Cytophagic panniculitis and lupus panniculitis versus Weber-Christian disease.
Explore the source record for details and available documents.
SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Multiple subcutaneous nodules and recurrent flu-like episodes developed in a 60-year-old woman after she had received local intramuscular injections of procaine povidone for a seven-year period. A skin biopsy specimen showed distinctive gray-blue material in the histiocytes in sections stained with hematoxylin-eosin. Povidone is an inert substance that may accumulate in multiple organs, including the skin. Its presence can be verified by its affinity for special stains or by spectrophotometric analysis. In our patient, ultrastructural study showed amorphous inrahistiocytic deposits in the tissues. -revious reports have indicated that this substance may eventually accumulate in noninjected skin sites and may lead to pulmonary and arthritic symptoms in some patients, but no other patients, to our knowledge, have had complications associated with fever.
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, alpha1-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.
Eighty-two cases of nodular panniculitis of the legs were examined clinically, histologically and immunohistologically. Clinically the cases could be divided into four groups: typical erythema nodosum (ENty) (35 cases), erythema nodosum migrans (ENmi) (11 cases), erythema induratum (EI) (11 cases) and the remaining 25 cases not consistent with the others as "non-definite panniculitis" (NDP). The main histological categories were septal panniculitis and lobular panniculitis, the former including erythema nodosum, both typical and migrans, the latter EI and NDP. Lobular panniculitis was divided into three subgroups in which the most prominent histological features were epithelioid cell granuloma, vasculitis and palissading granuloma, respectively. Immunoglobulins in the vessel walls were found in 5 of the 46 cases of erythema nodosum, in 19 of the 36 EI and NDP cases and, in the histological groups in 4 of the 43 cases of septal panniculitis and in 19 of the 35 cases of lobular panniculitis, respectively. Fibrin was found in the walls of the papillary capillaries and deep dermal vessels in the majority of cases of lobular panniculitis. In EI and NDP the follow-up time was 40 months, on average. Twenty-two patients were treated with antituberculous drugs, 15 became symptomless, as did 5 of the 12 patients who were not treated at all.
The classification of inflammatory disorders of the subcutaneous tissue has mystified dermatologists for decades. Overlapping clinical and histologic features, and a lack of specific treatments have added to the confusion. This article initially classifies the various panniculititides by their primary histopathologic pattern: (1) septal panniculitis without vasculitis, (2) septal panniculitis with vasculitis, (3) lobular panniculitis without vasculitis, and (4) lobular panniculitis with vasculitis. Subsequently, we describe the key clinical findings in the most important forms of panniculitis. We begin with the most common form of panniculitis, erythema nodosum. Indeed, in many patients suspected of having panniculitis, a worthwhile question to consider initially might be, "Is this, or is this not, erythema nodosum?" before engaging in an elaborate (and expensive) exercise in differential diagnosis.
BACKGROUND: The purpose of the present paper was to describe the clinical manifestations and treatment of patients with panniculitis. METHODS: From January 1983 to December 2002, 4294 patients were treated for pediatric rheumatological diseases at Pediatric Rheumatology Unit, University of São Paulo, Brazil. Of these, 35 children and adolescents (0.8%) presented with panniculitis: erythema nodosum (EN) or Weber-Christian disease (WCD). Clinical characteristics, laboratory exams, biopsy of the lesion, treatment and clinical course were studied. RESULTS: Of the 35 patients, 29 presented with EN and six with WCD, one of these with cytophagic histiocytic panniculitis. Mean age at symptom onset was 85 months (6-204 months) and the mean duration of follow up was 55 months (1-144 months). All the patients presented with inflammatory subcutaneous nodules. The patients with WCD presented with systemic manifestations and cutaneous atrophy. The principal etiologies of EN were streptococcal infection (42%), undetermined (13.5%), pulmonary tuberculosis (10%), and acute rheumatic fever (10%). Biopsy of the nodules indicated septal panniculitis in 14 patients with EN and lobular panniculitis without vasculitis in the patients with WCD, one of which had cytophagic histiocytic panniculitis. There was recurrence in 11 patients (38%) with EN and in all those with WCD. Non-steroidal anti-inflammatory drugs were used in 15 patients with EN and corticosteroids and/or immunosuppressive drugs in the six patients with WCD. Three patients died. CONCLUSIONS: EN is the most frequent panniculitis, with a benign course and is mainly associated with infections. WCD is a severe disease, with systemic involvement, that proceeds with cutaneous atrophy and requires the use of corticosteroids and or immunosuppressive drugs.
BACKGROUND: Eosinophilic panniculitis is characterized by a prominent infiltration of subcutaneous fat with eosinophils. It has been identified in patients who have a variety of associated clinical conditions. OBJECTIVE: Our purpose was to describe clinical conditions associated with eosinophilic panniculitis. METHODS: We describe five patients with eosinophilic panniculitis. These patients had a variety of clinical conditions including arthropod bites, gnathostomiasis, and polyarteritis nodosa. We review the literature on patients with eosinophilic panniculitis. RESULTS: Eosinophilic panniculitis most often presents as a nodule. Gnathostomiasis, leukocytoclastic vasculitis, and erythema nodosum appear to be the most common conditions associated with eosinophilic panniculitis. Other disorders include atopic and contact dermatitis, eosinophilic cellulitis, injection granuloma, arthropod bites, streptococcal and other bacterial infections, toxocariasis, B- and T-cell lymphoma, and refractory anemia with excess blasts. CONCLUSION: Once a diagnosis of eosinophilic panniculitis has been established, appropriate evaluation for an associated clinical condition should be performed.
BACKGROUND: Mesenteric panniculitis is a rare condition with no standard therapy. AIM: To assess the safety and efficacy of thalidomide for the treatment of patients with symptomatic mesenteric panniculitis using a newly established clinical disease activity index (Mesenteric Panniculitis Subjective Assessment Score). METHODS: In an open-label pilot study, five patients with symptomatic mesenteric panniculitis received oral thalidomide, 200 mg nightly, for 12 weeks. The primary end-point was a reduction in the Mesenteric Panniculitis Subjective Assessment Score by > or = 20% at 12 weeks or complete remission (absence of symptoms). RESULTS: Four (80%) of the five patients responded. The median Mesenteric Panniculitis Subjective Assessment Score at baseline was 39 and at week 12 was 25 (average decrease of 44%). One patient achieved complete remission by week 4, which was sustained. At 12 weeks, three (75%) patients experienced a global response, five (100%) patients had a > or = 20% (range, 29-98%) decrease in erythrocyte sedimentation rate and three (75%) patients had a > or = 20% (range, 61-93%) decrease in C-reactive protein. Abdomino-pelvic computed tomography scans were unchanged in all five patients. There were no serious adverse events. CONCLUSIONS: Thalidomide is safe, well tolerated and efficacious in the treatment of some patients with symptomatic mesenteric panniculitis. Further study is indicated.
The extent of eosinophils in skin biopsy and peripheral blood specimens of patients with lupus panniculitis and morphea profunda was determined by studying 37 biopsies from 33 patients with lupus panniculitis and 55 biopsies from 53 patients with morphea profunda. Specimens from 8 (24%) of 33 patients with lupus panniculitis showed eosinophils, and 13 (25%) of 53 patients with morphea profunda had eosinophils in the subcutaneous tissue. In all cases, the diagnosis of lupus panniculitis or morphea profunda was established on the basis of other, more characteristic histologic features; the presence of eosinophils was incidental and not a diagnostic criterion. Occasional cases of lupus panniculitis or morphea profunda had numerous eosinophils. However, in most of the specimens, eosinophils were absent or observed in small numbers. Only 3% of patients with lupus panniculitis showed an increase in the number of eosinophils in the peripheral blood, whereas 47% of patients with morphea profunda had peripheral eosinophilia. The degree of hyaline necrosis in lupus panniculitis or of sclerosis in morphea profunda did not correlate with the number of eosinophils present in the biopsy specimen or peripheral blood.
BACKGROUND: Drug-induced panniculitis are uncommon. We report the second case of panniculitis induced by MINE chemotherapy. CASE REPORT: A 31-year-old woman with relapsed Hodgkin disease was treated with MINE cytostatic regimen. Multiple erythematous and painful nodules of panniculitis developed on her chest, abdomen and thighs fifteen days after the beginning of drug administration with a second flare up after second administration of the same drugs. The eruption cleared slowly after treatment withdrawal. DISCUSSION: To our knowledge, our case is the second reported case of panniculitis induced by MINE chemotherapy. Drug-induced panniculitis is uncommon and usually induced by steroid treatment. Some cases of panniculitis induced by atenolol, potassium bromide, apomorphine, interferon alpha and interleukin 2 have been described. Few cutaneous adverse effects are reported with MINE chemotherapy: rash, erythema and swelling of extremities. A case of inflammatory swelling of thighs with hemorrhagic panniculitis due to this treatment has been described recently.