Direct Immunofluorescence for Dermatologic Disorders:A Single-Center Retrospective Analysis for 11 Years / 대한피부과학회지

Background@#Direct immunofluorescence (DIF) is a histochemical technique used to detect tissue-bound autoantibodies and diagnose various immune-mediated skin diseases. @*Objective@#This study aimed to evaluate the sensitivity of DIF for each disorder, and the consistency between clinical, histopathological, and DIF results. @*Methods@#A retrospective study was conducted in 194 patients who underwent skin biopsy and DIF testing at our hospital between January 2011 and December 2021. An antibody panel against immunoglobulin G (IgG), IgA, IgM, C3, C1q, and fibrinogen was used. The concordance rate and κ-coefficient between the clinical, histopathological, and DIF results were evaluated. @*Results@#DIF was observed to be positive in 87 cases; 51 cases of immune-mediated bullous diseases, seven cases of connective tissue diseases (CTDs), 25 cases of vasculitis, and four cases of other diseases. The overall sensitivity of DIF for immune-mediated bullous diseases was 71.8%, which was higher than that of histopathology (64.8%). In CTDs and vasculitis, the overall sensitivities of DIF were 30.4% and 65.8%, respectively, which were lower than those of histopathology (73.9% and 84.2%, respectively). In addition, good concordance among the clinical, histological, and DIF results was observed. @*Conclusion@#DIF is a useful diagnostic method, especially for immune-mediated bullous diseases, lupus erythematosus, and Henoch-Schonlein purpura. However, in other CTDs and vasculitis cases, the sensitivity of DIF is relatively low. Therefore, the diagnostic value of DIF along with clinical and histopathological findings will be maximized only when the DIF test is performed for appropriate diseases.

Spontaneous Improvement of Eosinophilic Dermatosis of Hematologic Malignancy Concurrent with Follicular Lymphoma after Rituximab and Bendamustine Therapy / 대한피부과학회지

Eosinophilic dermatosis of hematological malignancy (EDHM) is a rare condition associated with various hematologic malignancies, characterized by pruritic skin eruptions. We present a case of a 66-year-old woman with follicular lymphoma who developed urticarial and vesicular lesions indicative of EDHM following chemotherapy.The diagnosis was confirmed through histological analysis, revealing eosinophilic infiltration. Treatment included additional chemotherapy sessions and topical corticosteroids, resulting in complete resolution of skin lesions and lymphoma. EDHM requires careful differentiation based on clinical and histological findings. The pathogenesis remains unclear, but addressing underlying hematologic malignancies appears crucial in management. Early recognition of EDHM is essential for appropriate intervention due to its limited therapeutic options.

A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm with Mutations in DNMT3A, TET2, SRSF2, and ATRX Genes / 대한피부과학회지

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive lymphoma with an overall incidence of 0.04 cases per 100,000 people. BPDCN is a hematopoietic clonal neoplasm that originates from plasmacytoid dendritic cell precursors. A 63-year-old man presented with multiple erythematous nodules over his whole body, including his face, trunk, and both upper and lower extremities that appeared 1 month ago. Skin biopsy showed diffuse dermal infiltration by monomorphic atypical lymphocytes with large, irregular nuclei and scant cytoplasms. Immunohistochemical staining was positive for CD4, CD56, and CD123. The karyotype test showed abnormalities in male chromosomes 47, XY, ＋8 [2]/46, and XY [25], and mutations in DNMT3A, TET2, SRSF2, and ATRX genes were identified in a next-generation sequencing (NGS)-based acute myeloid leukemia gene panel test. The patient was diagnosed with BPDCN and treated with a KALLA 1406 regimen; however, he died on the 17th day of treatment.

Additive Antinociception between Intrathecal Sildenafil and Morphine in the Rat Formalin Test

The possible characteristics of spinal interaction between sildenafil (phosphodiesterase 5 inhibitor) and morphine on formalin-induced nociception in rats was examined. Then the role of the opioid receptor in the effect of sildenafil was further investigated. Catheters were inserted into the intrathecal space of male Sprague-Dawley rats. For induction of pain, 50 microliter of 5% formalin solution was applied to the hindpaw. Isobolographic analysis was used for the evaluation of drug interaction between sildenafil and morphine. Furthermore, naloxone was intrathecally given to verify the involvement of the opioid receptor in the antinociception of sildenafil. Both sildenafil and morphine produced an antinociceptive effect during phase 1 and phase 2 in the formalin test. The isobolographic analysis revealed an additive interaction after intrathecal delivery of the sildenafil-morphine mixture in both phases. Intrathecal naloxone reversed the antinociception of sildenafil in both phases. These results suggest that sildenafil, morphine, and the mixture of the two drugs are effective against acute pain and facilitated pain state at the spinal level. Thus, the spinal combination of sildenafil with morphine may be useful in the management of the same state. Furthermore, the opioid receptor is contributable to the antinocieptive mechanism of sildenafil at the spinal level.

A Case of Mesenteric Castleman's Disease with Systemic Involvement / 대한소아혈액종양학회지

Castleman's disease (CD) is a lymphoproliferative disorder of unknown origin and rarely occurs in children. It has been further classified into two major subgroups: solitary or localized and multicentric CD. Furthermore, there are two main histological variants: hyaline-vascular, plasma cell types. Clinically, hyaline-vascular type is rarely associated with systemic symptoms, but the plasma cell type is frequently associated with the constitutional symptoms of fever, malaise, night sweat and the abnormal laboratory markers. Surgical excision of the affected lymph node plays an important role in the treatment of this disease. We encountered a case of the hyaline-vascular type CD located in the mesentery with systemic involvement. The clinical and biochemical abnormal findings improved after surgical resection of the involved lymph node.