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Skin Health and Disease Apr 2024Myelodysplastic syndrome (MDS) may present with specific skin lesions, such as leukaemia cutis, which is a well known poor prognostic marker of leukaemia with a high...
Myelodysplastic syndrome (MDS) may present with specific skin lesions, such as leukaemia cutis, which is a well known poor prognostic marker of leukaemia with a high risk of acute leukaemic transformation. However, less is known regarding non-specific cutaneous manifestations of MDS including the prevalence, types and their prognostic and therapeutic significance, which we aimed to determine through this systematic review. We searched electronic databases (PubMed, Medline and EMBASE) from inception up to 26 January 2023 for studies reporting cutaneous manifestations of MDS. Eighty eight articles (case reports = 67, case series = 21), consisting of 134 patients were identified. We identified 6 common cutaneous manifestations: neutrophilic dermatoses ( = 64), vasculitis ( = 21), granulomatous ( = 8), connective tissue disease (CTD) ( = 7; composed of dermatomyositis ( = 5), cutaneous lupus erythematosus ( = 1), and systemic sclerosis ( = 1)), panniculitis ( = 4), immunobullous ( = 1), and other ( = 29). Cutaneous features either occurred at time of MDS diagnosis in 25.3%, preceding the diagnosis in 34.7% (range 0.5-216 months), or after diagnosis in 40.0% (range 1-132 months). Prognosis was poor (40.2% death) with 34.1% progressing to acute myeloid leukaemia (AML). 50% of those with MDS who progressed to AML had neutrophilic dermatoses ( = 0.21). Myelodysplastic syndrome was fatal in 39.2% of neutrophilic dermatoses (median time from onset of cutaneous manifestation: 12 months), 50% of vasculitis (7.5 months), 62.5% of granulomatous (15.5 months) and 14.3% of CTD (7 months). Recognition of patterns of cutaneous features in MDS will improve early diagnosis and risk stratification according to subtype and associated prognosis.
PubMed: 38577044
DOI: 10.1002/ski2.323 -
BMJ Case Reports Apr 2024We report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a young man presenting with fever and facial swelling. He had pancytopenia and...
We report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a young man presenting with fever and facial swelling. He had pancytopenia and hemophagocytic syndrome (HPS) on evaluation. The histopathological examination of skin punch biopsy from the face and chest wall showed SPTCL. Given the associated HPS, he was started on steroid and multidrug chemotherapy following which he had symptomatic improvement.
Topics: Male; Humans; Lymphohistiocytosis, Hemophagocytic; Panniculitis; Lymphoma, T-Cell; Skin; Angioedema; Fever
PubMed: 38569732
DOI: 10.1136/bcr-2023-256615 -
Cureus Mar 2024Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a very rare cutaneous manifestation found in patients with acute pancreatitis. We report the case of a...
Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a very rare cutaneous manifestation found in patients with acute pancreatitis. We report the case of a 44-year-old man presenting with erythematous, painful lesions on the lower extremities and ankle swelling. The rheumatology service was consulted for possible erythema nodosum. Extensive workup revealed elevated lipase and amylase levels, and computed tomography of the abdomen and pelvis revealed acute pancreatitis with necrotizing lesions and peripancreatic thoracic collections. There were also changes of chronic pancreatitis. The original skin manifestations were eventually identified as pancreatic panniculitis by skin biopsy. The patient was treated for pancreatitis and pleural effusions, and his skin and joint symptoms completely resolved. Pancreatic panniculitis with polyarthritis is rare but may be the first presenting symptom of pancreatic disease. Rheumatology may be consulted for these patients especially if there are only skin and joint manifestations and no abdominal pain. Misdiagnosis of pancreatitis can lead to poorer outcomes and delay in care. Therefore, pancreatic disease should be on the differential for any patient with panniculitis and polyarthritis.
PubMed: 38562324
DOI: 10.7759/cureus.55362 -
JAAD Case Reports Apr 2024
PubMed: 38510836
DOI: 10.1016/j.jdcr.2023.12.025 -
The Journal of Dermatology Mar 2024Erythema nodosum (EN) may be idiopathic or secondary, and usually resolves naturally within 1-2 months. In atypical EN cases, the rash extends beyond the lower limbs...
Erythema nodosum (EN) may be idiopathic or secondary, and usually resolves naturally within 1-2 months. In atypical EN cases, the rash extends beyond the lower limbs to the upper limbs and trunk, and histopathological findings may be accompanied by vasculitis in addition to septal panniculitis. Few studies have examined the differences in the clinical characteristics of patients with EN based on rash distribution. We retrospectively examined whether there was a correlation with clinical information, such as the presence or absence of underlying diseases, by classifying the patients into two groups: the lower limbs group (the EN rash was confined to the lower limbs) and the beyond lower limbs group (the EN rash appeared beyond the lower limbs). Among the 86 adult patients diagnosed with EN at the Dermatology Department of Fujita Medical University between 2015 and 2020, there were 65 cases of the lower limbs group and 21 cases of the beyond lower limbs group. The frequency of underlying diseases was significantly higher in the beyond lower limbs group (76.2%, 16 cases) than in the lower limbs group (40.0%, 26 cases; P < 0.005). Vasculitis was more notable in the beyond lower limbs group (P < 0.05). Significantly higher vasculitis was noted in the EN group with underlying diseases (30.2%, 13 cases) than in the idiopathic EN group without underlying diseases (11.6%, 5 cases; P < 0.05). Neutrophil extracellular traps were positive in approximately 40% of cases in both groups. In the beyond lower limbs group, the possibility of severe cases with underlying diseases, vasculitis, and inflammation must be considered for effective treatment.
PubMed: 38507514
DOI: 10.1111/1346-8138.17195 -
Dermatology Online Journal Dec 2023Pancreatic panniculitis is an uncommon manifestation of pancreatic disease, affecting approximately 2-3% of patients with acute or chronic pancreatitis [1]. Its accurate...
Pancreatic panniculitis is an uncommon manifestation of pancreatic disease, affecting approximately 2-3% of patients with acute or chronic pancreatitis [1]. Its accurate diagnosis is crucial for effective treatment as skin manifestations usually precede systemic symptoms of pancreatitis. The diagnosis of pancreatic panniculitis requires identifying and confirming pancreatic disease in addition to the typical histopathological findings of lobular panniculitis without vasculitis. Herein, we present a patient with pancreatic panniculitis, underlying acute pancreatic failure, and kidney injury.
Topics: Humans; Panniculitis; Pancreatic Diseases; Diagnosis, Differential
PubMed: 38478676
DOI: 10.5070/D329663005 -
Dermatology Online Journal Dec 2023Discoid lupus erythematosus (DLE), a subtype of chronic cutaneous lupus may be observed in a linear pattern. A 21-year-old woman with history of chronic granulomatous...
Discoid lupus erythematosus (DLE), a subtype of chronic cutaneous lupus may be observed in a linear pattern. A 21-year-old woman with history of chronic granulomatous disease state presented to our clinic for a chronic six-year skin eruption on her left eyebrow, left cheek, and left forehead. A punch biopsy of involved left forehead skin was performed and revealed perivascular and periadnexal lymphohistiocytic infiltrate without features of morphea or panniculitis, confirming the histopathologic changes of cutaneous lupus erythematous. The patient was diagnosed with linear DLE, mimicking en coup de sabre, within Blaschko lines. The pathogenesis for DLE in association with chronic granulomatous disease is ambiguous; however, X-linked lyonization is crucial for both conditions and may explain cooccurrence of disease states.
Topics: Humans; Female; Young Adult; Adult; Scleroderma, Localized; Granulomatous Disease, Chronic; Skin; Lupus Erythematosus, Discoid; Panniculitis
PubMed: 38478669
DOI: 10.5070/D329662998 -
Cancers Feb 2024Cutaneous T-cell lymphomas (CTCLs) are a group of lymphoid neoplasms with high relapse rates and no curative treatment other than allogeneic stem cell transplantation... (Review)
Review
Cutaneous T-cell lymphomas (CTCLs) are a group of lymphoid neoplasms with high relapse rates and no curative treatment other than allogeneic stem cell transplantation (allo-SCT). CTCL is significantly influenced by disruption of JAK/STAT signaling. Therefore, Janus kinase (JAK) inhibitors may be promising for CTCL treatment. This study is a systematic review aiming to investigate the role of JAK inhibitors in the treatment of CTCL, including their efficacy and safety. Out of 438 initially searched articles, we present 13 eligible ones. The overall response rate (ORR) in the treatment with JAK inhibitors in clinical trials was 11-35%, although different subtypes of CTCL showed different ORRs. Mycosis fungoides showed an ORR of 14-45%, while subcutaneous-panniculitis-like T-cell lymphoma (SPTCL) displayed an ORR ranging from 75% to 100%. Five cases were reported having a relapse/incident of CTCL after using JAK inhibitors; of these, three cases were de novo CTCLs in patients under treatment with a JAK inhibitor due to refractory arthritis, and two cases were relapsed disease after graft-versus-host disease treatment following allo-SCT. In conclusion, using JAK inhibitors for CTCL treatment seems promising with acceptable side effects, especially in patients with SPTCL. Some biomarkers, like pS6, showed an association with better responses. Caution should be taken when treating patients with an underlying autoimmune disease and prior immunosuppression.
PubMed: 38473222
DOI: 10.3390/cancers16050861 -
European Journal of Dermatology : EJD Dec 2023
Topics: Humans; Panniculitis; Lymphoma, T-Cell; Skin Neoplasms; Diagnosis, Differential; Lymphoma, T-Cell, Cutaneous
PubMed: 38465570
DOI: 10.1684/ejd.2023.4626 -
European Journal of Dermatology : EJD Dec 2023
Topics: Humans; Gastrointestinal Stromal Tumors; Pyrazoles; Pyrroles; Panniculitis; Mutation; Gastrointestinal Neoplasms; Protein Kinase Inhibitors; Antineoplastic Agents; Receptor, Platelet-Derived Growth Factor alpha; Triazines
PubMed: 38465561
DOI: 10.1684/ejd.2023.4632