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Anais Brasileiros de Dermatologia Jul 2019Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and...
Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.
Topics: Adult; Autoantibodies; Desmosomes; Diagnosis, Differential; Female; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Immunotherapy; Male; Middle Aged; Pemphigus; Skin; Surveys and Questionnaires
PubMed: 31365654
DOI: 10.1590/abd1806-4841.20199011 -
Arthritis Research & Therapy Jul 2019A specific subset of psoriasis patients is characterized by subclinical inflammatory changes. These patients frequently present with arthralgia and have a higher risk to... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
A specific subset of psoriasis patients is characterized by subclinical inflammatory changes. These patients frequently present with arthralgia and have a higher risk to develop psoriatic arthritis (PsA). We hypothesized that IL-17A inhibition in this subset of patients can intercept the link between skin and joint disease and resolves pain and inflammatory changes.
METHODS
Psoriasis, but no PsA, patients were included in the open prospective exploratory Interception in very early PsA (IVEPSA) study. Patients had to have nail or scalp involvement or a high psoriasis area severity index (PASI) (> 6) as well as inflammatory or erosive changes in MRI or CT. Patients received treatment with the anti-interleukin (IL)-17A antibody secukinumab over 24 weeks. Clinical assessments of skin and joint disease were done at baseline and after 12 and 24 weeks, MRI and CT at baseline and after 24 weeks.
RESULTS
Twenty patients were included, 85% of them reporting arthralgia and 40% had tender joints at the examination. Eighty-three percent had at least one inflammatory lesion in the MRI, most of them synovitis/enthesitis. Skin disease (PASI: p < 0.002; BSA: p < 0.003) and arthralgia (VAS pain: p < 0.003) significantly improved after 24 weeks. Total PsAMRIS (p = 0.005) and synovitis subscore (p = 0.008) also significantly improved. Erosions and enthesiophytes did not progress, while bone mass in the distal radius significantly (p = 0.020) increased after 24 weeks.
CONCLUSIONS
These data suggest that very early disease interception in PsA is possible leading to a comprehensive decline in skin symptoms, pain, and subclinical inflammation. IVEPSA therefore provides rationale for future early interventions with the concept to prevent the onset of PsA in high-risk individuals.
TRIAL REGISTRATION
Trial registry name PSARTROS; trial registry number: NCT02483234; June 26, 2015.
Topics: Adult; Antibodies, Monoclonal, Humanized; Arthritis, Psoriatic; Female; Follow-Up Studies; Humans; Interleukin-17; Magnetic Resonance Imaging; Male; Middle Aged; Prospective Studies; Risk Factors; Severity of Illness Index; Tomography, X-Ray Computed
PubMed: 31349876
DOI: 10.1186/s13075-019-1957-0 -
Annals of Dermatology Apr 2017Morgellons disease is a rare disease with unknown etiology. Herein, we report the first case of Morgellons disease in Korea. A 30-year-old woman presented with a 2-month...
Morgellons disease is a rare disease with unknown etiology. Herein, we report the first case of Morgellons disease in Korea. A 30-year-old woman presented with a 2-month history of pruritic erythematous patches and erosions on the arms, hands, and chin. She insisted that she had fiber-like materials under her skin, which she had observed through a magnifying device. We performed skin biopsy, and observed a fiber extruding from the dermal side of the specimen. Histopathological examination showed only mild lymphocytic infiltration, and failed to reveal evidence of any microorganism. The polymerase chain reaction for was negative in her serum.
PubMed: 28392653
DOI: 10.5021/ad.2017.29.2.223 -
Indian Journal of Dermatology 2015A 40-year-old male human immunodeficiency virus (HIV) -positive patient on highly active antiretroviral therapy (HAART) developed bilateral cervical lymphadenopathy with...
A 40-year-old male human immunodeficiency virus (HIV) -positive patient on highly active antiretroviral therapy (HAART) developed bilateral cervical lymphadenopathy with fine needle aspiration cytology (FNAC) showing yeast cells of Penicillium marneffei. The adenopathy disappeared after 9 months of itraconazole therapy at a dose of 200 mg/day. Seven years later and 2 days following second-line HAART, the patient presented with generalized papulonodules and ulceronecrotic lesions. Biopsy of the skin lesion revealed plenty of yeast forms dividing by binary fission morphologically resembling Penicillium marneffei. Significant improvement was observed at 2 weeks of starting itraconazole 400 mg/day. After 3 months, the dose was reduced to 200 mg/day and advised to continue for 6 months. Penicillosis presenting initially in the form of cervical adenopathy and later, developing typical skin lesions rapidly progressing to ulcerative and necrotic erosions may be due to continued immunosuppression followed by immune reconstitution inflammatory syndrome (IRIS).
PubMed: 25814751
DOI: 10.4103/0019-5154.152607