Subject(s)
Connexins/genetics , Hearing Loss, Sensorineural/genetics , Keratoderma, Palmoplantar/genetics , Mutation , Polymorphism, Single Nucleotide , Skin/pathology , Adult , Connexin 26 , Female , Genetic Predisposition to Disease , Hearing Loss, Sensorineural/pathology , Humans , Keratoderma, Palmoplantar/pathology , Phenotype , SyndromeABSTRACT
BACKGROUND: Toxic epidermal necrolysis (TEN) is a severe and potentially lethal drug reaction for which no standard treatment is available. OBJECTIVE: To describe a case series of patients with TEN treated with a single dose of etanercept. METHODS: We observed 10 consecutive patients with TEN. For each patient, we recorded the presence of comorbidities and all the drugs recently started (ie, in the last month). In all cases, 50 mg of etanercept was administered in a single subcutaneous injection. The clinical severity of disease was computed using the SCORe of Toxic Epidermal Necrosis (SCORTEN) scale. Using the probabilities of death linked to each level of SCORTEN score, we calculated the expected probability of death in our patients. Healing was defined as complete reepithelialization, and a time to healing curve was then obtained using the Kaplan-Meier method. RESULTS: All patients promptly responded to treatment, reaching complete reepithelialization without complications or side effects. The median time to healing was 8.5 days. LIMITATIONS: This is a small, uncontrolled case series. CONCLUSION: These preliminary results suggest the possibility that tumor necrosis factor-alfa may be an effective target for control of TEN, a dangerous skin condition for which no effective cure has yet been found.
Subject(s)
Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Immunoglobulin G/therapeutic use , Receptors, Tumor Necrosis Factor/therapeutic use , Stevens-Johnson Syndrome/drug therapy , Adult , Aged , Anti-Inflammatory Agents, Non-Steroidal/adverse effects , Etanercept , Female , Humans , Immunoglobulin G/adverse effects , Male , Middle Aged , Re-Epithelialization , Severity of Illness Index , Time FactorsABSTRACT
BACKGROUND: In dental diseases, significant discrepancies were observed in the oral health-related quality of life evaluation between patients and providers. Few studies have been performed specifically on the impact of oral mucosal diseases on patients' health. OBJECTIVE: We sought to compare the evaluation of the severity of oral mucosal conditions in providers and patients. METHODS: Patients with an oral mucosal condition were recruited at the oral health care unit of a dermatologic hospital. Severity was evaluated both by the physician and by the patient, using a global severity assessment score on a 5-point scale. The 14-item Oral Health Impact Profile was used to evaluate oral health-related quality of life, the 12-item General Health Questionnaire for psychologic problems, and the 20-item Toronto Alexithymia Scale for alexithymia (ie, the difficulty in identifying and expressing feelings). RESULTS: Data were complete for 206 patients. The agreement between patients' and providers' evaluation was very low (Cohen κ = 0.18). Severity was particularly underestimated by the physician in patients with alexithymia (43% compared with 25% of patients with no alexithymia) and with psychologic problems (44% vs 25%). LIMITATIONS: Because of the high number of different conditions, and thus the small figures in each group, it was not possible to analyze the concordance between patient and provider in each single condition. CONCLUSION: Even in the severity assessment of his or her own disease, it is plausible that a patient does not provide a simple clinical evaluation, but includes subjective aspects. It is important for the physician to take into account the severity the patient perceives in making treatment decisions, and in evaluating clinical improvement.
Subject(s)
Mouth Diseases/physiopathology , Mouth Mucosa/physiopathology , Physicians , Quality of Life , Self-Assessment , Adolescent , Adult , Cross-Sectional Studies , Female , Health Personnel , Humans , Male , Middle Aged , Oral Health , Sensitivity and Specificity , Severity of Illness Index , Sickness Impact Profile , Surveys and Questionnaires , Young AdultABSTRACT
Lichen planus is an inflammatory disease of the skin and mucous membranes characterized by vacuolization of basal keratinocytes associated with a prominent junctional lymphocyte infiltrate which comprises T lymphocytes, NK cells, myeloid and plasmacytoid dendritic cells. Basal keratinocyte damage is considered as being a consequence of a lymphocytic cytotoxic attack, mostly mediated by perforin+CD8+ T lymphocytes. NK cells have been described to infiltrate inflamed skin and significantly contribute to the amplification of immune-mediated skin diseases, thanks to their cytotoxic activity and the release of pro-inflammatory cytokines. Here, we investigated the characteristics and functional properties of NK lymphocytes involved in lichen planus. Double staining immunohistochemistry showed a considerable number (6.42 ± 2.2% of the total cellular infiltrate) of CD3-CD56+ cells in early lichen planus lesions, mostly distributed in the papillary dermis and at the epidermal-dermal interface. Skin NK cells isolated from lichen planus lesions belong to the CD56highCD16- subset, are highly positive for perforin and natural cytotoxic receptors NKG2D and NKp44, and, in accordance with their phenotype, are negative for KIRs receptors CD158a and CD158b. Skin CD56highCD16- NK cells display a CCR6+CXCR3+CCR5+ChemR23+ chemokine receptor asset for homing into inflamed skin. In terms of cytokine release, skin CD56highCD16- NK cells are able to secrete IFN-γ, TNF-α and hardly release IL-22, IL-17 and IL-4. Overall, our data propose a pro-inflammatory role of NK lymphocytes in lichen planus.
