the development, differentiation and growth of various human tissues. Zinc homeostasis is primarily regulated by two zinc transporter families (solute-linked carrier families, SLC). Disturbances in zinc metabolism may give rise to disorders that typically manifest themselves on the skin. An autosomal recessive zinc deficiency disorder, acrodermatitis enteropathica is caused by a mutation in the gene coding for the ZIP4 transporter. Due to intestinal malabsorption, affected infants develop clinical signs and symptoms shortly after weaning. Acquired zinc deficiency is a rare but underdiagnosed disorder associated with various etiologies and variable clinical manifestations. Depending on the patient's age, a multitude of causes have to be considered. Given the characteristic periorificial and acral lesions, the clinical diagnosis is usually made by dermatologists. Laboratory confirmation includes measurement of plasma zinc levels and - as a supplementary measure - zinc-dependent enzymes such as alkaline phosphatase. Oral zinc replacement therapy frequently leads to clinical remission within a few days. Depending on the cause, disease management should include cooperation with pediatricians and gastroenterologists in order to guarantee optimal patient care.
Assuntos
Dermatopatias/etiologia , Zinco/fisiologia , Acrodermatite/etiologia , Acrodermatite/patologia , Criança , Pré-Escolar , Diagnóstico Diferencial , Doenças do Cabelo/etiologia , Humanos , Lactente , Síndromes de Malabsorção/complicações , Dermatopatias/patologia , Cicatrização/fisiologia , Zinco/deficiênciaRESUMO
HINTERGRUND: Die Tinea capitis ist die häufigste Dermatomykose des Kindesalters. Das Erregerprofil zeigt unterschiedliche geographische Verteilungsmuster und variiert im Laufe der Zeit. PATIENTEN UND METHODIK: Zwischen 1990-2014 an der Würzburger Universitätsklinik für Dermatologie erhobene Daten von 150 Patienten mit mykologisch gesicherter Tinea capitis wurden hinsichtlich Alter, Geschlecht und Erregerspektrum analysiert und über zwei Zeiträume von jeweils 12,5 Jahren miteinander verglichen. ERGEBNISSE: Obwohl eine Tinea capitis am häufigsten bei Kindern der Altersgruppe zwischen 0 und 5 Jahren diagnostiziert wurde, lag der Anteil betroffener Erwachsener mit 16 % höher als bislang berichtet. Der zoophile Dermatophyt Microsporum canis konnte am häufigsten als Erreger der Tinea capitis identifiziert werden, jedoch war ein Anstieg von Infektionen mit den anthropophilen Pilzen Trichophyton tonsurans und Trichophyton rubrum zu verzeichnen. Tendenziell sank der Anteil zoophiler im Verhältnis zu den anthropophilen Erregern. Im zeitlichen Verlauf zeigte sich eine zunehmende Heterogenität des Erregerspektrums: Dermatophyten wie Trichophyton soudanense und Trichophyton violaceum, Trichophyton anamorph von Arthroderma benhamiae sowie Trichophyton schoenleinii und Microsporum audouinii konnten erstmalig bzw. nach langer Zeit wieder erneut isoliert werden. SCHLUSSFOLGERUNGEN: Wenngleich Microsporum-canis-Infektionen noch dominieren, sind zunehmend anthropophile Erreger nachzuweisen. Angesichts des unerwartet hohen Anteils von Erwachsenen sollte eine Tinea capitis in allen Altersgruppen differenzialdiagnostisch in Betracht gezogen werden.
Assuntos
Tinha do Couro Cabeludo/terapia , Terapia Combinada , Terapias Complementares , Medicina Baseada em Evidências , Alemanha , HumanosRESUMO
Treatment modalities of chronic plaque psoriasis have dramatically changed over the past ten years with a still continuing shift from inpatient to outpatient treatment. This development is mainly caused by outpatient availability of highly efficient and relatively well-tolerated systemic treatments, in particular BioLogicals. In addition, inpatient treatment is time- and cost-intense, conflicting with the actual burst of health expenses and with patient preferences. Nevertheless, inpatient treatment with dithranol and UV light still is a major mainstay of psoriasis treatment in Germany. The current study aims at comparing the total costs of inpatient treatment and outpatient follow-up to mere outpatient therapy with different modalities (topical treatment, phototherapy, classic systemic therapy or BioLogicals) over a period of 12 months. To this end, a retrospective cost-of-illness study was conducted on 120 patients treated at the University Medical Centre Mannheim between 2005 and 2006. Inpatient therapy caused significantly higher direct medical, indirect and total annual costs than outpatient treatment (13,042 versus 2,984 ). Its strong influence on cost levels was confirmed by regression analysis, with total costs rising by 104.3% in case of inpatient treatment. Patients receiving BioLogicals produced the overall highest costs, whereas outpatient treatment with classic systemic antipsoriatic medications was less cost-intense than other alternatives.
Assuntos
Assistência Ambulatorial/economia , Custos Hospitalares/estatística & dados numéricos , Psoríase/economia , Psoríase/terapia , Administração Tópica , Corticosteroides/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Análise de Variância , Anticorpos Monoclonais/uso terapêutico , Feminino , Alemanha , Humanos , Masculino , Pessoa de Meia-Idade , Fototerapia/economia , Análise de Regressão , Retinoides/uso terapêutico , Estudos Retrospectivos , Vitamina D/uso terapêuticoRESUMO
The recently proposed entity of cutaneous follicular helper T (T(FH)) cell lymphoma (CT(FH)CL) harbors distinct clinical and histopathologic features. Here, diagnostic pitfalls are exemplified in a case report and by review of the literature. A 45-year-old patient developed rapidly growing nodules and plaques on upper arms and buttocks, which were initially misdiagnosed as primary cutaneous follicle center B-cell lymphoma (CFCL). Consequently, systemic therapy with rituximab failed and consecutive skin biopsies revealed CT(FH)CL (CD3+CD4+CD10+PD-1+bcl6+ICOS+CXCL13+). Interestingly, the prima vista PD-1-positive and CD10-positive tumor cells lost PD-1 expression in follow-up biopsies while retaining CD10, ICOS and CXCL13 expression. All biopsy specimens displayed an identical clonal T-cell population. Initially, nodules were controlled by local radiotherapy and oral psoralen combined with ultraviolet A (PUVA) therapy. However, disease recurred and progressed rapidly with disseminated nodules. Treatment with bexarotene, methotrexate and polychemotherapy failed to stop disease progression. Finally, modified total skin electron beam radiation resulted in complete remission. Disease stabilized on maintenance therapy with bexarotene in combination with ultraviolet A (UVA) therapy. The case highlights that because of concomitant B-cell stimulation, CT(FH)CL clinicopathologically is prone to be mistaken for CFCL. Importantly, CT(FH)CL might lose PD-1 while retaining CD10 expression in later stages, which may lead to confusion in distinguishing CT(FH)CL from CFCL.