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Cureus Apr 2024This case presents a patient with two transposed rare diagnoses developed within 10 years. Thrombotic thrombocytopenic purpura (TTP) and essential thrombocythemia (ET)...
This case presents a patient with two transposed rare diagnoses developed within 10 years. Thrombotic thrombocytopenic purpura (TTP) and essential thrombocythemia (ET) are disease processes that present with opposite clinical and laboratory findings. The patient was diagnosed with ET over a decade after the initial TTP diagnosis when she was found to have extreme thrombocytosis during routine laboratory monitoring. The patient was found to have the calreticulin (CALR) mutation variant of ET which is associated with increased platelet production and she was started on hydroxyurea and aspirin. Subsequent management of the patient's TTP relapses and large fluctuations in her platelet counts necessitated adjustments to the standard ET treatment regimen. There is little to no literature on this rare comorbidity and further investigation is needed for the association between these diseases and modifications to standard treatment to prevent relapses and sequelae.
PubMed: 38716021
DOI: 10.7759/cureus.57716 -
European Journal of Case Reports in... 2024Annular erythema is a rare manifestation of leukocytoclastic vasculitis. It may be associated with various drugs, infections, malignancies, or systemic diseases.
BACKGROUND
Annular erythema is a rare manifestation of leukocytoclastic vasculitis. It may be associated with various drugs, infections, malignancies, or systemic diseases.
CASE DESCRIPTION
A 36-year-old woman with no personal medical history presented with annular erythema with target lesions and petechial purpura. The patient had fever and joint arthralgia. A skin biopsy showed leukocytoclastic vasculitis with IgA deposits on direct immunofluorescence. The diagnosis of immunoglobulin A vasculitis with annular leukocytoclastic vasculitis was made. The patient showed global improvement with topical steroids without relapse.
CONCLUSION
An annular variant of leukocytoclastic vasculitis is a rare manifestation of immunoglobulin A vasculitis.
LEARNING POINTS
Annular erythema may reveal an IgA vasculitis.Annular leukocytoclastic vasculitis is a rare manifestation of IgA vasculitis.This presentation is treated with topical steroids.
PubMed: 38715881
DOI: 10.12890/2024_004278 -
European Journal of Case Reports in... 2024Autoimmune diseases are not contraindications for immune checkpoint inhibitors (ICI) therapy in patients with cancer. However, immune-related adverse events (irAEs) are...
BACKGROUND
Autoimmune diseases are not contraindications for immune checkpoint inhibitors (ICI) therapy in patients with cancer. However, immune-related adverse events (irAEs) are frequently observed in patients receiving ICIs including dermatitis, thyroiditis, colitis, and pneumonitis. Thrombocytopenic purpura, aplasia, and haemophagocytic lymphohistiocytosis (HLH) are rarely observed during ICIs.
CASE DESCRIPTION
We report the case of a male patient with pre-existing untreated HLA B27 and ankylosing spondylitis with gastric cancer and liver metastases. The 79-year-old man was treated with anti-HER2 trastuzumab and anti-PD-1 nivolumab. Seventeen days after the seventh cycle of treatment, he presented at the emergency department with acute fever, confusion, and hypotension. Laboratory results showed pancytopenia, and elevation of ferritin and triglyceride. No infections were detected. Although not seen in a bone marrow biopsy, clinical presentation, and absence of infection, together with an H-score of 263, indicated HLH. The patient was treated with dexamethasone for four days and discharged on a tapering dose of steroids. At the two-month follow-up, clinical presentation was normal and blood test almost normalised. At 8 months, no liver metastases were observed.
CONCLUSIONS
In a patient with a pre-existing autoimmune condition, immunotherapy led to the development of HLH, which was controlled by glucocorticoid. Absence of the feature of haemophagocytosis in the bone marrow biopsy did not exclude the diagnosis, as HLH can occur in the spleen or in the liver. Glucocorticoid therapy did not prevent the anti-cancer effect of ICIs, and liver metastases disappeared 8 months post-HLH. This case warrants further research on the interplay between autoimmunity and ICI response, as well as ICI-induced irAEs.
LEARNING POINTS
Haemophagocytic lymphohistiocytosis (HLH) post seventh cycle of trastuzumab (anti-HER2) and nivolumab (anti-PD-1) was controlled with glucocorticoid.Breach of tolerance was due to immunotherapy-induced HLH in a patient with pre-existing autoimmune condition (HLA B27- positive ankylosing spondylitis).There was a complete disappearance of liver metastases 8 months post-HLH.
PubMed: 38715877
DOI: 10.12890/2024_004370 -
Archivos Espanoles de Urologia Apr 2024The objective of this study was to examine the influence of total intravenous anaesthesia (TIVA) compared to combined intravenous and inhalation anaesthesia (CIIA) in... (Comparative Study)
Comparative Study
OBJECTIVE
The objective of this study was to examine the influence of total intravenous anaesthesia (TIVA) compared to combined intravenous and inhalation anaesthesia (CIIA) in paediatric patients undergoing renal biopsy.
METHODS
A total of 86 children with nephrotic syndrome, acute glomerulonephritis, chronic glomerulonephritis, IgG nephropathy, systemic lupus erythematosus and purpura nephritis were selected from January 2018 to January 2023 in our hospital. All children were divided into the total intravenous anaesthesia group and intravenous inhalational anaesthesia group according to the anaesthesia method. The experimental group comprised 46 children with renal diseases who underwent static aspiration compound anaesthesia during renal biopsy at our hospital from January 2018 to January 2023. Conversely, the control group included 40 children with renal diseases who underwent total intravenous anaesthesia during renal biopsy at the hospital within the same period. Hemodynamic parameters, such as mean arterial pressure (MAP), heart rate (HR), and oxygen saturation (SPO), were assessed at four different time points: Before anesthesia induction (T0), during anesthesia induction (T1), after anesthesia induction (T2), and at the conclusion of the surgery (T3). Puncture success rate, time to renal puncture, time to get out of bed, postoperative recovery from anaesthesia (including time to postoperative awakening and time to return to spontaneous respiration) and incidence of adverse anaesthetic reactions were also included.
RESULTS
We observed notable variations in HR and MAP at T2 and T3, as well as SPO levels, duration of awakening from anaesthesia and time taken to resume spontaneous respiration between the two groups at T2 ( < 0.05). No statistically significant variances were detected between the two groups concerning adverse reactions to anaesthesia, puncture success rate, duration to renal puncture and time to mobilisation from bed ( > 0.05).
CONCLUSIONS
In conclusion, compared with the total intravenous anaesthesia, the implementation of the sedation-aspiration-combined anaesthesia in renal biopsy in children with renal disease features less haemodynamic fluctuation, better postoperative anaesthesia recovery and does not increase the incidence of adverse reactions.
Topics: Humans; Child; Male; Female; Anesthesia, Intravenous; Anesthesia, Inhalation; Kidney; Biopsy; Child, Preschool; Kidney Diseases; Adolescent; Postoperative Complications
PubMed: 38715163
DOI: 10.56434/j.arch.esp.urol.20247703.31 -
Cureus Apr 2024Cryoglobulinemia is an uncommon condition characterized by the presence of cryoprecipitable immune complexes in circulation, leading to clinical symptoms like purpura,...
Cryoglobulinemia is an uncommon condition characterized by the presence of cryoprecipitable immune complexes in circulation, leading to clinical symptoms like purpura, muscle weakness, and joint pain. Specifically, mixed cryoglobulinemia involves the formation of these complexes due to rheumatoid factors, mainly IgM, occasionally IgG or IgA. Previously, Hepatitis C (HCV) was a common cause of mixed cryoglobulinemia, as the chronic HCV infection triggered immune responses that resulted in cryoglobulin formation. However, the emergence of direct-acting antivirals (DAAs) for HCV treatment has shifted the landscape, with autoimmune and lymphoproliferative disorders becoming more prominent etiological factors for mixed cryoglobulinemia. This case report features a 67-year-old woman with a history of Hepatitis C-related cirrhosis. She presented at the emergency department with signs of septic shock and widespread joint pain, particularly in the knees, shoulders, and neck. Effective sepsis management was achieved using antibiotics, albumin infusion, and midodrine. Nonetheless, significant cervical and bilateral knee pain persisted. Further examination uncovered hypocomplementemia and positive results for rheumatoid factors (IgA, IgM, IgG) and cryoglobulin agglutination, confirming the diagnosis of mixed cryoglobulinemia. This case emphasizes the importance of considering mixed cryoglobulinemia in chronic Hepatitis C patients displaying fatigue and joint pain, even in the absence of the traditional clinical manifestations. Moreover, the case underscores the dual benefits of DAA treatment for Hepatitis C in individuals with mixed cryoglobulinemia by achieving viral eradication and alleviating cryoglobulinemia-related symptoms, thus preventing further organ damage.
PubMed: 38707103
DOI: 10.7759/cureus.57684 -
Cureus Apr 2024Since the beginning of the pandemic, many skin manifestations associated with COVID-19 have been reported. New reports show that COVID-19 can lead to autoimmune diseases...
INTRODUCTION
Since the beginning of the pandemic, many skin manifestations associated with COVID-19 have been reported. New reports show that COVID-19 can lead to autoimmune diseases (AIDs) and autoinflammatory diseases, especially dermatological.
METHODS
A prospective study was conducted by the dermatology department of the Centre Hospitalier Universitaire Ibn Rochd (CHU Ibn Rochd) of Casablanca in Morocco since the beginning of the pandemic including 18 patients with COVID-19-related skin manifestations.
RESULTS
Eighteen cases were collected with confirmed SARS-CoV-2 infection. The mean COVID score was 0.7. A percentage (94.44%) of the cases had general symptoms. Skin involvement was variable, mainly maculopapular rash (44.44%), purpura (27.77%), urticaria, varicelliform rash, necrotic lesions of the face, and pityriasis rosea Gibert (PRG)-like lesions. Mucosal involvement was found in 50%. Viral reactivation was found in 5.55%. Telogen effluvium was found in 22.22%. Moreover, AID was triggered by COVID-19: lupus (11.11%), associated with antiphospholipid syndrome (APL Sd) (5.55%), psoriasis (11.11%), alopecia, and pemphigus. Severe toxidermia was potentiated by SARS-CoV-2 infection (22.22%): Stevens-Johnson syndrome (Sd), acute generalized exanthematous pustulosis (APEG), and drug reaction with eosinophilia and systemic symptoms (DRESS).
CONCLUSION
The interest of this work is to report our experience during the COVID-19 pandemic to understand some pathophysiological mechanisms of its dermatological manifestations and to draw the attention of clinicians to the link of this infection with autoimmune and autoinflammatory diseases and toxidermia.
PubMed: 38707102
DOI: 10.7759/cureus.57587 -
The American Journal of Case Reports May 2024BACKGROUND Pigmented purpuric dermatosis (PPD) is a rare disease that is poorly understood but thought to result from inflammation of the capillaries causing...
BACKGROUND Pigmented purpuric dermatosis (PPD) is a rare disease that is poorly understood but thought to result from inflammation of the capillaries causing extravasation of erythrocytes into the soft tissue. There are a variety of potential causes, including medications, such as acetaminophen and aspirin, abnormal humoral immunity, and excessive exercise. Although benign, PPD can be bothersome to patients due to associated pruritus, weeping, and poor cosmetic results. Treatment of this lesion is difficult, with no standardized regimen and a tendency for relapse once treatment is discontinued. CASE REPORT This case reports on a 77-year-old man who presented to an outpatient dermatology clinic with bilateral lower extremity edema with associated weeping and erythema for 1 year. A biopsy was conducted and resulted as PPD. He began treatment with excimer laser therapy after conservative and topical treatment options failed, with resolution of symptoms without recurrence for approximately 1 year. CONCLUSIONS PPD is notoriously difficult to treat, and historic treatment options include topical corticosteroids, oral supplements, and immunomodulators, all of which come with a range of adverse effects. However, new literature supports the use of phototherapy to treat PPD, with varying results. Previously implemented options include but are not limited to phototherapy with psoralen plus ultraviolet A, narrow band ultraviolet B, advanced fluorescence technology pulsed light, and fractional non-ablative 1540-nm erbium: glass laser, each with varying degrees of success. This case discusses the successful treatment of recalcitrant PPD with excimer laser therapy and maintenance of remission for approximately 1 year.
Topics: Humans; Male; Aged; Purpura; Lasers, Excimer; Pigmentation Disorders
PubMed: 38698617
DOI: 10.12659/AJCR.942853 -
Cureus Mar 2024Intracerebral hemorrhage (ICH) is a rare and severe complication of immune thrombocytopenic purpura (ITP) that can be spontaneous. Viral illnesses, other infections,...
Intracerebral hemorrhage (ICH) is a rare and severe complication of immune thrombocytopenic purpura (ITP) that can be spontaneous. Viral illnesses, other infections, autoimmune disorders, and medications can cause ITP. ITP causes a significant decrease in platelet levels, increasing bleeding risk. ITP can be treated by steroids, intravenous immunoglobulin, plasmapheresis, platelet transfusion, biological agents, and splenectomy. ICH treatment involves the treatment of underlying ITP, as well as any neuro-interventional procedures needed. In this case report, we look at the presenting symptoms and treatment course of an interesting case of ICH in a patient who developed ITP after a viral upper respiratory infection.
PubMed: 38690508
DOI: 10.7759/cureus.57284