Dermatopathic Lymphadenopathy Research Articles

Lymphoma and Other Lymph Node Pathologies Among Adult Patients with Lymphadenopathy in Abakaliki, Nigeria.

Lymphadenopathy is usually due to benign or malignant conditions. It can also be local or systemic in distribution and can involve peripheral or deep-seated lymph nodes. This study aimed to determine the prevalence of lymphoma and the distribution pattern of lymph node pathologies among adult patients who presented with lymphadenopathy and its relationship with age and sex. A retrospective study was conducted, and a record of all cases of lymphadenopathy with histological diagnosis over 5-year period (January 2017 to December 2021) was extracted from Departments of Anatomical Pathology of Alex Ekwueme Federal University Teaching Hospital, Abakaliki. The data generated were analyzed using Statistical Package for Social Sciences (SPSS) software, version 26. One hundred and ninety results were extracted with an age range of 18 to 94 years and a mean age of 41 ± 16 years. They were made up of 75 (39.5%) males and 115 (60.5%) females, with a male-to-female ratio of 1:1.5. The prevalence of lymphoma was 50.0% (95/190). Thirty-five (18.4%) were Hodgkin's lymphoma (HL), while 60 (31.6%) were non-Hodgkin's lymphoma (NHL). Other pathologies manifested by cases of lymphadenopathy include metastatic tumor deposits (38 (20%)), reactive lymphoid hyperplasia (29 (15.3%)), and tuberculous lymphadenitis (18 (9.5%)). Others include sinus histiocytosis (4 (2.1%)), dermatopathic lymphadenitis (5 (2.6%)), and Castleman's disease (1 (0.5%)). About half of all patients who presented with lymphadenopathy were lymphoma with a high prevalence of 50%, and the majority were NHL. Other major causes of lymphadenopathy were metastatic tumor deposits, reactive lymphoid hyperplasia, and tuberculous lymphadenitis. Any case of lymphadenopathy should be properly investigated early for effective management.

Correlation Between Clinical and Histological Features of Dermatopathic Lymphadenopathy: Analysis of 39 Lymph Nodes.

Background. Dermatopathic lymphadenopathy is a well-defined histopathological entity with an underestimated prevalence in the general population. Objective. The present study was conducted to analyze the characteristics of histologically diagnosed cases of dermatopathic lymphadenopathy at our unit. We also aimed to investigate any association between the clinical features of the patients and the histological findings. Methods. A total of 39 lymph node samples from 39 patients diagnosed with dermatopathic lymphadenopathy were included in this prospective cohort study. Results. Thirty-four (87%) patients had a dermatological disorder. The presence of paracortical eosinophils were significantly higher in patients with dermatological disorders (P = .001), while the presence of dilated sinuses was significantly more common in patients without a dermatological disorder (P = .035). The presence of dilated sinuses and medullary histiocytes were significantly more common in patients with lower body surface area involvement of the disease compared to the ones with a higher body surface area (P = .003, P = .034; respectively). Conclusion. Most of the patients included in the study had one of a broad spectrum of undiagnosed dermatological disorders. The clinical significance of the relation between histological and clinical findings in dermatopathic lymphadenopathy remains to be explained. Dermatopathic lymphadenopathy should always be considered in differential diagnoses of patients with persistent lymph node enlargement even when absolute dermatological disorders are not present. Since various skin disorders may be the cause of lymphadenopathy, performing a full-body examination before lymph node excision might prevent unneccessary procedures.

HHV8-Positive Castleman Disease and In Situ Mantle Cell Neoplasia within Dermatopathic Lymphadenitis, in Longstanding Psoriasis.

A 73-year-old man presented with multiple lymphadenopathy. He had a 20-year history of palmoplantar psoriasis evolved to a diffuse erythrodermic picture in the last two years. Topic and systemic medications including prednisolone, acitretin, anti-IL17 (ixekizumab), TNF inhibitor (adalimumab), anti-IL23 (guselkumab), methotrexate, cyclosporine, and phosphodiesterase 4 inhibitor (apremilast) were ineffective. Repeated skin biopsies excluded mycosis fungoides, confirming psoriasis; molecular analysis of T-cell receptor genes ruled out clonality. The axillary lymph node histology documented a dermatopathic lymphadenitis, often associated with chronic cutaneous inflammatory diseases. At an accurate morphological evaluation, features of HHV8-positive multicentric Castleman disease were observed. Moreover, in a few follicles, in situ mantle cell neoplasia was identified. The translocation t(11;14)(q13;q32), characteristic of mantle cell lymphoma, and the monoclonal IGH gene rearrangement were present. HHV8 DNA was identified on plasma sample. Multicentric Castleman disease in psoriatic patients is a rare event and it might be favored by the immunomodulatory treatment in longstanding psoriasis. Multicentric Castleman disease patients are predisposed to developing simultaneous or subsequent lymphoma. In situ mantle cell neoplasia often behaves indolently, although it may progress to overt mantle cell lymphoma. Rituximab achieved a good control of psoriasis. Unfortunately, the patient developed Staphylococcus aureus sepsis for which he is currently on antibiotic therapy.

Dermatopathic lymphadenitis: Cytological diagnosis

Dermatopathic lymphadenopathy usually presents with multiple enlarged lymph nodes. It is often seen in patients with skin diseases with either exfoliative or eczematoid inflammatory erythrodermas. Multiple enlarged lymph nodes usually point to the diagnosis of lymphoma. Fine-needle aspiration cytology helps in reaching the correct diagnosis at the preliminary stage. There are very few case reports on cytological features of this disease in literature. Here, we describe a case of dermatopathic lymphadenopathy in a 15-year-old female with subtle skin disease.

Clinical, histopathologic, and immunoarchitectural features of dermatopathic lymphadenopathy: an update.

Dermatopathic lymphadenopathy is a distinctive form of paracortical lymph node hyperplasia that usually occurs in the setting of chronic dermatologic disorders. The aim of this study is to update our understanding of the clinicopathologic and immunophenotypic features of dermatopathic lymphadenopathy. The study cohort was 50 lymph node samples from 42 patients diagnosed with dermatopathic lymphadenopathy. The patients included 29 women and 13 men with a median age of 49 years (range, 12-79). Twenty-two (52%) patients had a dermatologic disorder, including mycosis fungoides (n = 6), chronic inflammatory dermatoses (n = 13), melanoma (n = 1), squamous cell carcinoma (n = 1), and Kaposi sarcoma in the context of human immunodeficiency virus infection (n = 1). Twenty (48%) patients did not have dermatologic manifestations. Lymph node biopsy specimens were axillary (n = 22), inguinal (n = 21), cervical (n = 4), and intramammary (n = 3). All lymph nodes showed paracortical areas expanded by lymphocytes; dendritic cells, including interdigitating dendritic cells and Langerhans cells; and macrophages. Melanophages were detected in 48 (98%) lymph nodes. Immunohistochemical analysis provided results that are somewhat different from those previously reported in the literature. In the paracortical areas of lymph node, S100 protein was expressed in virtually all dendritic cells, and CD1a was expressed in a significantly greater percentage of cells than langerin (80 vs. 35%, p < 0.0001). These results suggest that the paracortical regions of dermatopathic lymphadenopathy harbor at least three immunophenotypic subsets of dendritic cells: Langerhans cells (S100+, CD1a+(high), langerin+), interdigitating dendritic cells (S100+, CD1a+(low), langerin-), and a third (S100+,CD1a-, langerin-) minor population of dendritic cells. Furthermore, in more than 60% of dermatopathic lymph nodes, langerin highlighted trabecular and medullary sinuses and cords, showing a linear and reticular staining pattern, which could be a pitfall in the differential diagnosis with Langerhans cell histiocytosis involving lymph nodes.

Histopathology and expression of the chemokines CXCL10, CXCL13, and CXCR3 and the endogenous TLR-4 ligand S100A8/A9 in lymph nodes of patients with adult-onset Still\u2019s disease

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease manifesting with a persistent high-spiking fever, a typical rash, and lymphadenopathy. Endogenous factors related to interleukin-1, such as S100A8/A9 and several chemokines including CXCL10, CXCR3, and CXCL13, potentially play roles in its pathogenesis. We describe the histopathological features and chemokine expression pattern in lymph nodes (LNs) of patients with AOSD. Formalin-fixed, paraffin-embedded excisional LN tissues from 48 patients with AOSD were histologically reviewed. CXCL10, CXCR3, CXCL13, and S100A8/A9 expression was evaluated immunohistochemically. The pathology of LN was characterized by paracortical hyperplasia with proliferation of histiocyte, immunoblast, CD8-positive lymphoid cell and blood vessel. Most cases required differential diagnosis from dermatopathic lymphadenitis (n = 16, 33.3%), T cell lymphoma (n = 11, 22.9%), and histiocytic necrotizing lymphadenitis (HNL) (n = 9, 18.8%). The expression levels of CXCL10 and CXCR3 were higher in patients with AOSD than in those with T cell lymphoma, HNL, tuberculous lymphadenitis, and reactive hyperplasia. It is important to recognize the aforementioned histopathologic findings of nodal involvement of AOSD because improper diagnosis and treatment can be avoided. Immunohistochemical staining for chemokines, CXCL10 and CXCR3, may aid in differentiating AOSD from other mimickers.

1040 RESOLUTION OF EXFOLIATIVE DERMATITIS AND ALOPECIA AREATA OPHIASIS WITH THE TREATMENT OF OBSTRUCTIVE SLEEP APNEA

The comorbidity of atopic dermatitis and obstructive sleep apnea can have a reciprocal improvement with the use of continuous positive airway pressure. Patient is a 17 year old African American female with a history of severe exfoliative dermatitis exacerbations secondary to uncontrolled atopic dermatitis (AD) with eczema herpeticum who presented with dermatopathic lymphadenitis, alopecia areata ophiasis, night sweats, nocturia, habitual snoring with hypertrophy of the tonsils to the sleep medicine department for her snoring and nocturnal sweating. Night time sweats produced an intense pruritic response. Nocturia occurred four times a night. Autoimmune and infectious disease work ups were negative. Skin and lymph node biopsies revealed dermatopathic lymphadenitis secondary to exfoliative dermatitis. Treatments included avoidance of suspected AD triggers, emollients, hypo-allergenic soaps and topical steroids. The application of topical steroids resulted in exfoliative dermatitis with eczema herpeticum and staph infections multiple times. Gabapentin improved her pruritic condition and a slight improvement in her skin and scalp was noted. A sleep study revealed the patient to have an Apnea-Hypopnea index of 16.4, excessive sweating and severe snoring were also noted on her sleep study. The mother of the patient declined the use of immune modulating therapies including topical steroids, methotrexate, cyclosporine, and tacrolimus as well as the recommendation for a tonsillectomy. Patient and mother agreed to the use of CPAP and photographing the progression of the disease. No change in the patient’s medications nor consistency were noted. After 10 months of excellent CPAP compliance, photographical evidence as well as physical examination revealed the patient’s hair returned as well as her an improvement in her skin was noted. Nocturia resolved after the use of CPAP. Patient reported two weeks of not using her CPAP machine and reported a new onset of hair loss and worsening of eczema. The use of continuous positive airway pressure has the potential for reducing inflammatory responses in the skin for patients suffering from atopic dermatitis as well as obstructive sleep apnea, reducing the need for immune modulating therapies.

Cytology of HIV Lymphedenopathy- A study of 100 cases

100 patients of lymphadenopathy with human immunodeficiency virus infection were studied by fine needle aspiration cytology of the affected smears. Smears collected were stained by different staining techniques like Papanicolaou taining, Haematoxylin and eosin (H& E) staining, giemsa staining and AFB as well as PAS staining. The maximum number of cases was reported in the age group of 21 to 30 years. Majority of the patients were males. The maximum number of cases had reactive lymphadenitis (52%), followed by tuberculosis (45%) non-Hodgkin’s lymphoma (2%) and dermatopathic lymphadenitis (1%). FNAC is an important diagnostic tool in the evaluation of lymphadenopathy in HIV-positive patients.

Florid dermatopathic lymphadenopathy-A morphological mimic of Langerhans cell histiocytosis.

Key Clinical MessageThe histopathology of reactive florid dermatopathic lymphadenopathy shows overlap with Langerhans cell histiocytosis (LCH) involving the lymph node, which may lead to misdiagnosis. These entities can be distinguished by recognition of the sinus‐based distribution of Langerhans cells in LCH, in contrast to the paracortical distribution of Langerhans cells, pigment‐laden histiocytes, and small lymphocytes in dermatopathic lymphadenopathy.

Cyclin D1 Is Expressed in Neoplastic Cells of Langerhans Cell Histiocytosis but Not Reactive Langerhans Cell Proliferations.

Langerhans cell histiocytosis (LCH) is characterized by frequent activating mutations involving the mitogen-activated protein kinase (MAPK) pathway. Therefore, downstream markers of MAPK pathway activation such as cyclin D1 may be useful as novel diagnostic markers of neoplasia in LCH. The goal of this study was to investigate cyclin D1 expression in LCH and reactive Langerhans cell accumulations using immunohistochemistry on archival tissue. All LCH cases tested (39/39) showed cyclin D1 expression in CD1a/Langerin cells. Most cases (22/39; 56%) showed strong cyclin D1 expression in the majority (≥50%) of lesional cells. Only a few cases (6/39; 15%) showed cyclin D1 expression in a small subset (<20%). Nearly all LCH cases (26/27; 96%) showed p-ERK expression by immunohistochemistry, parallel to cyclin D1 expression. CD1a Langerhans cells in all cases of florid dermatopathic lymphadenopathy and normal skin were negative for cyclin D1, as demonstrated by CD1a/cyclin D1 double staining. The majority of skin specimens (14/18; 78%) with dermatitis-related changes did not show cyclin D1 expression in the CD1a epidermal Langerhans cell aggregates. A minority (4/18; 22%) showed weak cyclin D1 staining in a small subset (5% to 10%) of CD1a Langerhans cells. We conclude that cyclin D1 is ubiquitously expressed in LCH, in keeping with the known near universal MAPK activation in this disease. Further, it is not significantly expressed in reactive Langerhans cell proliferations in lymph node or skin. Therefore, cyclin D1 immunohistochemistry may be useful in excluding non-neoplastic mimics of LCH.

Şiddetli Eritrodermi ve Dermatopatik Lenfadenopati ile Ortaya Çıkan Akut Miyeloid Lösemi Olgusu

Şiddetli Eritrodermi ve Dermatopatik Lenfadenopati ile Ortaya Çıkan Akut Miyeloid Lösemi Olgusu

E11 Golimumab-Induced Dermatopathic Lymphadenopathy: A Curious Side Effect of Golimumab?

E11 Golimumab-Induced Dermatopathic Lymphadenopathy: A Curious Side Effect of Golimumab?

Acute-phase ITIH4 levels distinguish multi-system from single-system Langerhans cell histiocytosis via plasma peptidomics.

BackgroundLangerhans cell histiocytosis (LCH) is a proliferative disorder in which abnormal Langerhans cell (LC)-like cells (LCH cells) intermingle with inflammatory cells. Whether LCH is reactive or neoplastic remains a controversial matter. We recently described Merkel cell polyomavirus (MCPyV) as a possible causative agent of LCH and proposed interleukin-1 loop model: LCH is a reactive disorder with an underlying oncogenic potential and we now propose to test this theory by looking for acute markers of inflammation. We detected MCPyV-DNA in the peripheral blood cells of patients with high-risk organ-type (LCH-risk organ (RO) (+)) but not those with non–high-risk organ-type LCH (LCH-RO (−)); this difference was significant. LCH-RO (−) is further classified by its involvement of either a single organ system (SS-LCH) or multiple organ systems (MS-LCH). In patients with LCH-RO (−), MCPyV-DNA sequences were present in LCH tissues, and significant differences were observed between LCH tissues and control tissues associated with conditions such as dermatopathic lymphadenopathy and reactive lymphoid hyperplasia. Although MCPyV causes subclinical infection in nearly all people and 22 % of healthy adults will harbor MCPyV in their buffy coats, circulating monocytes could serve as MCPyV reservoirs and cause disseminated skin lesions.MethodsPlasma sample from 12 patients with LCH-RO (−) (5 MS-LCH and 7 SS-LCH) and 5 non-LCH patients were analyzed by peptidomics. Mass spectrometry (MS) spectra were acquired and peptides exhibiting quantitative differences between MS-LCH and SS-LCH patients were targeted.ResultsOne new candidate biomarker, m/z 3145 was selected and identified after obtaining a MS/MS fragmentation pattern using liquid chromatography-MS/MS. This peak was identified as a proteolytic fragment derived from inter-alpha-trypsin inhibitor heavy chain 4 (ITIH4, [PDB: Q14624]).ConclusionsPeptidomics of LCH have revealed that the level of acute-phase ITIH4 distinguishes MS-LCH-RO (−) from SS-LCH-RO (−). Acute-phase proteins serve non-specific, physiological immune functions within the innate immune system. LCH may be a reactive disorder with both underlying neoplastic potential of antigen presenting cells harboring BRAF mutations and hyper-immunity of other inflammatory cells against MCPyV infection. Among LCH-RO (−), MCPyV-DNA sequences were present in both MS-LCH tissues and SS-LCH tissues without significant differences. ITIH4 may show that LCH activity or LCH subtypes correlates with the systemic or localized reactions of MCPyV infection.

Dermatopathic Lymphadenopathy With Increased IgG4-Positive Plasma Cells.

Both dermatopathic lymphadenopathy (DL) and immunoglobulin G4-related disease (IgG4-RD) are frequently complicated with allergic diseases. However, the relationship between DL and IgG4-RD is not well known. To clarify this relationship on the basis of clinical and pathological findings, including IgG4-positive (IgG4+) plasma cell infiltration in lymph nodes (LNs) of DL patients, we analyzed LNs of 11 DL patients using immunostaining of IgG, IgG4, forkhead box P3 (FOXP3), transforming growth factor (TGF)-β, interferon (IFN)-γ, and matrix metalloproteinase (MMP)-1, MMP-8, and MMP-13. Toluidine blue staining was also performed to identify mast cells. Of 3 patients with a high ratio of IgG4+/IgG+ cells (>40%) and elevated serum IgG4 levels, 2 developed IgG4-RD, whereas the other patient did not. Of 8 patients with a low ratio of IgG4+/IgG+ cells (<40%) or no infiltration of IgG4+ cells, 5 who could be followed did not develop IgG4-RD. The numbers of mast cells were similar to those of TGF-β-positive cells, and serial sections showed that mast cells possibly produce TGF-β. LNs of DL patients with a high ratio of IgG4+/IgG+ cells had significantly more mast cells and TGF-β-positive cells than those of patients with a low ratio of IgG4+/IgG+ cells or no infiltration of IgG4+ cells. However, no fibrosis was observed in LNs of both groups. IFN-γ was positive in interdigitating dendritic cells, Langerhans cells, and macrophages. MMP-1, MMP-8, or MMP-13 was expressed in macrophages. The lack of fibrosis in LNs may have been due to the production of IFN-γ, MMP-1, MMP-8, or MMP-13. Thus, DL with increased IgG4+ cells seems to be a phenotype of IgG4-RD in LNs.

Increase of DC-LAMP+ mature dendritic cell subsets in dermatopathic lymphadenitis of mycosis fungoides.

Little is known about the immunological milieu of the skin-draining lymph nodes (LNs) in mycosis fungoides (MF). We studied dendritic cell (DC) subsets in the dermatopathic lymphadenitis of MF patients. We immunohistochemically examined DC subsets and their distribution in 16 LN samples from 14 patients with MF (N1 LN, eight patients; N2, four; and N3, four), and we compared them with non-metastatic sentinel LNs from eight patients with melanoma. The number of S-100 protein+ DCs was markedly increased in the LNs from the MF patients and the major component was DC-LAMP+ mature DCs in the outer and paracortex areas, where DC-SIGN+ immature DCs were relatively decreased in proportion. In contrast, DC-SIGN+ cells were relatively increased in proportion compared to DC-LAMP+ cells in the medulla. Although no significant difference was observed in the proportions of CD1a+ or Langerin+ DCs among the N1, N2, and N3 nodes, CD163+ M2-type macrophages were increased in number in the N2 and N3 nodes. Our observations indicate that mature DCs accumulate in the outer and paracortex areas in dermatopathic lymphadenitis and M2-type macrophages might increase in number during disease progression.

Angiomyomatous Hamartoma of Lymph Nodes is Associated With Dermatopathic Lymphadenopathy

Angiomyomatous Hamartoma of Lymph Nodes is Associated With Dermatopathic Lymphadenopathy

Axillary masses in a woman with a history of breast cancer: Dermatopathic lymphadenopathy

The presence of axillary enlarged lymph nodes in the follow-up of a woman with a history of breast cancer should always be thoroughly indagated.Dermatopathic lymphadenopathy presents a specific pathologic pattern found within the lymph nodes and is usually associated with cutaneous rashes.Patients with various skin conditions can develop regional lymphadenopathy, which can result in the asymptomatic enlargement of the lymph nodes, especially in the inguinal, axillary and cervical regions.Dermatopathic lymphadenopathy should be considered in the differential diagnosis also in patients with minimal cutaneous findings.Dermatopathic lymphadenopathy is a benign process and management of these patients consists in simple clinical, mammographic and ultrasonographic follow-up.

Clinicopathologic features and prognosis of T lymphoblastic lymphoma associated with Langerhans cell histiocytosis

To study the clinicopathologic features, immunophenotype and molecular genetic changes of T lymphoblastic lymphoma (T-LBL) associated with Langerhans cell histiocytosis (LCH). Three cases of T-LBL associated with LCH were included. The morphologic characteristics were reviewed along with immunohistochemical profiling using EnVision method and TCR gene rearrangement by PCR. A review of composite lymphoma previously reported in the literature was performed. All three patients were male with the mean age of 61.7 years. One was Hans and the other 2 were Uyguers. All presented with superficial lymph node enlargement. Biopsy of lymph node showed two abnormal cell populations: distended sinus by large, pale histiocytes with nuclear grooves, and the interfollicular region containing immature-appearing cells with irregular nuclei slightly larger than that of small lymphocyte, dispersed chromatin, inconspicuous nucleoli, scant cytoplasm, and scattered mitotic figures. These cells presented in aggregates and small sheets interspersed with normal-appearing lymphocyte. The histiocytes were positive for CD1a, S-100 protein and CD68. The lymphoma cells were positive for CD3, CD7, TdT and CD34. TCR-γ gene rearrangement was detected in one case by PCR technology. One case involved bone marrow with double phenotype acute leukemia. Amongst the 8 including 5 reported cases, there were 4 males and 4 females. The mean age of the patients and the median age were 54 years. Lymphoadenopathy was the most common presentation. Bone marrow was involved in 4 cases. The time of follow-up was 2 to 27 months. The median survival was 5.5 months and the one-year survival rate was 33.3%. Diagnosis of T-LBL and LCH should be based on typical morphology, immunophenotype and molecular genetic findings, with differential diagnoses including Langerhans cell hyperplasia originated from dermatopathic lymphadenopathy. When involving lymph node, extensive sampling supplemented by immunohistochemical staining is important to reach a correct diagnosis. Although coexistent T-LBL and LCH is clonally related, the understanding of its pathogenesis requires further investigation.

Dermatopathic Lymphadenitis Associated With Human Papilloma Virus Infection and Verruca Vulgaris

Here we present a pediatric case of human papilloma virus associated with dermatopathic lymphadenitis (DL). A 5-year-old boy presented to the pediatric oncology clinic with swelling of the neck and warts on his lower jaw. His blood chemistry and complete blood count were normal, as was chest x-ray. HIV, EBV, CMV, and parvovirus serologies were negative. The patient was investigated for malignancy and lymphoma but no association was found. Histopathologic examination of the lymph node and the lesion revealed DL and verruca vulgaris, respectively. DL represents a benign form of reactive lymph node hyperplasia and described in patients with HIV and EBV infections. It is a rare entity described in patients with human papilloma virus infection. To our knowledge, this is the first report of DL in a patient with human papilloma virus infection.

Inappetenz, Nachtschweiß, chronisches Ekzem und inguinale sowie axilläre Lymphknotenpakete bei einem 28-jährigen Patienten

A 28-year-old man presented with loss of appetite, night sweats, eczema, and axillary and inguinal lymph node swelling. The tentative diagnosis of malignant lymphoma was made. To confirm the diagnosis, extirpation of a lymph node and a skin biopsy were performed. Systemic treatment with methylprednisolone resulted in an improvement of eczema and lymph node swelling. Because of the histological findings and clinical course, we diagnosed dermatopathic lymphadenopathy, also known as Pautrier-Woringer syndrome.