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PubMed1992

[Panniculitis in children].

Panniculitis is an inflammation of subcutaneous fat responsible for fairly uniform clinical features including nodules, plaques or swelling located in the subcutaneous tissue, occasionally progressing to atrophy of subcutaneous fat. Some forms of panniculitis specific of childhood are responsible for readily recognizable clinical patterns. Examples include acute neonatal fat necrosis, sclerema neonatorum, cryopanniculitis, steroid-induced panniculitis, and toxoplasmosis-related multinodular fat necrosis of Royer syndrome. With the exception of erythema nodosum, the cause of panniculitis is identified upon histologic examination of a deep skin biopsy which ensures the differentiation of infectious nodules, vasculitis nodules, and apparently primary forms of nodular panniculitis fitting the description of Weber-Christian syndrome, Rothmann-Makaï syndrome, histiocytic cytophagic panniculitis or lupus panniculitis.

Biopsy↗

PubMed1999

Musculoskeletal manifestations with panniculitis--a hospital based study on 62 patients in Kuwait.

The study describes 62 patients seen over a period of 4 years mainly presenting with an acute inflammatory ankle arthritis/periarthritis associated with panniculitis. Based upon the clinical characteristics of panniculitis two distinct categories could be defined. These included patients with characteristic lesions of erythema nodosum, seen in 51 (82%), the idiopathic form in 29 (57%), and secondary EN in 22 (43%). Associated conditions included Behçet's disease, oral contraceptive pills, pregnancy and penicillin treatment. Patients clinically not having typical EN was seen in 11 (18%). In this group the histopathology showed erythema induratum in 4, cutaneous-polyarteritis nodosa with minimal panniculitis in 3, Weber-Christian disease, cytophagic histiocytic panniculitis, and 'mixed' lobular and septal variety of panniculitis in 1 each. (In 1 patient the disease was not panniculitis but cutaneous variant of granuloma annulare). It is concluded that in a hospital setting EN associated with acute musculoskeletal symptoms, mainly ankle arthritis/periarthritis or an acute peripheral polyarthritis, was a common rheumatological problem in Kuwait. However, other forms of panniculitides also presented with musculoskeletal manifestations requiring accurate histopathological diagnosis for appropriate treatment.

Adolescent↗

PubMed1981

The histopathology of lupus erythematosus panniculitis.

Lupus erythematosus panniculitis is a clinical variant of lupus erythematosus in which the main pathologic process involves the deep corium and subcutaneous tissue. We reviewed twenty-nine cases of lupus panniculitis, as well as the cases previously reported in the literature. The histopathologic changes in lupus panniculitis are characterized by a lymphocytic panniculitis, hyaline degeneration of the fat, hyaline papillary bodies, and lymphoid nodular structures in the lower dermis and subcutaneous tissue. Direct immunofluorescence can be important in supplementing the histopathologic study of lupus panniculitis. Lesions of discoid lupus erythematosus are seen in 21% of cases. When this disorder exists in the absence of other typical cutaneous or systemic lesions, the diagnosis of lupus erythematosus has been questioned. We believe that the histopathologic findings of this entity are alone sufficient for a diagnosis of lupus panniculitis, even in the absence of cutaneous or systemic lesions.

Adult↗

PubMed1999

Neutrophilic lobular (pustular) panniculitis associated with rheumatoid arthritis: a case report and review of the literature.

Rheumatoid nodules, which affect the subcutis around joints, are the most frequent specific cutaneous lesions of rheumatoid arthritis (RA). Panniculitis is a rarely reported and nonspecific complication of RA. We report a 42-year-old woman with seropositive RA who presented with a 2-month history of lower leg panniculitis. Biopsy of a leg nodule showed a lobular neutrophilic infiltrate with lipophages and central basophilic necrosis. In addition, focal changes of lipomembranous fat necrosis indicative of ischemic damage were identified at the margins of the lobular infiltrate. Neutrophilic lobular panniculitis is commonly detected in panniculitis secondary to bacterial infections, pancreatitis, and factitial causes. However, this pattern of panniculitis has also been reported in some cases of erythema nodosum-like lesions found in Behçet disease or bowel bypass syndrome and in rare cases of seropositive RA. These reported histologic findings fall into the spectrum of neutrophilic vascular reactions described by Jorizzo and Daniels for RA-associated dermatoses. In view of these findings. RA and related neutrophilic dermatoses (e.g., Behçet disease) should be included in the differential diagnosis of neutrophilic lobular panniculitis.

Adult↗

PubMed2005

Sclerosing panniculitis is associated with pulmonary hypertension in systemic sclerosis.

BACKGROUND: There have been no reports of patients with sclerosing panniculitis and systemic sclerosis (SSc). OBJECTIVES: To evaluate the incidence of sclerosing panniculitis in patients with SSc, and to investigate the clinical features of such cases. METHODS: In total, 128 patients with SSc treated at our clinic were investigated retrospectively. RESULTS: SSc patients with sclerosing panniculitis had pulmonary hypertension (PH), especially isolated PH, at a significantly higher incidence than those without. Among the SSc patients with PH, those with sclerosing panniculitis had pulmonary infarctions at a higher incidence than those without. CONCLUSIONS: Our results suggest that thrombosis caused by venous hypertension of the leg may be the main cause of PH in patients with SSc and sclerosing panniculitis. Sclerosing panniculitis may be a useful marker of PH in patients with SSc.

Adult↗

PubMed2005

Sclerodermic linear lupus panniculitis: report of two cases.

Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or 'overlap' forms between lupus erythematosus panniculitis and localized scleroderma have been reported. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. The 2 patients (of the 14 patients with lupus erythematosus panniculitis seen by us since 1990) were females with a young age at the onset of disease (median, 25 years). In 1 case, evolution into systemic lupus erythematosus with severe renal involvement occurred whereas the other patient, who had a spontaneous abortion and exhibited anticardiolipin antibodies, should be followed and screened for the emergence of antiphospholipid syndrome. Thus, the clinical behavior of this variant seems to be more aggressive, as compared with the usual course of lupus erythematosus panniculitis, which is considered to be a benign disease, although some reports have suggested that its prognosis is not always favorable. The linear distribution could be the clinical hallmark of such a unique, 'sclerodermic' subset of lupus erythematosus panniculitis.

Adult↗

PubMed2000

CT evaluation of mesenteric panniculitis: prevalence and associated diseases.

OBJECTIVE: This study was undertaken to assess the prevalence of mesenteric panniculitis on CT and to describe its appearance and associated diseases. SUBJECTS AND METHODS: A total of 7620 consecutive abdominal CT examinations were prospectively evaluated for features common to mesenteric panniculitis such as a well-delineated inhomogeneous hyperattenuated fatty mass at the mesenteric root, envelopment of mesenteric vessels, and no evidence of invasion of the adjacent small-bowel loops that may be displaced. RESULTS: CT findings of mesenteric panniculitis were seen in 49 patients (0.6%). We found a female predominance. Mesenteric panniculitis coexisted with malignancy in 34 patients and with benign disorders in 11 patients. In the remaining four patients, mesenteric panniculitis, verified on histology, was considered to be responsible for the patients' clinical manifestations; no other abnormality was identified. Soft-tissue nodules (n = 39) and a fatty halo surrounding vessels and nodules (n = 42) were observed in most patients. Follow-up abdominal CT examinations in 29 of the 49 patients showed changes in only one patient. CONCLUSION: CT findings of mesenteric panniculitis may be seen in patients undergoing abdominal CT for various symptoms.

Adolescent↗

PubMed2004

[Successful treatment of Weber-Christian panniculitis with cyclosporin-A].

The inflammatory disease of the subcutaneous fatty tissue are called panniculitis. The panniculitis has got a septal and a lobular histopathologic type. In the opinion of the etiologic classification occurs idiopathic (primary) or secondary entity. The secondary illnesses develop as the result of physical, chemical, biochemical, immunological, degenerative and proliferative reactions. In a part of panniculitis the etiologic factor is unknown. The authors demonstrate a rare type of the panniculitis, the Weber-Christian syndrome. This disease is an acute relapsing panniculitis with unknown etiology, fever, general symptoms and typical subcutaneous nodes. In their case the histological examination of the subcutaneous node resulted in non-suppurative, lobular panniculitis. The oral metilprednisolon and the occurring twice parenteral cyclophosphamide treatment had been proved to be unsuccessful, for this reason they started cyclosporin A therapy. Because of the cyclosporin A treatment the subcutaneous nodes regressed, the general symptoms ceased and the pathological laboratory findings became normal. The patient is asymptomatic under permanent therapy now. The authors discuss the differential diagnostic of this illness and the ways of treatments.

Aged↗

PubMed1989

Lupus erythematosus panniculitis.

LE panniculitis is an uncommon but distinctive subset of LE. It may develop in patients with discoid LE or SLE or may occur as an isolated phenomenon. The typical clinical presentation is that of multiple indurated nodules or plaques (or both), often associated with lipoatrophy, there being a predilection for the proximal extremities and trunk. Because the clinical and histologic findings of LE panniculitis overlap with those of other connective tissue diseases, evaluation of patients suspected of having LE panniculitis should include a complete history and physical examination as well as serologic studies, determination of peripheral blood counts, and tests of renal function. A deep excisional biopsy rather than punch biopsy should be performed for diagnosis. The characteristic histologic pattern includes hyaline necrosis of fat; lymphoid nodules, often with germinal centers; and lymphocytic lobular panniculitis. Direct immunofluorescence testing of skin may help confirm the diagnosis in patients who have less than classic histologic features. LE panniculitis tends to have a chronic course marked by recurrent nodules or plaques (or both). Antimalarial agents, with or without courses of systemic steroids, are beneficial in most patients.

Diagnosis, Differential↗

PubMed2006

Localized panniculitis secondary to subcutaneous glatiramer acetate injections for the treatment of multiple sclerosis: a clinicopathologic and immunohistochemical study.

BACKGROUND: Glatiramer acetate has been shown to be effective in reducing the relapse and improving the disability of patients with multiple sclerosis. The most common adverse effects at the injection sites include pain, inflammation, and induration that spontaneously disappear within hours or a few days. OBJECTIVE: We sought to characterize the histopathologic findings of localized panniculitis induced by glatiramer acetate at the injection sites. METHODS: Seven patients receiving daily glatiramer acetate injections for treatment of multiple sclerosis developed localized panniculitis at the injection sites. The lesions were histopathologically and immunohistochemically studied. RESULTS: The lesions consisted of a mostly lobular panniculitis, with lipophagic granuloma, namely histiocytes engulfing the lipids from necrotic adipocytes. In many areas, scattered neutrophils and eosinophils were seen both in the septa and in the fat lobules. Connective tissue septa showed widening and fibrosis in conjunction with many lymphoid follicles, presenting with germinal center formation. Immunohistochemically, the inflammatory infiltrate of the fat lobule consisted of CD68+ histiocytes and suppressor/cytotoxic T lymphocytes. In contrast, the lymphoid follicles in the septa and at the interface between septum and fat lobule were mainly composed of B lymphocytes. LIMITATIONS: Only one biopsy was performed in each patient and, therefore, it was not possible to study the histopathologic evolution of the panniculitic process. CONCLUSIONS: Localized panniculitis at the sites of subcutaneous injections of glatiramer acetate for treatment of multiple sclerosis seems to be a rare, but characteristic side effect of this therapy. The histopathologic pattern of these lesions consists of a mostly lobular panniculitis, with histiocytes and T lymphocytes in the fat lobule and thickened septa with scattered lymphoid follicles, which are mostly composed of B lymphocytes.

Adipocytes↗

PubMed1988

Alpha 1-antitrypsin deficiency associated with panniculitis.

Alpha 1-Antitrypsin is the principal serum protease inhibitor. In addition to the well-recognized association with early-onset emphysema and cirrhosis, alpha 1-antitrypsin deficiency may be associated with panniculitis. In this article we describe three patients in whom the recognition of certain clinical and histologic features of panniculitis eventually led to the diagnosis of alpha 1-antitrypsin deficiency. Two of our patients were young adults and one was a child. All three had draining, panniculitis, or cellulitis-like lesions at sites of prior trauma. The histopathologic findings were characterized by liquefactive dermal necrosis and collagenolysis of the fibrous septa of the subcutis. The combination of these clinical and microscopic findings should suggest the diagnosis of alpha 1-antitrypsin deficiency panniculitis. The suspicion can be verified by obtaining quantitative serum levels and enzyme phenotyping. The identification of the alpha 1-antitrypsin deficiency state as the cause of a distinct type of panniculitis adds additional evidence for the elimination of the term Weber-Christian disease.

Adult↗

PubMed2003

Nasal-type natural killer cell lymphoma preceded by benign panniculitis arising in an asymptomatic HTLV-1 carrier.

We report a case of an Epstein-Barr virus (EBV)-associated nasal-type natural killer cell lymphoma (NKCL) preceded by benign panniculitis, which arose in a 48-year-old woman with an asymptomatic human T-cell leukemia/lymphoma virus type-1 (HTLV-1) infection. A biopsy of the initial panniculitis lesion demonstrated lobular panniculitis with a germinal center composed of benign mononuclear cells with a phenotype of CD4+CD45RO+CD5sCD3+ cCD3 epsilon + T-cell intracellular antigen-1 (TIA-1)- and granzyme B-. One year after oral prednisolone therapy, the patient developed subcutaneous nodules composed of atypical lymphoid cells with a phenotype of CD4-CD45RO+CD56+sCD3-cCD3 epsilon + (TIA-1)+ and granzyme B+. In the initial panniculitis lesion, neither EBV-encoded RNA (EBER-1) nor clonal proliferation of EBV-infected cells was identified. In later lesions, however, a large number of atypical cells were positive for EBER-1, and a clonal expansion of EBV-infected cells was detected. No clonal rearrangement of T-cell receptor-alpha, -beta, or -gamma genes was found in either specimen. This patient was an asymptomatic carrier of human T-cell leukemia/lymphoma virus type-1 (HTLV-1) without clonal integration of proviral HTLV-1 in neither the peripheral blood nor the skin lesions. These observations suggest that EBV-associated NKCL occurred subsequently in the clinical course of benign panniculitis under the influence of immunosuppression caused by prednisolone treatment and HTLV-1 infection.

Antibodies, Viral↗

PubMedDate not supplied

Panniculitis in juvenile dermatomyositis.

Panniculitis is a rarely reported clinical finding in dermatomyositis. We present a 14-year-old African American boy with a 4-year history of dermatomyositis referred for evaluation of tender, indurated plaques and nodules on the trunk and proximal extremities. A biopsy specimen revealed epidermal and dermal changes consistent with dermatomyositis. Although calcification was absent, a striking lobular panniculitis was observed. A total of seven cases of clinical panniculitis in association with dermatomyositis have been published. As in our patient, it typically presents as indurated, tender plaques and nodules on the arms, thighs, and buttocks. Although in the vast majority of cases panniculitis found in association with dermatomyositis is subclinical and represents an incidental histopathologic finding, the case we present further emphasizes the need to recognize panniculitis as a rare, but clinically relevant pathologic feature seen in certain lesions of juvenile dermatomyositis.

Adolescent↗

PubMed1992

[Recurrent eyelid tumor in necrotizing panniculitis].

To our knowledge, necrotizing lid tumors occurring in the context of a necrotizing lobular panniculitis have not yet been described in the literature. Our patient, a 75-year-old male, presented with indolent, nonmoveable subcutaneous lumps that were centrally ulcerating. They appeared first on his upper lid, then on his lower lid and thereafter on the neck, back, upper extremity and abdomen, especially in the area of his cholecystectomy scar. A thorough work-up, which included repeated histopathological examinations performed by several laboratories, led to the diagnosis of an idiopathic necrotizing lobular panniculitis. A serum level determination performed later revealed no alpha 1-antitrypsin deficiency. As the eye-lid's subcutaneous tissue lacks fat, the association between the necrotizing lid tumors and the necrotizing panniculitis appears to be a paradox. In spite of the normal alpha 1-antitrypsin serum levels--determined when the patient's lesions had long ago healed--we think in retrospect that the differential diagnosis should have included the possibility of a decreased alpha 1-antitrypsin serum level (e.g. heterozygous MZ-phenotype). An alpha 1-antitrypsin level deficiency--with resulting decreased inhibition of collagenase and elastase--could account for the necrotizing process that also occurred in the eyelid's subcutaneous tissue. In the Van Gieson stain of this patient's eyelid biopsy, fragmentation of all visible collagen and elastic fibers was noted. In our opinion, the differential diagnosis does include lid involvement with secondary panniculitis caused by partially decreased alpha 1-antitrypsin serum levels and--by exclusion--idiopathic necrotizing lobular panniculitis. Therapeutic possibilities are briefly discussed.

Aged↗

PubMed2001

Postirradiation pseudosclerodermatous panniculitis.

Pseudosclerodermatous panniculitis is an unusual variant of panniculitis that results as a complication of megavoltage radiotherapy. Four women developed this unusual entity on the anterior chest and abdominal skin after receiving megavoltage therapy for either breast carcinoma or painful bone metastases from breast carcinoma. Histopathologically, the epidermis and dermis of the involved area showed little or no evidence of radiodermatitis. The main findings were confined to the subcutaneous tissue and consisted of thickened, sclerotic septa composed of both thick and thin collagen bundles, and a lobular panniculitis characterized by lipophagic granulomas and scattered lymphocytes and plasma cells. Additionally, one of the cases showed markedly dilated vascular spaces with the appearance of lymphatics in the upper part of the dermis. Pseudosclerodermatous panniculitis after irradiation is an unusual cutaneous complication of megavoltage radiotherapy that should be distinguished from subcutaneous metastatic disease, cellulitis, or connective tissue diseases involving the subcutaneous fat. The differential diagnosis can be established on the basis of the characteristic histopathologic features of postirradiation pseudosclerodermatous panniculitis.

Adult↗

PubMed2006

Panniculitis: an unusual cutaneous manifestation of rheumatoid arthritis.

Specific and nonspecific panniculitis may occur during the course of connective tissue diseases. However, association of panniculitis and rheumatoid arthritis has only rarely been described, and with so few reports, it is difficult to know the significance of this unusual association. We describe 2 patients with rheumatoid arthritis and features of Sjögren syndrome in whom panniculitis developed during the course of the disease. Histologic confirmation of septal panniculitis was established in one case and the panniculitis in this case improved as the arthritis resolved.

Arthritis, Rheumatoid↗

PubMed1989

Infection-induced panniculitis.

We evaluated the histopathologic findings in 15 cases of panniculitis due to infection. Organisms were identified by special staining in 14 cases, and in 6 of these the etiologic agent was confirmed by culture studies. In one additional case, lesional culture was positive despite negative special staining. Eight cases were caused by gram positive or gram negative bacteria, 2 by atypical mycobacteria, 2 by Nocardia, and 3 by fungi (Candida and Fusarium species). Most patients had conditions or were receiving therapies predisposing to immunosuppression, but at least 2 individuals had no other major medical problems. There were some variability in microscopic findings and overlap with established forms of panniculitis; 3 cases (2 due to bacteria and 1 to Fusarium) resembled acute, "neutrophilic" erythema nodosum, and evidence of vasculitis was seen in septicemias due to Pseudomonas, Nocardia, and Fusarium. Nevertheless, several distinctive features associated with infectious panniculitis of diverse etiologies included: epidermal alterations such as acanthosis and parakeratosis; dermal edema with a diffuse or perivascular neutrophilic infiltrate; and mixed septal-lobular panniculitis with neutrophilic infiltration, vascular proliferation and hemorrhage, and necrosis. Infection should be suspected in virtually any case of panniculitis, particularly when occurring in an immunosuppressed patients, but the microscopic features enumerated above should arouse particular suspicion of an infectious process.

Adult↗

PubMed2006

Pancreatic panniculitis associated with allograft pancreatitis and rejection in a simultaneous pancreas-kidney transplant recipient.

Pancreatic panniculitis is an uncommon condition that can occur in association with pancreatic disease. We present a case of pancreatic panniculitis in a female pancreas-kidney transplant recipient 5 months post-transplant. The patient was on standard immunosuppressive medications and had acute rejection of her renal allograft. The diagnosis of allograft pancreatitis and rejection presenting with pancreatic panniculitis was supported clinically, histopathologically and by laboratory and imaging data. This is the fourth case of pancreatic panniculitis occurring in a transplant recipient and the first in a simultaneous pancreas-kidney transplant recipient. It is also the first case associated with allograft rejection. Clinicians should be aware that pancreatic panniculitis may be a manifestation of underlying allograft pancreatic disease.

Biopsy↗