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Cureus Aug 2023We present the case of a 37-year-old Haitian male who presented with a seven-month history of skin lesions on his face and extremities, weight loss, intermittent chills,...
We present the case of a 37-year-old Haitian male who presented with a seven-month history of skin lesions on his face and extremities, weight loss, intermittent chills, difficulty in breathing, and bilateral paresthesias in his feet. The lesions were most prominent on the pinnae of the ears. Biopsy of the lesions revealed large, rounded granulomatous infiltrates and histiocytes. Acid fast (Ziehl-Neelsen technique) and Kinyoun stains were positive for numerous acid-fast mycobacteria within the histiocytes. A polymerase chain reaction (PCR) was positive for which confirmed a diagnosis of lepromatous leprosy. Further analysis revealed positive purified protein derivatives (PPD) and QuantiFERON-TB™ test (QIAGEN, Hilden, Germany) with negative chest x-ray and sputum cultures. Labs also revealed vitamin D and G6PD (glucose-6-phosphate-dehydrogenasedeficiency. The patient was started on a combined therapy regimen of rifampin, moxifloxacin, and minocycline. In addition, he was started on vitamin D supplementation. After undergoing treatment for one year, there was notable regression of the patient's cutaneous lesions. Treatment is planned to continue for a total of 24 months. This case exemplifies the successful treatment of Hansen's disease in a patient with a G6PD deficiency. The patient's G6PD deficiency required avoidance of dapsone, which is typically used in the treatment of Hansen's disease. Furthermore, the patient's positive PPD and QuantiFERON-TB tests led to a delay in the treatment in order to rule out active tuberculosis. Left untreated, Hansen's disease has a high morbidity risk. Treatment regimens require careful consideration of coexisting comorbidities.
PubMed: 37664264
DOI: 10.7759/cureus.42816 -
SAGE Open Medical Case Reports 2023This case report describes the first known successful treatment of exfoliative cheilitis with the interleukin-4 receptor alpha antagonist dupilumab in an adult patient....
This case report describes the first known successful treatment of exfoliative cheilitis with the interleukin-4 receptor alpha antagonist dupilumab in an adult patient. With no response to topical tacrolimus, hydrocortisone, dapsone, crisaborole or systemic doxycycline, low dose isotretinoin, or methotrexate, she had resolution with off-label dupilumab after 4 months of treatment.
PubMed: 37560382
DOI: 10.1177/2050313X231193076 -
The Journal of Dermatological Treatment Dec 2023Schnitzler syndrome (SchS) is a rare autoimmune and inflammatory disease mediated by interleukin-1 beta (IL-1β). Recurrent monoclonal gammopathy and chronic urticarial...
Schnitzler syndrome (SchS) is a rare autoimmune and inflammatory disease mediated by interleukin-1 beta (IL-1β). Recurrent monoclonal gammopathy and chronic urticarial rash are the symptoms required for diagnosis according to the Strasbourg criteria. The low prevalence of this syndrome (around 300 cases have been reported) and confusion with other inflammatory disorders may delay the diagnosis for up to 5 years. Although the most effective treatment for SchS is anakinra, some patients do not respond to this treatment. We report a case of SchS in a 64-year-old woman with multiple episodes of fever, severe rash, erythema, arthralgia and dyspnea. The patient was successfully treated with canakinumab after anakinra intolerance and failure of colchicine, prednisone, methotrexate and dapsone. After the first dose of canakinumab the skin wounds rapidly improved and the patient did not require any concomitant treatments. The cause of SchS is still unknown and a differential diagnosis is recommended, especially with adult-onset Still´s disease due to their similar symptoms. Canakinumab, a specific anti-IL-1β antibody, blocks its binding to receptors, thereby preventing IL-1β-induced gene activation and production of inflammatory mediators. Canakinumab has proven to be an effective drug in SchS, providing an alternative to anakinra.
Topics: Adult; Female; Humans; Middle Aged; Schnitzler Syndrome; Interleukin 1 Receptor Antagonist Protein; Antibodies, Monoclonal, Humanized; Exanthema
PubMed: 37551725
DOI: 10.1080/09546634.2023.2242705 -
Frontiers in Immunology 2023Epidermolysis bullosa acquisita (EBA) is a chronic, recurrent autoimmune subepidermal bullous disease characterized by the presence of autoantibodies targeting type VII... (Review)
Review
Epidermolysis bullosa acquisita (EBA) is a chronic, recurrent autoimmune subepidermal bullous disease characterized by the presence of autoantibodies targeting type VII collagen -- basement membrane zone antigen. Standard therapy for EBA includes a combination of systemic corticosteroids and dapsone; however, severe cases may require advanced treatment. The current article reports on four EBA cases in which biologics: infliximab, rituximab (Rtx), and intravenous immunoglobulin (IVIG) were applied. All patients fulfilled the clinical and immunological criteria of EBA: they presented tense blisters healing with atrophic scars on the skin on traumatized areas and in mucous membranes. The diagnosis of EBA was established using numerous techniques: direct and indirect immunofluorescence, salt split skin, ELISA, Fluorescence Overlay Antigen Mapping using Laser Scanning Confocal Microscopy. Since all the patients did not achieve long-term remission on standard treatment (prednisone, dapsone) due to ineffectiveness or side effects of drugs, they eventually were treated with biologics leading to extraordinary skin improvement and stopping the disease for 1-3 years. Biologics in all patients were tolerated very well. No side effects were observed during application as well as multi-month follow-up. The presented cases provide a premise that biological drugs can be a valuable component of EBA therapy.
Topics: Humans; Epidermolysis Bullosa Acquisita; Blister; Autoantibodies; Dapsone; Biological Products
PubMed: 37503352
DOI: 10.3389/fimmu.2023.1214011 -
Dermatology and Therapy Aug 2023Chronic spontaneous urticaria (CSU) is a condition in which wheals, angioedema, and pruritus occur spontaneously and recurrently for at least 6 weeks. The etiology of... (Review)
Review
Chronic spontaneous urticaria (CSU) is a condition in which wheals, angioedema, and pruritus occur spontaneously and recurrently for at least 6 weeks. The etiology of this disease is partially dependent on production of autoantibodies that activate and recruit inflammatory cells. Although the wheals can resolve within 24 h, symptoms have a significant detrimental impact on the quality of life of these patients. Standard therapy for CSU includes second-generation antihistamines and omalizumab. However, many patients tend to be refractory to these therapies. Available treatments such as cyclosporine, dapsone, dupilumab, and tumor necrosis factor alpha (TNFa) inhibitors have been used with success in some cases. Furthermore, various biologics and other novel drugs have emerged as potential treatments for this condition, and many more are currently under investigation in randomized clinical trials.
PubMed: 37386330
DOI: 10.1007/s13555-023-00972-6 -
The American Journal of Tropical... Jul 2023Leprosy is a chronic infection caused by Mycobacterium leprae and Mycobacterium lepromatosis that preferentially compromises peripheral nerve, skin, and mucous...
Leprosy is a chronic infection caused by Mycobacterium leprae and Mycobacterium lepromatosis that preferentially compromises peripheral nerve, skin, and mucous membranes. Colombia achieved the goal of leprosy elimination in 1997. However, in Urabá (Colombia), there has been an increase in leprosy cases beginning in 2020. This case report shows a leprosy relapse 5 decades after the initial infection debuted as a necrotizing erythema nodosum leprosum. Therefore, long-term follow-up of patients with risk factors for relapse is emphasized, especially those treated before the standard of multidrug therapy (dapsone, clofazimine, and rifampin). This case report stresses the importance the importance of clinical follow-up and surveillance of patients with these events of interest for the public health.
Topics: Humans; Leprosy, Lepromatous; Erythema Nodosum; Leprostatic Agents; Drug Therapy, Combination; Leprosy; Recurrence
PubMed: 37253443
DOI: 10.4269/ajtmh.22-0701 -
Chinese Medical Journal Jul 2023
Topics: Humans; Dapsone; Drug Hypersensitivity; Hypersensitivity; Syndrome
PubMed: 37057725
DOI: 10.1097/CM9.0000000000002492