Cytophagic Panniculitis Research Articles

Subcutaneous Panniculitis-Like T-Cell Lymphoma (SPTL) in a Child with Spontaneous Resolution

Subcutaneous panniculitis-like T-cell lymphomas (SPTLs) α/β are rare in childhood. The present report refers to a case of a 7-year-old male child presenting an extensive skin lesion that began when he was 5 years of age. Two biopsies were evaluated using the CD3, CD4, CD8, CD56, βF1, and TIA markers. A dense infiltrate of CD3+, CD4−, CD8+, CD56−, βF1+, and TIA+ pleomorphic lymphocytes was found in the subcutis. The previous biopsy showed cytophagic histiocytic panniculitis with a small focus on CD8+ and βF1+ malignant cells. The lesion regressed spontaneously. This case shows that prognosis may be excellent in SPTL (α/β). On the other hand, it also serves as an alert that a biopsy performed in an area of cytophagic panniculitis may lead to misdiagnosis.

Monitoring of phagocytic activity in histiocytic cytophagic panniculitis

Histiocytic cytophagic panniculitis presents with subcutaneous panniculitis. Histologically, it is characterized by phagocytosis of blood cells in the subcutaneous tissue and bone marrow. One patient with histiocytic cytophagic panniculitis is described in whom hemophagocytosis macrophages and histiocytes was observed histologically and was confirmed in vitro measuring phagocytosis by peripheral blood monocytes by means of chemiluminescence. In vitro measurements of phagocytosis corresponded well with the clinical course. Chemiluminescence for measuring phagocytosis in vitro may be suitable for analyzing disease activity and for testing therapeutic compounds in vitro. (J Am Acad Dermatol 2001;44:120-3.)

Weber-Christian panniculitis: A review of 30 cases with this diagnosis

Background: More than 60 years ago, Christian described a panniculitis that was later termed Weber-Christian disease . Objective: The purpose of this study was to investigate whether this is a specific disease or a nonspecific disease that embraces several specific conditions. Methods: We studied 30 cases diagnosed as Weber-Christian panniculitis and found it possible to make a more specific diagnosis. Results: In 12 patients, findings were compatible with erythema nodosum. Six patients had phlebitis or postphlebitic syndrome. Factitial panniculitis was diagnosed in five patients, and trauma had a role in the conditions of another three patients. Cytophagic panniculitis, lymphoma, and leukemia were recognized in one patient each. The lesion was lobular in almost all cases, and the presence of lipophagia was noted in 19 biopsy specimens. Granulomatous, neutrophilic, and lymphocytic pathologic changes were present in nine, eight, and eight tissue specimens, respectively. Conclusion: The recognition of distinct disease patterns of fat lesions as fat necrosis with pancreatic disease, α 1 -antitrypsin panniculitis, lupus and connective tissue disease panniculitis, involution lipoatrophy, lipomembranous panniculitis, factitial panniculitis syndromes, calcification panniculitis, lipophagic lipoatrophy, and cytophagic panniculitis has lessened the need for a less specific panniculitis category. All these diseases have been reported in the literature as “Weber-Christian disease.” Because separate and distinct forms of fat lesions have been described, we believe that the eponym should be abandoned and that more specific diagnoses should be made on the basis of pathogenesis or cause. (J Am Acad Dermatol 1998;39:56-62.)

Angiocentric lymphoma

Angiocentric T-cell lymphoma is a rare type of extranodal lymphoma that has, in the past, been dispersed amongst a variety of eponymous ‘pseudolymphomatous’ disorders. In this article the relationship of angiocentric T-cell lymphoma to the older descriptions of so-called lymphomatoid granulomatosis, Stewart's lethal midline granuloma and histiocytic cytophagic panniculitis is discussed. The characteristic histological, immunophenotypic, genotypic and clinical features are described.

Subcutaneous T-Cell Lymphoma

<h3>Background:</h3> Peripheral T-cell lymphomas involving subcutaneous tissue are rare. With modern immunohistochemical stains, subcutaneous T-cell lymphomas may be demonstrated in patients previously thought to have fatal Weber-Christian disease, malignant histiocytosis, and histiocytic cytophagic panniculitis. <h3>Observations:</h3> We describe two young women with subcutaneous lymphomas. They presented with erythematous nodular lesions, which suggested benign panniculitis. Skin biopsy specimens in both patients demonstrated large atypical lymphocytes in the subcutaneous tissue, karyorrhexis, and cytophagia. Immunohistochemical studies confirmed a T-cell phenotype. Molecular genetics studies performed in one patient showed a major T-cell clonal population. <h3>Conclusions:</h3> Subcutaneous T-cell lymphoma is a rare, distinct subtype of postthymic peripheral T-cell lymphoma. Cytophagic "histiocytic" panniculitis is frequently a prominent histologic feature. Early systemic chemotherapy may offer therapeutic advantages in patients with this disease. (<i>Arch Dermatol.</i>1993;129:1171-1176)

Panniculitis and fever in children

We describe three children with panniculitis and associated systemic manifestations including fever. Histopathologic features, such as the presence of lobular or septal inflammation, presence of vasculitis, character of the cellular infiltrate, and presence of erythrophagocytosis, were useful in classifying this group of panniculitides. In one patient with subcutaneous polyarteritis nodosa, corticosteroid therapy was effective; in two other patients with histiocytic cytophagic panniculitis, there were poor responses to steroids, intravenously administered immune globulin, dapsone, or antimalarial drugs; cyclosporine was very effective and appeared to be the drug of choice.

Histiocytic cytophagic panniculitis: Molecular evidence for a clonal T-cell disorder

Histiocytic cytophagic panniculitis is a systemic disease of unknown etiopathogenesis that invariably involves the subcutaneous fat and is histologically characterized by phagocytosis of blood elements by histiocytes that appear to be benign. Immunophenotypic and genotypic studies of biopsy specimens of the lesions of a 58-year-old woman showed that the lymphocytic infiltrates accompanying the histiocytes in the subcutis were composed of clonal T-cells with rearrangement of the surface receptor gene. Our findings suggest that the primary abnormality in histiocytic cytophagic panniculitis may be a clonal T-cell proliferation.

Treatment of cytophagic panniculitis with cyclosporin A

Treatment of cytophagic panniculitis with cyclosporin A

Histiocytic cytophagic panniculitis: Report of a case in a 12-year-old girl

We report a 12-year-old girl with nodular skin lesions, fever, pancytopenia, coagulation defects and a fatal outcome within 4 months. Histopathology revealed proliferation of histiocytes with haemophagocytosis in skin, perivisceral fat and haemopoietic organs. The symptoms were most consistent with cytophagic histiocytic panniculitis.

Histiocytic cytophagic panniculitis

A 33 year old man developed fever, malaise, jaundice, pancytopenia, coagulation abnormalities, hepatomegaly, pleural effusions and a subcutaneous lump. Biopsies revealed a lobular panniculitis with the presence of cytophagic histiocytes; erythrophagocytosis was also demonstrated in the liver and bone marrow. Despite the use of chemotherapy (CHOP) his clinical condition deteriorated and he died 5 months after presentation. This illness is consistent with the recently described syndrome of histiocytic cytophagic panniculitis.

Histiocytic cytophagic panniculitis

Nineteen cases of histiocytic cytophagic panniculitis from our institution and from the literature were reviewed for their clinical and histopathologic features. All patients had cutaneous involvement consisting of cutaneous nodules and plaques that occasionally were ecchymotic and ulcerated and were characterized by infiltration of the subcutaneous tissue by large, benign histiocytes with cytophagic features. Thirteen of the patients had died (nine with hemorrhagic complications), four patients had a benign course with remission of the disease, and two patients responded to aggressive polychemotherapy, Immunohistochemical studies of paraffin-embedded tissue, performed in five cases, revealed a large number of infiltrating T cells in the subcutaneous inflammatory lesions.

Cytophagic panniculitis and B cell lymphoma

A 43-year-old woman had multiple, painful nodules of the legs and edema of both lower extremities for 3 years and leukopenia for 8 years. This histologic findings of lobular histiocytic panniculitis with "beanbag" cells were typical of cytophagic panniculitis. This case is unique in that B cell lymphoma developed in the spleen, as verified by immunoperoxidase staining, and demonstrates that the disease not only may be a progressive disease per se but also may be associated with nonhistiocytic malignancy.

Cytophagic histiocytic panniculitis with fever, cytopenia, liver failure, and terminal hemorrhagic diathesis

We have seen five adult patients with a clinical picture of recurrent histiocytic, cytophagic panniculitis, cytopenia, abnormal liver function tests, and a terminal, febrile bleeding diathesis. Originally thought to have Weber-Christian disease, these patients, we believe, represent a unique syndrome: lobular, histiocytic, cytophagic panniculitis. Erythrophagocytosis and cytophagocytosis are readily observed, but the cells do not show malignant features. Histiocytosis can be found at times in the bone marrow, lymph nodes, liver, spleen, and serosal tissues, as well as in the skin and subcutaneous tissues. The terminal hemorrhage in these patients is characterized by features of pancytopenia, liver failure, and intravascular coagulation. This disease may be separated from malignant histiocytosis by the chronic course, the primary involvement of the adipose tissue, and the benign histiocytes in the infiltrate. It has some similarities to other regional histiocytoses such as sinus histiocytosis, intestinal histiocytosis, and splenic histiocytosis.

Hemorrhagic Diathesis Associated With Benign Histiocytic, Cytophagic Panniculitis and Systemic Histiocytosis

Five patients had a histiocytic, cytophagic panniculitis and a febrile, progressive course, which included hepatosplenomegaly, pancytopenia, liver function abnormalities, and a hemorrhagic death. The terminal hemorrhage was characterized by features of intravascular coagulation and liver failure. The histiocytosis could be found at times also in bone marrow, lymph nodes, liver and spleen, and serosal tissues, as well as in skin and subcutaneous tissue. Adequate biopsy diagnosis will prevent such cases from being labeled as Weber-Christian disease with bleeding, and offer an opportunity for appropriate treatment of this new syndrome of histiocytic, cytophagic panniculitis.

Hemorrhagic diathesis associated with benign histiocytic, cytophagic panniculitis and systemic histiocytosis

Hemorrhagic diathesis associated with benign histiocytic, cytophagic panniculitis and systemic histiocytosis