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Pediatric Dermatology May 2020Lipoatrophic panniculitis (LP) is a rare childhood panniculitis characterized by sclerotic, atrophic plaques on the extremities. We present a case of LP diagnosed during...
Lipoatrophic panniculitis (LP) is a rare childhood panniculitis characterized by sclerotic, atrophic plaques on the extremities. We present a case of LP diagnosed during the inflammatory phase that was difficult to distinguish clinically from eosinophilic fasciitis. This report adds to the limited phenotypic spectrum of LP by differentiating the clinical features of disease activity from disease damage and highlighting the importance of biopsy in establishing a diagnosis.
Topics: Adolescent; Atrophy; Biopsy; Child; Connective Tissue Diseases; Humans; Panniculitis
PubMed: 32198786
DOI: 10.1111/pde.14127 -
British Journal of Haematology Jul 2023Frequent germline mutations of HAVCR2, recently identified in subcutaneous panniculitis-like T-cell lymphoma (SPTCL), are associated with an increased risk of...
Frequent germline mutations of HAVCR2, recently identified in subcutaneous panniculitis-like T-cell lymphoma (SPTCL), are associated with an increased risk of hemophagocytic lymphohistiocytosis (HLH). However, SPTCL-HLH represents a challenge because of the difficulties in treatment with poor survival. Its malignant nature, specifically harbouring HAVCR2 mutations, has also been questioned. To better understand its pathology and treatment, we analysed the clinical data of six patients diagnosed at our centre. The median age at onset was 10.5 years (range, 0.8-12.4). Five patients presented with skin lesions of subcutaneous nodules/plaques and/or ulceration. All patients developed HLH; notably, one infant only had HLH without skin involvement. Histopathologically, only two patients were diagnosed with SPTCL and three were reported as panniculitis with no sufficient evidence of lymphoma. Genetically, germline homozygous mutation of HAVCR2 (p.Y82C) was identified in all patients, with a median diagnosis time of 4.6 months. All patients initially received corticosteroids, immunosuppressants or chemotherapy, achieving unfavourable responses. Strikingly, they responded well to ruxolitinib targeting inflammatory cytokines, allowing rapid disease resolution and/or long-term maintenance of remission. The excellent efficacy of ruxolitinib highlights this disease as an inflammatory condition instead of neoplastic nature and indicates novel agents targeting key inflammatory pathways as an encouraging approach for this disease entity.
Topics: Child; Child, Preschool; Humans; Infant; Germ-Line Mutation; Hepatitis A Virus Cellular Receptor 2; Lymphohistiocytosis, Hemophagocytic; Panniculitis
PubMed: 37062931
DOI: 10.1111/bjh.18817 -
Journal of Gastrointestinal and Liver... Jun 2023A 67-year-old man with previous cardiovascular disease was referred to our consultation due to a 5-month history of recurrent epigastric pain. Esophagogastroduodenoscopy...
A 67-year-old man with previous cardiovascular disease was referred to our consultation due to a 5-month history of recurrent epigastric pain. Esophagogastroduodenoscopy and full blood workup presented no alterations. CT scan showed an irregularly shaped mass at the root of the mesentery, measuring 40x25x47mm, with spiculated contours and retractile behaviour (a). Simultaneous densification of the adjacent fat and infracentimetric ganglionic formations scattered throughout the mesentery were shown. Surgical biopsy revealed extensive storiform fibrosclerosis, with the presence of interstitial lymphoplasmocytic infiltrate and obliterative phlebitis (b); the plasma cells had mostly IgG expression, with IgG4:IgG ratio >40% (c), accounting for more than 30- 40 IgG4 plasma cells per field. The serum IgG4 level was 137mg/dL. A diagnosis of IgG4-related sclerosing mesenteritis was made, without other organ involvement. Prednisolone (0.6mg/kg/d) improved partially the abdominal pain, so steroid sparing strategy with off-label rituximab was associated. Due to its low prevalence, the understanding of this entity is scarce, and its diagnosis is challenging. Unlike other manifestations of IgG4-related disease, the intra-abdominal disease is identified in later stages, due to unspecific symptoms. This case aims to raise awareness about this condition as a differential diagnosis of abdominal pain.
Topics: Male; Humans; Aged; Panniculitis, Peritoneal; Immunoglobulin G; Prednisolone; Abdominal Pain; Mesentery
PubMed: 37345601
DOI: 10.15403/jgld-4921 -
BMJ Case Reports Jul 2018While the majority of panniculitides are benign in nature, there are rare instances when panniculitis presents as the initial sign of a complex disease state. We...
While the majority of panniculitides are benign in nature, there are rare instances when panniculitis presents as the initial sign of a complex disease state. We describe a case of panniculitis initially diagnosed as lupus profundus that was challenged when features of haemophagocytic lymphohistiocytosis became apparent. We illustrate how some key clinical features and newer investigations can help differentiate between benign and malignant panniculitis.
Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow; Female; Humans; Lymphoma, T-Cell; Panniculitis; Panniculitis, Lupus Erythematosus; Remission Induction; Stem Cell Transplantation
PubMed: 29970609
DOI: 10.1136/bcr-2018-225170 -
Dermatologic Therapy Nov 2022This study aimed to investigate the relationship between centrifugal lipodystrophy (CLD) and lupus erythematosus panniculitis (LEP), and the efficacy and safety of...
This study aimed to investigate the relationship between centrifugal lipodystrophy (CLD) and lupus erythematosus panniculitis (LEP), and the efficacy and safety of hydroxychloroquine (HCQ) for treating CLD in children. A total of 29 cases clinically diagnosed as CLD (n = 24) and CLD/LEP overlap (n = 5) were enrolled and all were confirmed by skin biopsies of CLD and LEP. The clinicopathological findings, clinical outcomes, and prognosis with the treatment of HCQ between CLD and LEP were compared. All 29 cases (male: female = 1:1.6; median age at onset: 3 years) had cutaneous lesions of centrifugally expanding lipoatrophy, of which five cases overlapped with LEP lesions presented as erythematous indurated plaque (n = 2), subcutaneous nodules (n = 2) and alopecia along Blaschko's lines (n = 1). Antinuclear antibodies were found in six (25.0%) CLD and two (40.0%) overlapped patients (p = 0.597). Histopathologically, of the 24 cases of CLD, 14 (58.5%) exhibited subcutis loss or mild lobular inflammation. Ten (41.7%) cases displayed lobular panniculitis with moderate to dense lymphohistiocytic infiltrate and plasma cells, similar to the five cases of overlap. Small clusters of CD123 positive plasmacytoid dendritic cells were found in 62.5% (5/8) of CLD and 66.7% (2/3) of overlap cases (p > 0.99). HCQ (5 mg/kg/d) treatment showed improvement in 91.3% (21/23) of CLD and all overlap cases, including four cases unresponsive to previous oral glucocorticosteroid treatment. Our findings suggested that CLD and LEP represent a spectrum within the same disease. HCQ (5 mg/kg/d) was effective and safe for treating CLD (age >1.5 years), and early treatment and a regular long-term follow-up are essential.
Topics: Child; Humans; Male; Female; Infant; Panniculitis, Lupus Erythematosus; Hydroxychloroquine; Panniculitis; Lipodystrophy; Alopecia
PubMed: 36100983
DOI: 10.1111/dth.15825 -
JAMA Dermatology Apr 2023
Topics: Humans; Scalp; Panniculitis, Lupus Erythematosus
PubMed: 36790778
DOI: 10.1001/jamadermatol.2022.5631 -
BMJ Case Reports Aug 2014We present the case of a 55-year-old Caucasian man presenting with polyarthritis, weight loss and multiple tender cutaneous nodules. Abnormal liver function tests...
We present the case of a 55-year-old Caucasian man presenting with polyarthritis, weight loss and multiple tender cutaneous nodules. Abnormal liver function tests prompted imaging of the liver which demonstrated liver metastases. Biopsy of the liver lesions confirmed the diagnosis of metastatic pancreatic neuroendocrine carcinoma.
Topics: Biopsy; Diagnosis, Differential; Humans; Male; Middle Aged; Pancreas; Pancreatic Diseases; Panniculitis; Tomography, X-Ray Computed
PubMed: 25150233
DOI: 10.1136/bcr-2014-204290 -
International Journal of Dermatology Apr 2021Panniculitis, or inflammation of the fatty tissue, is an ongoing diagnostic challenge to both dermatologists and pathologists. The basis of the current panniculitis... (Review)
Review
Panniculitis, or inflammation of the fatty tissue, is an ongoing diagnostic challenge to both dermatologists and pathologists. The basis of the current panniculitis classification is histology, whether the inflammation is mainly located in the fibrovascular septa or in the adipose lobules thereafter with or without vasculitis. However, overall, the difficulty rises due to various terminologies and lack of clinical relevance with this classification. In addition to that, the majority of panniculitides have mixed infiltration of both lobular and septal and not a clear-cut distinction. The aim of this article is to provide a novel clinical algorithm to the diagnosis of panniculitis and thus to provide guidelines for all clinicians who may encounter this challenging condition in their clinical practice.
Topics: Adipose Tissue; Humans; Inflammation; Panniculitis; Vasculitis
PubMed: 33040341
DOI: 10.1111/ijd.15224 -
Italian Journal of Dermatology and... Feb 2022
Topics: Humans; Panniculitis; Skin; Stress Disorders, Post-Traumatic; Survivors; Torture
PubMed: 33890731
DOI: 10.23736/S2784-8671.21.06868-7 -
Journal of the European Academy of... Aug 2018Panniculitis occurring in dermatomyositis is uncommon, with only a few cases described in the literature, most of them as case reports.
BACKGROUND
Panniculitis occurring in dermatomyositis is uncommon, with only a few cases described in the literature, most of them as case reports.
OBJECTIVE
This report describes the clinicopathological and immunohistochemical findings in a series of 18 patients with panniculitis associated with dermatomyositis.
METHODS
In each patient, we collected the clinical data of the cutaneous lesions as well as the characteristic clinical and laboratory findings. A series of histopathologic findings was recorded in the biopsy of each patient. A panel of antibodies was used in some cases to investigate the immunophenotype of the infiltrate. Data of treatment and follow-up were also collected.
RESULTS
Of the 18 patients, 13 were female and 5 were male, ranging in age from 13 to 74 years (median, 46.4 years). In addition to panniculitis, all patients presented pathognomonic cutaneous findings of DM and reported proximal muscle weakness prior to the diagnosis of panniculitis. Muscle biopsy was performed in 17 patients and MRI in one, all with the diagnosis of inflammatory myopathy. None of the patients presented any associated neoplasia. Panniculitis lesions were located in the upper or lower limbs. Histopathology showed a mostly lobular panniculitis with lymphocytes as the main component of the infiltrate. Most cases showed also numerous plasma cells and lymphocytes surrounding necrotic adipocytes (rimming) were frequently seen. Lymphocytic vasculitis and abundant mucin interstitially deposited between collagen bundles of the dermis were also frequent findings. Late-stage lesions showed hyaline necrosis of the fat lobule and calcification. Immunohistochemistry demonstrated that most lymphocytes of the infiltrate were T-helper lymphocytes, with some B lymphocytes in the lymphoid aggregates and small clusters of CD-123-positive plasmacytoid dendritic cells in the involved fat lobule.
CONCLUSION
Panniculitis in dermatomyositis is rare. Histopathologic findings of panniculitis dermatomyositis are identical to those of lupus panniculitis. Therefore, the final diagnosis requires clinic-pathologic correlation.
Topics: Adolescent; Adult; Aged; B-Lymphocytes; Biopsy; Dendritic Cells; Dermatomyositis; Female; Humans; Interleukin-3 Receptor alpha Subunit; Male; Middle Aged; Muscle, Skeletal; Panniculitis; T-Lymphocytes, Helper-Inducer; Young Adult
PubMed: 29524269
DOI: 10.1111/jdv.14932