ABSTRACT
Introduction: Dermatitis herpetiformis (DH) is a rare autoimmune, polymorphous blistering disorder, characterized by severe itch or burning sensation, which represents the cutaneous manifestation of celiac disease (CD). The current estimation of DH versus CD is around 1:8 and the affected individuals have a genetic predisposition. Pathogenetically, IgA autoantibodies against the epidermal transglutaminase, an essential constituent of the epidermis, cause DH and are reported to develop through cross-reaction with the tissue transglutaminase, with IgA auto-antibodies causing CD. Immunofluorescence techniques allow for a rapid diagnostics of the disease using patient sera. Evaluation of IgA endomysial deposition with indirect immunofluorescence on monkey oesophagus is highly specific, but moderately sensitive, with some operator-dependent variability. Recently, indirect immunofluorescence with monkey liver as a substrate has been proposed as an alternative, well-functioning diagnostic approach with higher sensitivity in CD. Methods: The objective of our study was to evaluate whether monkey oesophagus or liver tissue shows advantage for diagnostics in patients with DH, compared to CD. To that end, sera of 103 patients with DH (n=16), CD (n=67) and 20 controls ere compared by 4 blinded experienced raters. Results: For DH, we found a sensitivity of 94.2% for monkey liver (ML) compared to 96.2% in monkey oesophagus (ME), while specificity in ML was superior (91.6% versus 75%) to ME. In CD, ML had a sensitivity of 76.9% (ME 89.1%) and specificity of 98.3% (ME 94.1%). Discussion: Our data show that ML substrate is well suitable for DH diagnostics.
Subject(s)
Autoimmune Diseases , Celiac Disease , Dermatitis Herpetiformis , Animals , Fluorescent Antibody Technique, Indirect , Primates , Liver , Immunoglobulin A , HaplorhiniABSTRACT
The anti-inflammatory properties of the topical herbal composition VEL-091604 with gentian root, licorice root, and willow bark extract were assessed in a randomized, prospective, placebo-controlled, double-blind ultraviolet (UV)-erythema test study with 42 healthy volunteers in comparison to 1% hydrocortisone acetate. The efficacy and tolerability of VEL-091604 cream 2 times daily over 2 wk was evaluated in an open-label, prospective proof of concept study in 10 subjects with atopic dermatitis using a lesional SCORAD severity score. In the UV-erythema test VEL-091604 cream significantly reduced inflammation compared to placebo and was as effective as 1% hydrocortisone acetate. The clinical study with atopic subjects revealed a significant and rapid reduction of the lesional SCORAD severity score in the test areas after 1 and 2 wk. No adverse events were recorded. It is concluded that the herbal cream VEL-091604 with licorice root, willow bark, and gentian root extract display anti-inflammatory properties in vivo. It is a promising new treatment option for atopic dermatitis that warrants further investigation in controlled studies.
Subject(s)
Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Dermatitis, Atopic/drug therapy , Gentiana/chemistry , Glycyrrhiza/chemistry , Herbal Medicine , Plant Extracts/therapeutic use , Salix/chemistry , Adolescent , Adult , Anti-Inflammatory Agents, Non-Steroidal/administration & dosage , Anti-Inflammatory Agents, Non-Steroidal/isolation & purification , Child , Child, Preschool , Double-Blind Method , Female , Humans , Male , Middle Aged , Plant Bark/chemistry , Plant Extracts/administration & dosage , Plant Roots/chemistry , Prospective Studies , Skin Cream/therapeutic use , Young AdultABSTRACT
Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant mesenchymal tumor. The incidence of DFSP is 0.1% of all cancers and less than 2% of all soft tissue sarcomas (STS). It can appear at any age, most commonly in individuals aged between 20 and 50 years. The usual location of DFSP is the trunk and it is limited to the dermis. Wide radical excision is the preferred surgical method for therapy of DFSP without distant metastasis. The probability of regional or distant metastases is less than 5%. Patients with positive or close surgical margins have an elevated risk of local recurrence after resection. Adjuvant radiotherapy administered either before or after the surgical treatment reduces the risk of local recurrence.
Subject(s)
Dermatofibrosarcoma/surgery , Skin Neoplasms/surgery , Adult , Combined Modality Therapy , Dermatofibrosarcoma/diagnosis , Dermatofibrosarcoma/pathology , Humans , Middle Aged , Neoplasm Recurrence, Local/pathology , Radiotherapy, Adjuvant , Skin Neoplasms/diagnosis , Skin Neoplasms/pathologyABSTRACT
OBJECTIVE: Medullary thyroid cancer (MTC) is a rare and particularly aggressive type of thyroid cancer of neuroendocrine origin. It occurs in hereditary and sporadic forms and its aggressiveness is related to the clinical presentation and the type of RET mutation. METHODS: In this article, we present the criteria, as reviewed in contemporary literature, regarding lymph node dissection and radical neck dissection in patients with either sporadic or hereditary MTC. RESULTS: Early diagnosis and treatment remains the key to a 100% cure rate. CONCLUSIONS: Routine central lymph node dissection is the minimum procedure recommended for all sporadic and hereditary MTCs. Routine lateral lymph node dissection on either side is necessary when lymph node metastases are found in the central neck compartment.
