Mixed Panniculitis Research Articles

Early-onset recurrent panniculitis as a phenotype of NLRC4-associated autoinflammatory syndrome: Characterization of pathogenicity of the p.Ser445Pro NLRC4 variant.

Monoallelic NLRC4 gain-of-function variants cause an inflammasomopathy with diverse clinical forms including infantile enterocolitis, recurrent macrophage activation syndrome, cold-induced urticaria-like lesions (or familial-cold autoinflammatory syndrome, FCAS4), and painful subcutaneous nodules. Here, we identified a large family with six consecutive generations affected. Genetic analyses detected the heterozygous p.Ser445Pro NLRC4 variant in three patients, which has been previously reported in a Dutch family with FCAS4. We aimed to describe the clinicopathological features and the functional consequences of the detected NLRC4 variant. Patients presented an early-onset (3months-6 years) inflammatory disease characterized by recurrent panniculitis, fever and arthralgia. Histopathological examination showed perivascular and interstitial lymphohistiocytic infiltrates in the dermis and mixed panniculitis. Functional analysis supported the conclusion that the p.Ser445Pro NLRC4 variant leads to a constitutive activation of NLRC4-inflammasome and increased plasma levels of IL-18. Prompt recognition of early-onset panniculitis through clinicopathological examination and laboratory biomarkers may allow targeted therapies.

Etiological factors and histopathological features in erythema nodosum: a 6-year retrospective cross-sectional study

Erythema nodosum (EN) is the most common type of panniculitis. The most frequent etiological factors are streptococcal pharyngitis, sarcoidosis, Behçet's disease, and tuberculosis. Our objective was to identify the etiological factors and to evaluate the patients' clinical, laboratory, and histopathological findings. Eighty-eight patients diagnosed with EN at our clinic between 2013 and 2019 were evaluated retrospectively. Sixty-five patients were evaluated histopathologically. The patients' ages ranged between 17 and 76 (mean age: 41.91 ± 13.07 years). EN was 7.8 times more frequent in women. Patients presenting with idiopathic EN were significantly older than secondary cases (p < 0.05). Sixty-one patients (69.3%) had an underlying disease (secondary EN). The most common etiological factors were upper respiratory tract infections (n = 26), followed by Behçet's disease (n = 20). Septal panniculitis was present in 89.2% of cases evaluated histopathologically. Mixed or lobular panniculitis was present in 35.7% of Behçet's disease patients with EN-like lesions. Vasculitis was also noted in 35.7% of Behçet's disease patients. Our data confirm the predominance of upper respiratory tract infections and Behçet's disease among patients with EN in Turkey. Behçet's disease patients presenting with EN-like lesions may show mixed panniculitis and vasculitis, whereas classic EN patients predominantly show septal panniculitis.

EPA-1802 – Munchausen syndrome - comprehension of factitial dermatitis

Introduction Munchausen Syndrome (MS) is the most severe and chronic psychiatric disorder of factitious disorders. As a rule, external incentives are absent, being the core motivation the willingness to assume the sick role. Objectives/Aims Literature review on topic Munchausen Syndrome, with presentation of a clinical case. Methods Performed a literature review. Clinical data was collected in clinical process of the patient. Results The authors report a case of 47 years-old women, presenting marital and family stable relationship and good professional status, with MS. Was admitted to inpatient psychiatry unit in June 2013, by history of 177 episodes admissions at Dermatology and Surgery Departments, with 346 days of hospitalization, due to mixed panniculitis of infectious etiology with repeated abscesses, which started with a bite of a dog in the lower limb, 4 years before. In this period, was resistant to all treatments, only improving during hospitalizations. Medical conditions were excluded. During hospitalization showed depressive mood and refuted the self-inflicted nature of the injuries. Was referred for outpatient psychiatric unit, with the diagnosis of factitious dermatitis, medicated with venlafaxine 75 mg daily, Lorazepam and Flurazepam. Conclusions We highlight the case by dermatological repercussions and by psychodynamic myriad, given that the patient was raised by her grandparents after being abandoned by her mother at 20 months, which continues to avoid the rapprochement. As enshrined in the literature, the skin can be a vector for expression of certain psychosomatic unconscious conflicts and these in turn may have a cardinal role in the MPS.

Mixed Panniculitis Responding to Cyclosporin-A with a 12-Month Follow-up: A Case Report

Panniculitides represent a heterogeneous group of inflammatory diseases involving subcutaneous fat. Subcutaneous fat is normally organized into adipose cells, adipocytes, and septa of connective tissue. The inflammation involving such tissues can be more represented in septa (septal panniculitis) or in lobules (lobular panniculitis) or be equally distributed in both (mixed panniculitis). A bioptical study is necessary in order to discern between different forms. Vascular involvement is also different in such diseases, as it can interest arteries, or veins, or both. Different grades of fat necrosis can also be observed, such as adipocytes without nuclei, lipophagic necrosis, liquefactive fat necrosis, microcystic fat necrosis, ischaemic fat necrosis. Panniculitis can be idiopathic or secondary to other diseases such as systemic sclerosis, rheumatoid arthritis, systemic erithematous lupus and many others. Therapies usually vary on the single patient but the general orientation leads to the use of immunosuppressive drugs such as thalidomide, corticosteroids, cyclosporin-A, hydroxychloroquine and cyclophosphamide. We report a case of a 19-year-old female affected by primary mixed panniculitis, associated with fever and deep asthenia, that resolved in a few weeks and was maintained with oral cyclosporin-A.

Panniculitis: clinical overlap and the significance of biopsy findings

Panniculitides are well-recognized clinicopathologic entities but the non-specificity of their clinical and pathological features often troubles the diagnostician. This study retrospectively evaluates the clinical overlaps and the significance of histological findings among various panniculitides. The clinical evaluation in 55 panniculitides cases suggested the diagnosis of typical erythema nodosum (EN) in 26 cases, atypical EN in 17 cases, atypical nodular vasculitis (NV) in two cases, soft tissue infection in five cases and five cases remained unclassified. Skin biopsy evaluation provided definite panniculitis diagnosis in 53 cases including EN (28 cases), leukocytoclastic vasculitis (seven cases), NV (four cases), superficial thrombophlebitis (ST) (two cases), eosinophillic panniculitis (EP) (three cases), infection-related panniculitis (five cases), and one case each of erythema nodosum leprosum (ENL), lupus panniculitis (LP), pancreatic fat necrosis and acne conglobata with two cases remaining unclassified. Histologically, 'predominantly septal' and 'mixed panniculitis' were the chief inflammatory patterns in EN cases, while mixed panniculitis was seen in most LCV cases and predominantly lobular and mixed panniculitis in NV cases. Biopsy evaluation of a panniculitis lesion is usually significant, and the application of a combination of histologic features rather than of a single biopsy finding or an inflammatory pattern is helpful in the diagnosis of panniculitis.

Subcutaneous Nodules at Injection Sites: Histopathological Pitfalls in Diagnosis

Subcutaneous swellings uncommonly occur at vaccination or desensitization injection sites and may present long after the procedure. Such lesions can show a range of histopathological appearances that are not well recognized, often leading to erroneous diagnoses. We describe the clinicopathological features of 13 cases (12 adults, 1 child) with subcutaneous swellings presenting at injection sites (upper arm n12, thigh n1). In the cases studied the histological appearances were varied, although predominantly the subcutis was involved. Six cases showed a mixed panniculitis with focal fat necrosis and fibrosis. Four cases demonstrated a pseudolymphomatous pattern, mimicking a B cell lymphoma. One case was reminiscent of lupus profundus and 3 other cases resembled deep granuloma annulare. Importantly, all cases contained histiocytes with violaceous granular cytoplasm, representing ingested aluminium salts employed for adsorption of antigens in the injection preparations. Energy dispersive x‐ray microanalysis in 8 cases confirmed a positive aluminium peak. Electron microscopy in 3 cases showed crystalline material in the histiocytes. In conclusion, the spectrum of histological appearances in so called aluminium granuloma can be misleading and it is important to identify the characteristic histiocytes of this condition, for otherwise erroneous diagnoses, with serious clinical implications, can follow.

Paniculitis lúpica asociada a lupus eritematoso sistémico

Abstract.—Lupus panniculitis or lupus erythematosus profundus in a mixed panniculitis with a predilection for the face, proximal extremities, trunk or buttocks. It occurs in 2% of lupus erythematosus patients. The serum antinuclear antibody is ussally positive in about 70% of case, but only 25%- 50% fulfil the American Rheumatism Association criteria for systemic lypus erythematosus.We report a 54-year-old women who developed firm, erythematous nodules located mainly on the face, trunk and buttocks. Histological examination showed a mixed panniculitis with hyaline fat necrosis and calcification. Laboratory tests revealed lymphopenia and anemia. ANA and anti-DNA were positive. For several months the patient had also been suffering from arthralgia with synovitis of the wrist and several interphalangeal joins. Overall, these clinical and serology findings were consistent with the diagnosis of lupus panniculitis associated with mild systemic lupus erythematosus. Treatment consisted of synthetic antipaludian medication and low dose systemic corticosteroids, under which the lesions regressed except one of them, which remained as a solitary plaque, on buttocks, with an important calcification.