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Introduction: Lymphangiomatosis is a rare abnormal proliferation of lymphatic vessels involving multiple organs like the brain, lung, heart, spleen, liver, and bones. Lymphangiomas constitute 5.6% of all benign tumors in infancy and adulthood. Case presentation: We report a case of a young lady who presented with constitutional symptoms and progressive dyspnea. Her medical history is significant for muco-cutaneous albinism, diffuse hemangiomas of the bone and viscera, and consumptive coagulopathy status post-splenectomy. After initial investigations, she was found to have right-sided pleural effusion. Pleural fluid analysis indicated chylothorax. She had multiple drainages of the pleural fluid done, and afterward, ligation of the right thoracic duct was performed with a trial of sirolimus, which improved her chylothorax. Clinical discussion: Several case reports have reported positive outcomes with sirolimus in the treatment of lymphangiomatosis. However, larger controlled studies are needed to confirm these findings. Conclusion: Sirolimus is promising as a medical treatment for diffuse pulmonary lymphangiomatosis.
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Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition known to affect multiple organ systems. While its manifestations are diverse, pulmonary involvement, especially of the pleura, remains less common. We report the case of a 99-year-old Saudi male with a medical history of diabetes mellitus, chronic kidney disease, and hypertension. He presented with dyspnea and syncope, with radiological findings revealing pleural effusion and a mass in the right hemidiaphragm. Laboratory investigations highlighted elevated serum IgG4 levels, and histopathological evaluation confirmed the diagnosis of IgG4-RD. Notably, the patient's thoracic histopathology differed from typical IgG4-RD presentations, emphasizing the variability of the disease. This case underscores the significance of recognizing IgG4-RD as a potential cause of unexplained pleural effusion. It also highlights the need for a comprehensive diagnostic approach, integrating laboratory values, histopathological findings, and clinical context. Given the potential variability in thoracic IgG4-RD histopathology, clinicians should maintain a heightened awareness of this condition to avoid missed diagnoses.
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BACKGROUND: Cobblestoning is the most common complication of minipunch grafting. OBJECTIVE: To assess the value of silicone gel application following minipunch grafting and the histopathological and immunohistochemical changes in cases with cobblestoning. METHODS: Minipunch grafting was performed in two similar vitiligo lesions in 27 cases with stable vitiligo. After healing, silicone gel was applied twice daily on one lesion while zinc oxide ointment was applied to the other lesion serving as a control. Four biopsies were taken from each case; normal, vitiliginous skin before treatment and the two treated lesions 3 months after therapy, for histopathology and immunohistochemical staining for MMP9 & tenascin-C. RESULTS: Repigmentation occurred in 19 cases (70.7%). The number of lesions showing excellent and good response was significantly higher on the silicone gel side (p < .001). In 20 cases, cobblestoning either occurred or was absent on both sides. Histopathologically, cobblestoning was similar to hypertrophic scarring. Both markers were elevated after therapy on both sides with no significant difference in percentage change between lesions showing positive and negative cobblestoning. CONCLUSION: Silicone gel application after minigrating improved the final response with no significant effect on the occurrence of cobblestoning. Cobblestoning resembled hypertrophic scarring histopathologically.
