Eosinophilic panniculitis: a clinicopathologic study

Study of 18 patients with biopsy diagnoses of eosinophilic panniculitis revealed diverse patterns of systemic disease, including Wells' syndrome, vasculitis, atopy, and erythema nodosum as well as localized panniculitis. Significant associated diseases included psychiatric illness, 6 (drug dependency, 4); atopy, 5 (asthma, 3); malignancies, 5; immune complex vasculitis, 4; thyroid disease, 2; Wells' eosinophilic cellulitis, 2; glomerulonephritis and sarcoidosis, 1 each. The skin lesions varied from urticarial papules and plaques to purpura, pustules, and ulcerative lesions but always included a nodular subcutaneous component, frequently as a presenting complaint. Eosinophilic panniculitis is a non-specific finding that can signify localized disease, such as an insect bite or injection lipophagic granuloma in a drug-dependent patient, or systemic lymphoma or immune reactive disease. Eosinophilic panniculitis in erythema nodosum is perhaps its most confusing presentation.     

Lupus erythematosus panniculitis: a clinicopathologic study

BACKGROUND: Lupus erythematosus panniculitis is a clinical variant of lupus erythematosus which involves the deep dermis and the subcutaneous fat. The purpose of this study was to ascertain the clinical profile of Asian patients with this condition. METHODS: This was a retrospective study of all histologically confirmed lupus panniculitis seen at our center between 1992 and 1997. The age, sex, past history/subsequent diagnosis of systemic lupus erythematosus (SLE), presence of clinical discoid lupus erythematosus (DLE) changes on overlying skin, direct immunofluorescence, serologic, and histologic findings were analyzed. RESULTS: There were 12 cases of lupus panniculitis, two of which were in patients already diagnosed with SLE and one in which the patient subsequently evolved into SLE. The mean age at diagnosis was 31.3 years. The face (50%), upper limbs (33%), and scalp (25%) were the most common sites of involvement. Thirty-three per cent had clinical evidence of DLE on the overlying skin, whilst 67% had histologic features of DLE on the overlying skin. A lupus band was present in 36%. Antinuclear antibody (ANA) was positive in three of 11 cases; these were in the two patients who already had SLE and in the only patient who progressed to SLE. All of the cases showed fat necrosis and, in the majority of cases, there was associated lobular and paraseptal inflammation. Thirty-three per cent showed lymphocytic vasculitis and 75% had mucin deposition. None had lymphoid nodules, subepidermal hyalinization, or calcification. CONCLUSIONS: Lupus panniculitis affects a younger age group in Asians as compared with the Western population. Although about one-third of patients show clinical evidence of overlying DLE, two-thirds of patients show histologic evidence of DLE. It tends to have a mild disease course in the majority of cases.     

Panniculitis with liquefaction--a special manifestation of Pfeiffer-Weber-Christian panniculitis

A case of nodular panniculitis with liquefaction in a 58-year-old woman patient is reported. It seems that Pfeiffer-Weber-Christian disease and nodular panniculitis with liquefaction are varying expressions of the same disease entity. The relationship to cases reported in the literature is discussed.     

Idiopathic nodular panniculitis in Niemann-Pick disease

Idiopathic nodular panniculitis is a condition characterized by the recurrent appearance of inflammatory nodules in the subcutaneous fat. In this report, an infant affected with Niemann-Pick disease and recurrent lobular panniculitis is described and discussed.     

Lipomembranous panniculitis: clinicopathologic correlation of 8 cases

IntroductionThe term lipomembranous panniculitis refers to a chronic inflammation of the subcutaneous cell tissue, probably representing a non-specific type of ischemic necrosis of the fatty tissue, common to several complaints. It is characterized by painful sclerotic subcutaneous deposits, located in the lower legs of obese, middle-aged woman, with a history of vascular insufficiency and stasis dermatitis. The condition may also appear in association with other inflammatory disorders.MethodsThis paper is a review of all cases of lipomembranous panniculitis diagnosed at the Skin Histopathology Laboratory at Santa Maria Hospital, Lisbon, from 1985-2005. In the histopathological study, the associated clinical processes were retrospectively analyzed and the risk factors/associated pathologies re-evaluated.ResultsEight patients were identified, all women, and with an average age of 49. Over half the patients were overweight. In most cases the lesions had been evolving for over 6 months and were associated with chronic venous insufficiency of the legs, both from a clinical point of view (7 patients) and a histopathological point of view (6 patients). In two of the cases, the lesions occurred in patients with connective tissue disorders.ConclusionGreat variability was observed with regard to the clinical morphology of the lesions, the proposed diagnoses and prescribed treatments, all of which possibly highlight the non-specific nature of the process.     

Panniculitis associated with amyopathic dermatomyositis

Panniculitis is a rare clinical finding in dermatomyositis, with less than 30 cases reported and there is only one case associated with the amyopathic subtype described in the literature. The present report describes a 49-year-old female patient that one year after being diagnosed with amyopathic dermatomyositis, presented indurated, painful, erythematous to violaceous nodules located on the upper limbs, thighs and gluteal region. Skin biopsy revealed lobular panniculitis with a lymphocytic infiltrate. The patient was treated with prednisone and methotrexate, but remained unresponsive to treatment.     

Calcifying panniculitis associated with renal insufficiency: a tissue calciphylaxis syndrome

Among the cutaneous manifestations of hyperparathyroidism, cases of panniculitis with calcification of the adipose tissue and necrosis of the skin have recently been reported, the mechanism incriminated being calciphylaxis, as defined by Selye on the basis of experiments. Experimental calciphylaxis consists of local or systemic calcium deposits followed by inflammatory necrosis or sclerosis. The deposits are induced by "provoking" or precipitating factors (metal salts, albumin, traumas) after a phase of sensitization (to parathyroid hormone, vitamins D2 or D3, dihydrotachysterol), provided a critical period is allowed between these two phases; the duration of that period depends on the experimental conditions. The case reported here concerns a 64-year old obese and diabetic woman who had presented with hard and tender nodosities and plaques in her abdominal and crural panniculi, ending in extensive and hyperalgesic necrosis (fig. 1 and 2). The panniculitis had occurred in a peculiar context: at the end of an episode of renal failure complicated with secondary hyperparathyroidism (serum PTH 12.9 mIU/ml; N = 1.5-4.4 mIU/ml) with moderate increase to 5,000 of the P x Ca product. Histological examination of a nodule of the thigh disclosed multiple foci of microcalcification (fig. 3, 4, 5) within the adipose lobules, in the interadipocyte spaces, in connective tissue septa and in the adventitia of small vessels (positive Von Kossa reaction). Electron microscopy showed dense calcium deposits between adipocytes, in subcutaneous septa (fig. 6, 8) and in more or less damaged vascular walls (fig. 9). Within the microfibrillar and granular fundamental substance, microcrystals looking like hydroxyapatite crystals (fig. 7) conglomerated into pincushion-like formations becoming increasingly denser and more compact.(ABSTRACT TRUNCATED AT 250 WORDS)     

Systemic nodular panniculitis in a patient with alpha-1 antitrypsin deficiency (PiSS phenotype)

The association of alpha-1 antitrypsin deficiency (PiZ phenotype) with systemic nodular panniculitis has been well documented. Despite reports of cases of systemic nodular panniculitis associated with other alpha-1 antitrypsin deficiency phenotypes, it is still not known if this association is fortuitous rather than causal. We report a case of systemic nodular panniculitis associated with alpha-1 antitrypsin deficiency (PiSS phenotype), with clinicopathological features similar to those reported in cases associated with the PiZ phenotype.     

Cutaneous zygomycosis associated with urate panniculitis

Cutaneous zygomycosis is being increasingly recognized as a serious and life-threatening infection in debilitated and immunosuppressed patients, including transplant patients. The organisms are morphologically distinct but difficult to grow in cultures from clinical samples. We report a case of cutaneous zygomycosis in a neonatal multi-visceral organ transplant patient, with subcutaneous panniculitis accompanied by extensive local acicular uric acid crystal deposition. Although the patient's serum uric acid was subsequently found to be in the normal range, transient hyperuricemia could not be excluded. Because we use a microwave-based processing system avoiding aqueous solutions, the crystals were maintained in the tissue sections and were shown by various methods to consist of monosodium urate. Early diagnosis combined with extensive debridement and prompt antifungal therapy resulted in a successful outcome. We have coined the term "urate panniculitis" to describe this phenomenon.     

Panniculitis in a patient with dermatomyositis

Panniculitis is a rarely reported clinical finding in dermatomyositis. It may precede the other manifestations associated with dermatomyositis by as much as 14 months. In all cases, myositis and panniculitis improve simultaneously during treatment. The present report describes the case of a 30-year-old female patient with clinical and histopathological findings consistent with panniculitis two months after the onset of the muscle and cutaneous symptoms that permitted diagnosis of dermatomyositis. The skin lesions regressed following steroid treatment.