Abdominal Neoplasms ; diagnosis ; diagnostic imaging ; Adolescent ; Autoantibodies ; blood ; Diagnosis, Differential ; Female ; Humans ; Mouth Mucosa ; pathology ; Paraneoplastic Syndromes ; diagnosis ; immunology ; pathology ; Pemphigus ; diagnosis ; immunology ; pathology ; Skin ; pathology ; Tomography, X-Ray Computed

Inflammatory myofibroblastic tumors (IMTs) are benign neoplasms that can occur at different anatomic sites with nonspecific clinical symptoms. A 48-yr-old woman presented with a 2-month history of a relapsed oral ulcer, progressive dyspnea, and a thoracic pain induced by breathing. A tumorous mass was noticed in the right costodiaphragmatic recess on chest computed tomography and magnetic resonance imaging, and the patient underwent a right costotransversectomy with excision of the tumor, which originated from the 12th intercostal nerve. Histology and immunohistochemistry showed that the tumor was an IMT of the intercostal nerve. The patient's postoperative course was not favorable; dyspnea persisted after surgery, and a progressive pulmonary compromise developed. The cause of the respiratory failure was found to be bronchiolitis obliterans, which in this case proved to be a fatal complication of paraneoplastic pemphigus associated with an IMT. This case of IMT of the spinal nerve in the paravertebral region is unique in terms of its location and presentation in combination with paraneoplastic pemphigus, which is rare. A brief review of the heterogeneous theories concerning the pathogenesis, clinicopathological features, and differential diagnosis of this disease entity is presented.
Diagnosis, Differential ; Fatal Outcome ; Female ; Granuloma, Plasma Cell/complications/*pathology ; Humans ; Intercostal Nerves/*pathology ; Lung Diseases/etiology/pathology ; Middle Aged ; Paraneoplastic Syndromes/etiology/*pathology ; Pemphigus/etiology/*pathology ; Peripheral Nervous System Neoplasms/complications/*pathology

Adult ; Asian Continental Ancestry Group ; Biopsy, Needle ; Disease Progression ; Erythema ; complications ; physiopathology ; Female ; Foot Dermatoses ; complications ; diagnosis ; pathology ; Hand Dermatoses ; complications ; diagnosis ; pathology ; Humans ; Immunohistochemistry ; Pemphigus ; diagnosis ; pathology ; Prognosis ; Pruritus ; complications ; physiopathology ; Rare Diseases ; Singapore ; Syndrome

OBJEVTIVETo investigate the expression of transient receptor potential lvanilloidreceptor 4 (TRPV4) protein in pemphigus vulgaris (PV), bullous pemphigoid (BP), dermatitis herpetiformis (DH), and epidermolysis bullosa acquisita (EBA), and explore the role of TRPV4 in the pathogenesis of these diseases.

METHODSTRPV4 protein in normal skin tissues and lesions of PV, BP, DH, and EBA were detected with immunohistochemistry.

RESULTSThe positivity rate of TRPV4 protein expression was 61.90% in PV, 81.81% in BP, 72.22% in DH, and 68.42% in EBA. TRPV4-positive rates in these lesions were significantly lower than the rate in normal skin tissues (93.33%) and also differed significantly among these lesions (PV

CONCLUSINLow TRPV4 expressions may affect the formation and reconstitution of skin connection. TRPV4 may play an role in the occurrence and development of autoimmune bullous skin disorders.

Dermatitis Herpetiformis ; metabolism ; Diagnosis, Differential ; Epidermolysis Bullosa Acquisita ; metabolism ; Humans ; Pemphigoid, Bullous ; metabolism ; Pemphigus ; metabolism ; Skin ; pathology ; TRPV Cation Channels ; metabolism

Anti-Bacterial Agents ; therapeutic use ; Anti-Inflammatory Agents ; therapeutic use ; Female ; Foot Diseases ; diagnosis ; drug therapy ; pathology ; Humans ; Middle Aged ; Neomycin ; therapeutic use ; Pemphigus ; diagnosis ; drug therapy ; pathology ; Prednisone ; therapeutic use

OBJECTIVETo study the pathogenesis and the differential diagnosis of Castleman's disease.

METHODSHistopathology, immunohistochemical staining and clinical courses of 26 cases of Castleman's disease (CD) were studied with follow-up study of 16 cases.

RESULTSThe present study included 6 cases of multicentric type, 20 cases of localized type in the clinical aspects and 19 cases with hyaline vascular type, 4 cases with plasma cell type, 3 cases with mixed type in the histologic aspect. The Multicentric type presented systemic lymphadenopathy, anemia, hyperglobulinemia, hepatosplenomegaly, skin changes, and lung disorder and kidney disfunction, of which 1 case died of respiratory and renal insufficiency. 13 of the 20 localized cases were of the hyaline vascular type, and with good prognosis. 7 of the 20 cases showed paraneoplastic pemphigus associated with hyperglobulinemia (4/7) and lung disease (5/7). The pathologic features composed of proliferation of the mantle zone B cell, follicular dendritic cell, plasma cell and small vessels. In immunohistochemical staining, kappa and lambda light chains were detected in each CD case.

CONCLUSIONSMany diseases are similar to CD clinicopathologically. It is important to make differential diagnosis through pathological study. Castleman's disease is a lymphoproliferative disorder. The pathogenesis of this multicentric disorder may be associated with autoimmune disease.

Adolescent ; Adult ; Aged ; Antigens, CD20 ; analysis ; Castleman Disease ; metabolism ; pathology ; surgery ; Child ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hyperplasia ; Immunohistochemistry ; Leukocyte Common Antigens ; analysis ; Lymph Nodes ; pathology ; Male ; Middle Aged ; Pemphigus ; pathology ; Treatment Outcome

The transition between the main subtypes of pemphigus, pemphigus vulgaris (PV), and pemphigus foliaceus (PF) has rarely been reported. Moreover, the development of PV in a patient with PF is much more unusual than that of PF in a patient with PV. We report a 48-year-old man who presented with cutaneous lesions showing the typical clinical and histological features of PF. Five years later, his skin lesions became extensive and he developed oral erosions. His condition did not respond well to steroids and azathioprine. Histological examination of a vesicle disclosed suprabasal acantholysis in contrast to the subcorneal acantholysis discovered upon initial histological evaluation. Indirect immunofluorescence revealed IgG antikeratinocyte cell surface antibodies at a titer of 1:640. The titer was 1:160 at initial diagnosis. Upon immunoblotting, the patient's serum reacted with 130 kiloDalton (kDa) and 160 kDa proteins, suggesting desmoglein (Dsg) 3 and 1, respectively. We herein report an unusual case of PV that developed from PF during the disease's flare-up.
Time Factors ; Steroids/therapeutic use ; Skin/pathology ; Pemphigus/*diagnosis/pathology ; Middle Aged ; Male ; Immunoglobulin G/chemistry ; Immunoblotting ; Humans ; Fluorescent Antibody Technique, Indirect ; Female ; Disease Progression ; Cell Membrane/metabolism ; Blotting, Western ; Azathioprine/therapeutic use ; Autoantigens/chemistry ; Autoantibodies/chemistry ; Aged ; Adult

OBJECTIVETo study the clinicopathologic features of follicular dendritic cell sarcoma (FDCS) and its differential diagnosis.

METHODSTen cases of FDCS were studied by light microscopy, immunohistochemistry and in-situ hybridization. The clinical features and follow-up information were analyzed.

RESULTSAmongst the 10 cases of FDCS studied, the male-to-female ratio was 1:1. The mean age of the patients was 42 years. Six of them were located in cervical and peritoneal lymph nodes and four in extranodal sites (including tonsil, pelvic cavity, tail of pancreas and spleen). Histologically, the tumor cells had whorled, storiform or diffuse growth patterns. They were spindle in shape and contained syncytial eosinophilic cytoplasm, with round or oval nuclei, vesicular chromatin, distinct nucleoli and a variable number of mitotic figures. Multinucleated tumor giant cells and intranuclear pseudoinclusions were occasionally seen. There was a sprinkling of small lymphocytes and neutrophils within the tumor as well as in the perivascular region. Immunohistochemical study showed that the tumor cells were diffusely or focally positive for CD21, CD23, CD35 and D2-40, but negative for LCA, CD20, CD3, CD1a, HMB45 and CK. Some of them showed EMA, CD68 and S-100 reactivity. In-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) showed positive signals in only one case (which was diagnosed as inflammatory pseudotumor-like FDCS). Of the 7 patients with follow-up information available (duration: 2 months to 39 months; mean: 14 months), 2 cases with paraneoplastic pemphigus died of pulmonary infection at 5 and 7 months respectively. The remaining 5 patients were alive and disease-free after surgical excision (+/- chemotherapy and radiotherapy).

CONCLUSIONSFDCS is a rare low to intermediate-grade malignant tumor. Appropriate application of FDC markers, such as CD21, CD35 and D2-40, would be helpful for arriving at a correct diagnosis. Most cases are associated with good prognosis after surgical treatment, with or without chemotherapy and radiotherapy. Patients with paraneoplastic pemphigus carry a less favorable prognosis.

Adult ; Antibodies, Monoclonal, Murine-Derived ; metabolism ; Dendritic Cell Sarcoma, Follicular ; complications ; metabolism ; pathology ; surgery ; Dendritic Cell Sarcoma, Interdigitating ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Lymph Node Excision ; Lymph Nodes ; pathology ; surgery ; Male ; Meningioma ; pathology ; Middle Aged ; Nasopharyngeal Neoplasms ; pathology ; Paraneoplastic Syndromes ; complications ; Pemphigus ; complications ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Receptors, IgE ; metabolism ; Tonsillar Neoplasms ; metabolism ; pathology ; surgery ; Young Adult