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Cytophagic histiocytic panniculitis with atypical lymphocytes in peripheral blood.

A 26-year-old female patient developed high fever, hepatosplenomegaly and subcutaneous nodules. Atypical lymphocytes were present in the peripheral blood. Skin biopsy revealed lobular panniculitis. Bone marrow examination showed prominent phagocytosis by benign histiocytes. The diagnosis of cytophagic histiocytic panniculitis was made, and the disease has been well controlled with oral prednisolone. This is the first report of cytophagic histiocytic panniculitis with atypical lymphocytes in the peripheral blood, which are frequently seen in virus-associated hemophagocytic syndrome. This observation suggests that underlying viral infection may be one factor in the development of cytophagic histiocytic panniculitis.

Adult↗

[Subcutaneous panniculitis at a third level hospital retrospective study of 113 cases].

INTRODUCTION: The panniculitides or hypodermitis are a wide and heterogeneous group of diseases characterized by the presence of subcutaneous inflammatory nodules located generally in low limbs. OBJECTIVES: Show the clinicopathologic characteristics of the cases diagnosed as panniculitis for a 5 years period; their epidemiology, etiopathogenic factors, evolution and treatment. MATERIAL AND METHODS: A retrospective study of 113 patients diagnosed as panniculitis by histopathologic report of cutaneous biopsy since 1997 to 2001 were performed. RESULTS: The sex proportion was of 4.65 women to every man. The mean age of these patients was 49 +/- 20 years old. 100% had legs affectation, 7.1% in arms, 5.9% in trunk and 0.9% in face. 9.7% presented fever and arthromyialgias, especially in young people (p < 0.05). The etiopathogenic factors implied were tuberculosis, streptococcic pharyngoamygdalitis, sarcoidosis and different drugs. The most frequent histological pattern was septal panniculitis without vasculitis. 24.8% did not realize any treatment; 32.5% took nonsteroideal anti-inflammatory drugs; 31.6% potassium iodide; 16% systemic corticosteroids and 7.1% tuberculostatic drugs. Evolution of the patients was good and independently from the treatment performed. CONCLUSIONS: Panniculitides are a group of diseases with similar clinic, but very heterogeneous in their etiology and histopathologic findings. They are more frequent in women and with more expressive clinic in young people. Up to in the 59.3% of the studied cases a causal etiologic agent was identified. These factors are compatible with the published ones in other previous panniculitis series. When a paniculitis is suspected, the most efficient tool is the clinical diagnosis. Histological confirmation by cutaneous biopsy it must be done when it is possible.

Adipose Tissue↗

A 30 year history of panniculitis.

The differential diagnosis of panniculitis may be challenging due to the uniform clinical presentation of different panniculitis subsets. We describe a 45-year-old woman with a 30 year history of panniculitis, who had repeatedly failed to fulfill diagnostic criteria for various panniculitis subsets. Finally, erythema induratum was diagnosed and she was successfully treated with antituberculous chemotherapy. The wide spectrum of histological alterations of chronic erythema induratum as well as the sensitivity of polymerase chain reaction for Mycobacterium tuberculosis in erythema induratum lesions is discussed.

Antitubercular Agents↗

[Panniculitis and macrophage activation syndrome in a child with lupus erythematosus].

INTRODUCTION: Panniculitis is rarely presented in the course of systemic lupus erythematosus. When it occurs, it is mainly related to lupus profundus. However, when panniculitis is associated with a reactive hemophagocytic syndrome, panniculitis could be linked to this hemophagocytosis reaction. CASE REPORT: We report a case of an 11-year-old girl treated for several years for systemic lupus erythematosus, who simultaneously presented panniculitis and an hemophagocytic syndrome. The reality of both diagnoses was based on the analysis of biological and histological data. Therapy with immunosuppressive drugs led to relief of the symptoms. DISCUSSION: We emphasize our discussion on the pathophysiology of lupus profundus and hemophagocytosis with regards to the role of cytokines and circulating immune complexes in both diseases. They may enhance the hypodermal necrosis observed in lupus profundus and induce macrophage cell dysregulation through cytotoxic cells known in hemophagocytosis. The immunomodulative action of immunosuppressive therapy with inhibition of cells involved in immune response, may explain its efficacy.

Antigen-Antibody Complex↗

From circumscribed scleroderma (morphea) to subcutaneous panniculitis-like T-cell lymphoma: case report.

Subcutaneous panniculitis-like T-cell lymphoma is a rare form of non-Hodgkin lymphoma. It presents clinically as panniculitis, with erythematous, firm subcutaneous infiltrates and recurrent papulonodules. A 45-year-old male Croat presented with a 6-month history of tender erythematous subcutaneous skin lesions without systemic symptoms. Analysis of a lesion biopsy specimen showed circumscribed scleroderma (morphea). During hospitalization, skin biopsies from two subcutaneous lesions on the stomach were repeated, and pathohistologic analysis corresponded primarily to panniculitis in the context of morphea. Pathohistologic analysis and immunohistochemistry of a specimen obtained by tumor excision from the back indicated subcutaneous panniculitis-like T-cell lymphoma. Histologic analysis revealed a dense infiltrate of atypical T-lymphoid cells expressing CD8(+) phenotype located in the subcutaneous tissue with histiocyte-phagocytizing apoptotic cells. Specific hematologic analysis and medical treatment were continued. This case report illustrates the importance of continuous follow-up with repeat biopsy and use of immunohistologic techniques for early diagnosis.

Humans↗

[Mesenteric panniculitis of the sigmoid. A case report and review of the literature].

Mesenteric panniculitis is an extremely rare disease in which the normal fatty architecture of the mesentery is replaced by fibrosis, necrosis and calcification. One hundred twenty-four cases of mesenteric panniculitis have been described in the literature; only five of these involved the sigmoid mesentery. The following case report is of mesenteric panniculitis confined to the sigmoid colon. Symptomatology, pathology, treatment and outcome of this disorder are discussed. It seems that this disease is a well established clinical entity of unknown etiology with a benign course and favorable outcome. Radical surgical treatment is not advised. If mesenteric panniculitis is encountered at operation, biopsy to establish the diagnosis is indicated. If the mechanical effects are significant, they should be corrected.

Biopsy↗

Panniculitis associated with severe alpha 1-antitrypsin deficiency. Treatment and review of the literature.

Panniculitis associated with homozygous severe alpha 1-antitrypsin deficiency was documented in three women hospitalized for painful cutaneous and subcutaneous ulcerations (severe panniculitis with spontaneous ulceration and drainage of clear or serosanguineous fluid). None had a history of trauma or infection. One patient responded rapidly and completely to treatment with dapsone. One patient, who had more extensive disease, failed to respond to prednisone plus dapsone; infusions of alpha 1-proteinase inhibitor concentrate led to resolution of her panniculitis. One patient who had severe and extensive panniculitis and pleural effusions failed to respond to corticosteroids but did well when both dapsone and infusions of alpha 1-proteinase inhibitor concentrate were added to her treatment program.

Adipose Tissue↗

Septal granulomatous panniculitis in Sweet's syndrome. Report of two cases.

Two patients had Sweet's syndrome (acute febrile neutrophilic dermatosis) with subcutaneous lesions resembling erythema nodosum. In both, the erythema nodosum--like lesions histologically showed a septal granulomatous panniculitis with numerous Miescher's granulomas. This type of subcutaneous involvement in Sweet's syndrome should be differentiated from panniculitis caused by deep subcutaneous neutrophilic infiltration. Involvement of the panniculus in Sweet's syndrome may then occur in two different patterns: a septal granulomatous panniculitis, as in our two cases, or a neutrophilic lobular panniculitis, as has been previously reported.

Adult↗

[Lupus erythematosus panniculitis (author's transl)].

Lupus erythematosus panniculitis is a rare clinical variant of lupus erythematosus. In this report we described a 38 year-old female patient who had suffered from chronic discoid lupus erythematosus for several years before developing widespread inflammatory, sclerotic and ulcerative lesions, which were first diagnosed as Weber-Christian panniculitis. It was only when the patient developed other signs and symptoms of systemic lupus erythematosus that the subcutaneous lesions were recognized as lupus panniculitis. A combined regimen of tetracyclines and chloroquine resulted in a remission, both with regard to regression of the lesions and suppression of the serological parameters of disease activity. The findings in this particular patient and similar reports in the literature form the basis for a discussion of the entity of lupus panniculitis.

Chloroquine↗

[Pseudo-tumorous panniculitis of the mesentery. An unusual initial stage of acute lupus erythematosus in a 10-year-old girl].

Lupus panniculitis usually affects the hypodermis and the dermis profundus (Irgang's lupus profundus). In our case, the systemic lupus erythematosus was preceded by an intraabdominal panniculitis, occurring as a voluminous tumor. Histological examination showed the usual picture of panniculitis, but there were also severe fibrinoid necrotic lesions, which should have alerted us. Lupus must be included among the etiologies of intraabdominal panniculitis.

Acute Disease↗

Connective tissue panniculitis.

Two patients with lobular lymphohistiocytic panniculitis had chronic lesions that produced subcutaneous atrophy and that were responsive to antimalarial drugs. Massive lymphocytic infiltrate was associated with caseation necrosis of the fat lobules. In both patients, an unusual antibody to extractable nuclear antigen was seen, and antinuclear antibody was sometimes present. The unusual manifestation, course of the disease, serologic findings, and response to therapy suggest that the panniculitis is related to (but does not evolve into) lupus erythematosus panniculitis or subcutaneous morphea or both. A satisfactory term for this entity would be "connective tissue panniculitis."

Adipose Tissue↗

[Mesenteric panniculitis: a case of acute and fatal presentation].

Mesenteric panniculitis is a rare process in which there is an inflammation of the mesenteric adipose tissue due to unknown causes and mechanisms. It evolves with abdominal pain and/or mass, among other symptoms, generally with a chronic and bening course. The concomitant tests are generally non-specific, the diagnosis being anatompathological. We present the case of an old woman who died hours after starting an episode of abdominal pain. The autopsy showed the presence of mesenteric panniculitis. The sudden onset of the clinical signs, without other justifying cause than the panniculitis itself, suggests that this must be considered in the differential diagnosis of acute abdominal pain. In addition, the absence of other morphological findings which could suggest a fatal cause make us to consider the relationship between this and the panniculitis.

Abdomen, Acute↗

[Panniculitis-fasciitis syndrome disclosing systemic erythematous lupus].

INTRODUCTION: Panniculitis is a common manifestation of lupus, but is rarely the first manifestation in systemic lupus erythematosus. We report a case associated with fasciitis. CASE REPORT: A 67-year-old man was hospitalized for fever, pleuropericarditis, subcutaneous diffuse infiltration of the two thighs and the abdomen due to panniculitis and lupic fasciitis. Antiphospholipid antibody test was positive. There was non antiphospholipid syndrome. COMMENTS: Panniculitis can occur simultaneously or a few months or years before systemic lupus erythematosus. An association with fasciitis should be entertained whenever the cutaneous involvement is extensive and firm. In such a situation, deep biopsies are indicated. Observations of further cases will help identify the role of general steroid therapy in fasciitis-panniculitis and systemic lupus erythematosus.

Aged↗

Resolution of panniculitis after placement of pancreatic duct stent in chronic pancreatitis.

The association of panniculitis and pancreatitis is well described. However, panniculitis remains a relatively uncommon manifestation of pancreatic inflammation. We report a case in which treatment of pancreatitis by the placement of a pancreatic stent led to simultaneous resolution of both the pancreatitis and the associated panniculitis. There are no other reports in the literature demonstrating resolution of panniculitis subsequent to stent placement or definitive surgery.

Adult↗

Acute panniculitis: a clinical and histopathologic study of 34 cases.

Thirty-four patients had histopathologic findings of acute panniculitis. 15 had clinical findings of erythema nodosum; 6 had infectious lesions; and 5 had Weber-Christian-like conditions with recurrent febrile suppurative or nonsuppurative nodular eruptions; 3 of the 5 had amylase and lipase enzyme abnormalities with or without pancreatic disease. Five additional patients had acute panniculitis that would be termed "deythema induratum" or "nodular vasculitis" by most clinicians, and the subsequent pathologic findings and courses were consistent with this view. Two patients had limited, traumatic panniculitis, forming a distinctive group, but trauma was a factor in lesion formation in all groups, and one patient had vasculitis. Whereas acute panniculitis, with or without fat necrosis, may be associated with many inflammatory syndromes of the skin and subcutaneous tissue and may rarely occur in all, the most common relationship is with erythema nodosum in its most acute forms.

Acute Disease↗

[Mesenteric panniculitis simulating Crohn disease].

Mesenteric panniculitis is a rare disease involving the adipose tissue of the mesentery. We report a case of a 27-year-old woman with mesenteric panniculitis, who presented clinical and radiological features mimicking Crohn's disease. In the outcome, she presented a small bowel perforation, unusual in this pathology, and an annexial involvement. This case reminds us of the role of sepsis and repeated abdominal surgery in relation to the pathogenesis of mesenteric panniculitis. We report the first case of mesenteric panniculitis mimicking Crohn's disease.

Adult↗

Lupus panniculitis: clinical perspectives from a case series.

OBJECTIVE: To review clinical and laboratory features of lupus panniculitis from a large group of patients. METHODS: Retrospective chart review of patients diagnosed with lupus panniculitis at a tertiary medical center from 1976 to 1993. RESULTS: Lupus panniculitis occurred most frequently in adult women. Skin lesions involved proximal extremities, trunk, face, and scalp. Only 4 of 40 patients fulfilled criteria for systemic lupus erythematosus (SLE), and, other than positive antinuclear antibodies, a paucity of other autoantibodies was seen. Average disease duration was 6 years (range 0-38). Treatment with antimalarial agents was undertaken in most cases. Disease related morbidity (disfigurement and disability) was relatively common, but death was rare. CONCLUSION: Lupus panniculitis is a chronic inflammatory disease of subcutaneous adipose tissue that can develop during the course of SLE, although most patients do not develop systemic lupus.

Adolescent↗

Panniculitis: a presenting manifestation of disseminated histoplasmosis in a patient with rheumatoid arthritis.

We report a case of histoplasma panniculitis in a patient with rheumatoid arthritis treated with multiple immunosuppressive agents including infliximab. The patient presented with erythematous plaques involving the right upper anterior abdominal wall, the right flank and later extension to involve the left flank and the left axilla. The histopathologic examination of these lesions was consistent with lobular panniculitis and the cultures of these lesions and blood grew Histoplasma capsulatum. The clinical and histopathologic picture of histoplasma panniculitis and rheumatoid arthritis-associated panniculitis can be very similar, but the treatment is clearly different. Our case emphasizes the importance of considering rare manifestations of opportunistic infections in an era of widespread use of immunosuppressive medications in the field of rheumatology.

Journal Article↗