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Persistent ulcerated necrobiosis lipoidica responding to treatment with cyclosporin.

We report two patients with severe ulcerated necrobiosis lipoidica (NL) who responded to cyclosporin. One patient had suffered persistent ulceration for a period of 7 years and the other had NL of recent onset. In both cases, ulceration healed completely after 4 months of therapy, and both patients have remained free of ulceration since discontinuing therapy. The possible mode of action of cyclosporin in the context of this debilitating disease is discussed.

Adult↗

Non-diabetic necrobiosis lipoidica. Hitherto unrecognized papulonecrotic, nodulo-ulcerative and familial forms of the disease.

Necrobiosis lipoidica of the legs, in which deep ulcers resembling erythema induratum, gummas or a variety of other chronic progressive ulcerating skin diseases occur, is described. In 2 cases the lesions were precipitated by a crush injury elsewhere in the same leg, but not at the site of the ultimate lesions. In 2 further pairs of siblings the same problem arose as a familial complaint without trauma. These cases were distinguished by severe necrosis in the absence of diabetes.

Adult↗

Granuloma annulare and necrobiosis lipoidica tissue reactions as a manifestation of systemic disease.

Granuloma annulare (GA) and necrobiosis lipoidica (NL) are generally considered to be idiopathic cutaneous palisading granulomatous dermatitides. There are sporadic reports of such lesions occurring in patients with coexistent systemic diseases other than diabetes mellitus. Having encountered 49 patients whose skin biopsies showed GA or NL lesions in the setting of extracutaneous disease, the authors set out to assess their clinical and histopathological findings to determine if any parameters were predictive of underlying systemic disease. Fifty-two skin biopsies from 49 patients having either GA or NL in whom there was a clinical history of an associated systemic disease were analyzed by light microscopy. The main systemic disease associations were rheumatologic, endocrine, hematologic, infectious, and inflammatory bowel diseases, ANCA positive vasculitic syndromes, and sarcoidosis. The clinical and histomorphological features were compared with those of a control group of patients whose skin biopsies showed GA or NL and in whom there was no history of extracutaneous disease. For the systemic disease group, patients were selected either retrospectively or prospectively from 160,000 cases accessioned in a 24-month period in the dermatopathology databases of Pathology Services, Inc (Cambridge, MA) and Central Medical Laboratories (Winnipeg, Canada). All systemic disease cases from the former service were analyzed blindly by the second author and from the latter service were analyzed blindly by the first author. Patients in the control group were obtained retrospectively from the Pathology Services Inc. database by the authors. The location of the lesions was atypical in 30 of 34 biopsies from systemic disease patients with a GA tissue reaction versus 10 of 22 biopsies of GA in the control group (P = .001). Six of 18 biopsies from patients with NL tissue reactions in the systemic disease group showed an atypical location, versus only 1 of 9 biopsies of NL from the control group (P = .19). The clinical diagnostic considerations were much broader in the systemic disease group versus the control group and included vasculitis, panniculitis, and connective tissue diseases including morphea in the former. In 22 of 34 GA biopsies and 16 of 18 NL biopsies from the systemic disease group, an active vasculopathy of leukocytoclastic, granulomatous, or thrombogenic subtypes was demonstrable. None of the GA or NL biopsies from the control group showed a similar active vasculopathy. An active vasculopathy was predictive of systemic disease in patients having either a GA-like or an NL-like tissue reaction (P < .001). Fifteen of 34 GA and 7 of 18 NL biopsies in the systemic diseases group showed extravascular neutrophilia in contrast to 3 of 22 GA (P = .02) biopsies and 2 of 9 NL (P = .33) biopsies in the control group. The finding of an active vasculopathy in a skin biopsy specimen showing a GA- or NL-like tissue reaction, particularly in the setting of an atypical clinical presentation both with respect to the location and appearance of lesions, should prompt consideration of an underlying systemic disease, as should extravascular neutrophilia in a skin biopsy showing a GA-like tissue reaction.

Adult↗

[Clofazimine--therapeutic alternative in necrobiosis lipoidica and granuloma anulare].

Twenty patients, ten suffering from disseminated granuloma anulare and ten from necrobiosis lipoidica, were treated with clofazimine 200 mg p.o. daily. Six patients in each group (60%) responded to this regimen, and three of the responders in each group achieved complete remission of the dermatosis. In eight patients (40%) no improvement at all was observed. All the patients treated had reddening of the skin, but this was reversible after the end of therapy, as were the other side-effects, i.e. diarrhoea and dryness of the skin, which were not experienced by all patients.

Administration, Oral↗

High dose nicotinamide in the treatment of necrobiosis lipoidica.

An open study of high dose nicotinamide in the treatment of 15 patients with necrobiosis lipoidica is reported. Of 13 patients who remained on treatment for more than 1 month, eight improved. Improvement took the form of a decrease in pain and soreness, a decrease in erythema and the healing of ulcers if present, although the skin did not return completely to normal in any patient. There were no significant side-effects, particularly with respect to diabetic control, an important finding as lesions tended to relapse if treatment was stopped.

Adult↗

Necrobiosis lipoidica: only a minority of patients have diabetes mellitus.

Although it is commonly accepted that necrobiosis lipoidica (NL) is associated with diabetes mellitus (DM), there is some controversy regarding the degree of this association. In a retrospective review of 65 patients with NL attending our dermatology out-patient clinics we found that just seven (11%) were known to have DM at the time of presentation. Seven further patients (11%) were diagnosed as having impaired glucose tolerance/DM at presentation and over a 15-year follow-up period. Thus, only a minority of our patients with NL had DM.

Administration, Topical↗

[Cutaneous microcirculation in pretibial necrobiosis lipoidica. Comparative laser Doppler flowmetry and oxygen partial pressure determinations in patients and healthy probands].

Necrobiosis lipoidica (NL) is a chronic, skin disease usually localized pretibially and often associated with diabetes mellitus. Primary vascular disturbances are considered pathogenetic for NL. In order to determine microcirculatory alterations in "idiopathic" NL linked with neither arterial hypertension nor diabetes mellitus, we performed simultaneous measurements of laser-Doppler flux (LDF) and oxygen tension (pcuO2) in 10 non-diabetic patients with NL and in 10 age- and sex-matched healthy volunteers. We examined the centre of the pretibial NL plaque, its border and the non-affected skin of the proximal lower leg at probe temperatures of 36 degrees C (flux) and 37 degrees C (pcuO2). Corresponding sites and temperatures were chosen for the controls. In addition, the degrees of hyperaemia caused by arterial occlusion (3 min) and during local heating (42 degrees C) were continuously measured. The initial pcuO2 values were found to be lowered, in contrast to increased fluxes within and around the NL lesions. In addition, responses of both flux and pcuO2 to the hyperaemic stimuli were weaker than in the corresponding skin of the healthy controls. Only minor differences from controls or none at all were found in clinically unaffected lower leg skin of the patients. Our results indicate a local alteration of microcirculation in patients with "idiopathic" NL that occurs independently of diabetes.

Adult↗

Diffuse necrobiosis lipoidica diabeticorum associated with non-insulin dependent diabetes mellitus.

We report a case of diffuse necrobiosis lipoidica (NL) which first appeared on the legs and scrotum, before gradually spreading across the back and arms; the patient also suffered from diabetes mellitus, and the NL lesion began to disappear as the diabetes mellitus was controlled. The possible contribution of various glycation and glycoxidation products of collagen to the pathogenesis of NL is discussed.

Collagen↗

Necrobiosis lipoidica in a 9-year-old girl with new-onset type II diabetes mellitus.

Necrobiosis lipoidica (NL) is an idiopathic dermatologic condition that is strongly associated with, but not pathognomonic for, diabetes mellitus. It is more commonly seen in women than men and in adults than children. We present the youngest child, to our knowledge, diagnosed with NL at initial presentation with type II diabetes mellitus. We review the literature and discuss pathogenesis, clinical features, and treatment options for NL.

Biopsy, Needle↗

Necrobiosis lipoidica diabeticorum: association with background retinopathy, smoking, and proteinuria. A case controlled study.

In order to evaluate patients with necrobiosis lipoidica diabeticorum and to compare them with age, sex, and duration of diabetes matched controls, 15 patients with necrobiosis were each matched with 5 control subjects with diabetes mellitus. Complications of diabetes, glycaemic control, and proteinuria were measured. Patients with necrobiosis (mean age 40, range 18-74 years) had a mean duration of diabetes of 14 (range 3-36) years; 8 patients were male, and 7 were female. For necrobiosis versus controls, background retinopathy (67% vs 27%, p = 0.009), proteinuria (53% vs 17%, p = 0.006), and smoking (60% vs 20%, p = 0.003) were all more common with necrobiosis. There were no significant differences between patients with necrobiosis and control patients in the prevalence of vascular disease and neuropathy. Glycosylated haemoglobin concentrations were higher in patients with necrobiosis (p = 0.02). Blood pressure measurements were similar in both groups. We conclude that smoking, proteinuria, and retinopathy were more prevalent in diabetic patients with necrobiosis; the skin lesion may therefore share common aetiological factors which affect the microvascular circulation, leading to damage to basement membranes and vascular endothelial cells.

Adult↗

[Necrobiosis lipoidica and Koebner phenomenon].

Our report describes a 52-year-old female patient with bilateral foci of necrobiosis lipoidica in pretibial scars. The skin changes appeared 6 months after internal fixation of a tibial fracture in each case.

Cicatrix↗

Concomitant granuloma annulare and necrobiosis lipoidica. Report of a case and review of the literature.

A case of concomitant granuloma annulare (GA) and necrobiosis lipoidica (NL) is presented. The etiology of these two disorders remains obscure. The similarity of the histopathology in GA and NL might suggest a common origin. However, a review of the 5 previous cases of concomitant GA and NL and recent biochemical, immunological and immunohistochemical studies comparing GA to NL indicates an independent etiology for these two histologically related disorders. Some evidence points toward a closer relationship of NL with diabetes than GA, while GA may be related to delayed-type hypersensitivity reactions.

Arm↗

[Studies on the relationship between diabetes mellitus and necrobiosis lipoidica].

From 1970 to 1974 in the dermatological clinic of Leipig University in 17 women and 3 men a necrobiosis lipoidica was established. Of these patients hitherto 12 female patients with histologally ascertained diagnosis underwent a clinico-angiological follow-up examination. Apart from this metabolic disturbances were thought for. In two thirds a disturbance of the carbohypdrate metabolism, in three fourths a disturbance of the lipometabolism was found; hyperlipoproteidaemia and diabetic change of the metabolism were simultaneously present in 7 out of 12 female patients. Uric acid level of the serum and rheumatic serology were, however, rarely pathological. A disturbance of the arterial blood supply of the legs could be ascertained in three fourths of the women who underwent a control examination. In these cases 15 to 19 extremities affected with necrobiosis had a disturbance of the blood supply. The microangiopathy histologically provable in foci of necrobiosis was to be seen only in one long-term diabetic also on the eye-ground. One third of the female patients suffered from hypertension. In all cases disturbances of the carbohydrate metabolism or of the lipometabolism and/or of the local blood supply could be proved. On the basis of these findings adequate examinations are recommendable in patients with necrobiosis lipoidical with regard to therapeutic consequences.

Adult↗