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Clinical Nephrology. Case Studies 2023We present two atypical cases of calciphylaxis presenting with ocular ischemic pathology - both without the hallmark cutaneous manifestations - to raise awareness of...
PURPOSE
We present two atypical cases of calciphylaxis presenting with ocular ischemic pathology - both without the hallmark cutaneous manifestations - to raise awareness of this rare yet highly disabling condition.
OBSERVATIONS
We report two cases of ophthalmic calciphylaxis presenting as (1) anterior ischemic optic neuropathy (AION) and cilioretinal artery occlusion in a 76-year-old woman with pre-dialysis kidney failure, and (2) AION with contralateral central retinal artery occlusion (CRAO) in a 44-year-old man on hemodialysis.
CONCLUSION AND IMPORTANCE
These cases highlight the need for judicious clinical suspicion of calciphylaxis in patients with kidney failure, presenting with microvascular ischemic ophthalmic pathology such as AION or CRAO. Confirmation with temporal artery biopsy is essential to direct targeted individualized multi-disciplinary treatment of calciphylaxis and avoid unnecessary steroid exposure in cases masquerading as giant cell arteritis (GCA).
PubMed: 38169875
DOI: 10.5414/CNCS111088 -
Cureus Nov 2023Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare subtype of non-Hodgkin lymphoma that manifests as panniculitis-like skin lesions. It frequently co-occurs...
Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare subtype of non-Hodgkin lymphoma that manifests as panniculitis-like skin lesions. It frequently co-occurs with hemophagocytic lymphohistocytosis, a life-threatening hyperinflammatory syndrome. The majority of SPTL cases express αβ T-cell receptors (SPTL-AB) and have a favorable prognosis with oral immunosuppressive agents. We report a 37-year-old male patient with HIV infection who had a history of low-grade fever for one year, multiple tender subcutaneous nodules on both thighs, and cytopenia. He received several courses of antibiotics without significant improvement. A random skin biopsy showed lobular panniculitis and he was treated with steroids, but his fever recurred after steroid withdrawal. A second skin biopsy confirmed the diagnosis of SPTL. A bone marrow examination revealed hemophagocytic lymphohistiocytosis. He was successfully treated with cyclosporin A and prednisolone and achieved a complete response after one year of drug discontinuation. Panniculitis-like skin lesions have various etiologies and may present as a clinical mimic of lupus erythematosus panniculitis. The selection of an optimal site for skin biopsy is crucial to avoid erroneous diagnoses and adverse outcomes. We report a case of SPTL in an HIV-positive patient, which illustrates this diagnostic challenge.
PubMed: 38156150
DOI: 10.7759/cureus.49564 -
Rheumatology and Immunology Research Dec 2023We report a case of a 68-year-old woman with chronic and severely destructive arthritis for 8 years with imaging features mimicking psoriatic arthritis (PsA) but...
We report a case of a 68-year-old woman with chronic and severely destructive arthritis for 8 years with imaging features mimicking psoriatic arthritis (PsA) but serological evidence of systemic lupus erythematosus. Both the lupus panniculitis-like rash and the presence of interstitial lung disease were considered manifestations of systemic involvement of SLE.
PubMed: 38125646
DOI: 10.2478/rir-2023-0031 -
Acta Dermato-venereologica Dec 2023
Topics: Humans; Macrophages; Panniculitis; Female; Aged; Drug Eruptions
PubMed: 38124380
DOI: 10.2340/actadv.v103.18855 -
Lupus Science & Medicine Dec 2023To assess the efficacy of anifrolumab, a type-1 interferon receptor subunit-1 monoclonal antibody, in treating refractory cutaneous lupus erythematosus (CLE) and lupus...
OBJECTIVE
To assess the efficacy of anifrolumab, a type-1 interferon receptor subunit-1 monoclonal antibody, in treating refractory cutaneous lupus erythematosus (CLE) and lupus non-specific mucocutaneous manifestations in patients with systemic lupus erythematosus (SLE).
METHODS
A case series comprising four SLE patients with refractory CLE received anifrolumab (300mg) as add-on therapy. Medical history, serological markers and images were collected. Cutaneous Lupus Erythematosus Disease Area and Severity Index-Activity (CLASI-A) was assessed at baseline and post-treatment visits.
RESULTS
Anifrolumab effectively treated refractory chronic cutaneous lupus erythematosus with lupus panniculitis and calcinosis cutis. Anifrolumab demonstrated rapid improvement in generalised discoid lupus, achieving a substantial reduction in CLASI-A from 40 to 8. Switching from belimumab to anifrolumab led to notable improvement in photosensitivity and tumid lupus. Anifrolumab effectively managed refractory subacute cutaneous lupus erythematosus, resulting in remarkable cutaneous improvement and successful tapering of prednisone and mycophenolate mofetil.
CONCLUSION
Anifrolumab demonstrates efficacy in treating refractory CLE subtypes and lupus non-specific mucocutaneous manifestations in SLE patients. Further studies are needed to establish response rates, optimal dosing, and long-term outcomes.
Topics: Humans; Lupus Erythematosus, Systemic; Lupus Erythematosus, Cutaneous; Antibodies, Monoclonal; Prednisone
PubMed: 38114267
DOI: 10.1136/lupus-2023-001007 -
Clinical, Cosmetic and Investigational... 2023Cytophagic histiocytic panniculitis (CHP) is a rare form of nodular panniculitis characterized by clinical manifestations such as skin erythema, nodules, fever,...
BACKGROUND
Cytophagic histiocytic panniculitis (CHP) is a rare form of nodular panniculitis characterized by clinical manifestations such as skin erythema, nodules, fever, pancytopenia, liver failure, plasmacytosis, and hepatosplenomegaly. We report a case of CHP that was initially misdiagnosed as subcutaneous panniculitis-like T-cell lymphoma (SPTCL) but achieved complete remission with a favorable prognosis.
METHODS
A 38-year-old female presented to the dermatology department with a 15-day history of subcutaneous nodules, generalized edema, and continuous fever.
RESULTS
The patient was diagnosed as CHP combined with hemophagocytic syndrome by typical clinical manifestations, low value of SUVmax in positron emission tomography/computed tomography (PET/CT), benign differentiated T cells, negative TCR gene rearrangement, pancytopenia, abnormal coagulation, hypertriglyceridemia, decreased NK cell count, impaired liver function, and the presence of hemophagocytic cells observed in bone biopsy smears.
CONCLUSION
In our case, the patient presented with hemophagocytic syndrome with hemodynamic instability, indicating an intensive treatment is needed. The diagnosis of SPTCL necessitates a meticulous process of differential diagnosis, along with the cautious administration of an aggressive chemotherapy regimen. Extended follow-up is imperative to ascertain the long-term outcomes.
PubMed: 38107669
DOI: 10.2147/CCID.S437208 -
Veterinary Research Communications Apr 2024Leishmaniosis is a vector-borne disease caused by different Leishmania species and transmitted by phlebotomine sand flies under natural conditions in Europe. Scientific...
Leishmaniosis is a vector-borne disease caused by different Leishmania species and transmitted by phlebotomine sand flies under natural conditions in Europe. Scientific information related to Leishmania infantum in dogs is extensive, where less information is available in cats and other companion animals. Recently, first clinical cases of L.infantum infection in domestic ferrrets (Mustela putorius furo) have been described. However, clinical information on leishmaniosis in this species is limited A 15-month-old male neutered domestic ferret was presented with chronic weight loss and the presence of coalescent, erythematous and firm subcutaneous nodules in the ventral abdominal subcutis. A fine-needle aspiration of these nodules was performed and the cytological examination revealed a granulomatous inflammation with the presence of macrophages contained a number of oval organisms with an eccentric nucleus and pale cytoplasm, compatible with Leishmania spp. amastigotes compatible with Leishmania spp. amastigotes. The nodules were surgically excised and histological examination showed a severe multifocal pyogranulomatous panniculitis. Specific immunohistochemistry and qPCR for L. infantum from excised nodules were positive. Additionally, L. infantum was cultured and isolated from the nodules by a fine-needle aspiration. An in-house Western Blot test for L. infantum was performed in serum sample and a positive result was obtained. This is the first reported case of nodular pyogranulomatous panniculitis due to L. infantum infection in a domestic ferret. Further studies are necessary to determine the relevance of domestic ferrets in the transmission of leishmaniosis. The description of new clinical forms of the disease is important as it can assist veterinarians in identifying these new clinical presentations.
Topics: Animals; Male; Cats; Dogs; Ferrets; Leishmania infantum; Panniculitis; Immunohistochemistry; Leishmaniasis, Visceral; Cat Diseases; Dog Diseases
PubMed: 38095745
DOI: 10.1007/s11259-023-10276-x -
Frontiers in Immunology 2023Germline mutation has been reported to be associated with subcutaneous panniculitis-like T-cell lymphoma (SPTCL) leading to Hemophagocytic lymphohistiocytosis (HLH)....
Germline mutation has been reported to be associated with subcutaneous panniculitis-like T-cell lymphoma (SPTCL) leading to Hemophagocytic lymphohistiocytosis (HLH). Several studies have indicated that mutation can cause HLH even in the absence of lymphoma, though the exact mechanism remains unclear. In this article, we reported five cases of mutation-associated HLH. Our analysis revealed an elevated level of IL-1RA in the serum of these patients. Furthermore, we investigated the potential mechanisms underlying HLH associated with mutation based on changes in cytokine levels. Our findings suggest that mutation may represent a distinct genetic defect underlying HLH, differing from traditional primary HLH.
Topics: Humans; Lymphohistiocytosis, Hemophagocytic; Panniculitis; Lymphoma, T-Cell; Mutation; Germ-Line Mutation; Hepatitis A Virus Cellular Receptor 2
PubMed: 38077348
DOI: 10.3389/fimmu.2023.1271324 -
Heliyon Dec 2023Anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis (DM) is a rare disease that can be easily misdiagnosed. Anti-MDA5 dermatomyositis is a subtype of...
Anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis (DM) is a rare disease that can be easily misdiagnosed. Anti-MDA5 dermatomyositis is a subtype of DM. It is distinguished by the presence of significant mucocutaneous characteristics, palmar papules, panniculitis, interstitial lung disease (ILD), and clinically amyopathic dermatomyositis (CADM). When combined with rapidly progressing ILD (RP-ILD), anti-MDA5 DM can be fatal. The literature indicates that nervous system involvement is uncommon in patients with anti-MDA5 DM. We report a case of anti-MDA5 DM with neuropsychiatric abnormalities and ILD. The patient suffered from persistent worsening mental disorders, while his ILD was relatively stable. The patient's neuropsychiatric abnormalities gradually subsided after receiving treatment with glucocorticoids, immunoglobulins, and immunosuppressants, leaving only a slow response and memory loss.
PubMed: 38076042
DOI: 10.1016/j.heliyon.2023.e22935 -
Haematologica Dec 2023The extranodal mature T-cell and NK-cell lymphomas and lymphoproliferative disorders represent a unique group of rare neoplasms with both overlapping and distinct... (Review)
Review
The extranodal mature T-cell and NK-cell lymphomas and lymphoproliferative disorders represent a unique group of rare neoplasms with both overlapping and distinct clinicopathological, biological, and genomic features. Their predilection for specific sites, such as the gastrointestinal tract, aerodigestive tract, liver, spleen, and skin/soft tissues, underlies their classification. Recent genomic advances have furthered our understanding of the biology and pathogenesis of these diseases, which is critical for accurate diagnosis, prognostic assessment, and therapeutic decision-making. Here we review clinical, pathological, genomic, and biological features of the following extranodal mature T-cell and NK-cell lymphomas and lymphoproliferative disorders: primary intestinal T-cell and NK-cell neoplasms, hepatosplenic T-cell lymphoma, extranodal NK/T-cell lymphoma, nasal type, and subcutaneous panniculitis-like T-cell lymphoma.
Topics: Humans; T-Lymphocytes; Killer Cells, Natural; Lymphoproliferative Disorders; Lymphoma, Extranodal NK-T-Cell; Biology
PubMed: 38037802
DOI: 10.3324/haematol.2023.282718