The pathogenesis of necrobiosis lipoidica. Necrobiosis lipoidica, a form fruste of diabetes mellitus.
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Necrobiosis lipoidica is a well recognized but comparatively rare cutaneous complication of diabetes mellitus. The aetiology is probably multifactorial with microangiopathy, immune complex formation, abnormal collagen synthesis and breakdown, and altered haemostasis all thought to play a part. Necrobiosis lipoidica often proves very resistant to treatment. We report a case of a 44-year-old woman with ulcerated necrobiosis lipoidica that healed following grafting with a tissue-engineered living dermal tissue.
Necrobiosis lipoidica has distinctive clinical and histopathologic features. Although theories abound, its cause and pathogenesis remain unknown. Despite many reports of effective treatments, a critical review of the literature suggests that none is uniformly effective.
Necrobiosis lipoidica is a granulomatous skin condition typically occurring on the legs. A patient with this condition presented with gross bilateral induration of the eyelids sufficient to close both eyes.
Necrobiosis lipoidica (NL) is a cutaneous disorder with distinctive clinical and morphologic characteristics. It is associated with diabetes mellitus in two thirds of cases. The aetiology and pathogenesis of NL are unknown but familial cases of NL seem to be extremely rare. We report the occurrence of NL in two sisters with normal glucose tolerance.
50 necrobiosis lipoidica cases which were observed in the last twelve years at the Dept. of Dermatology of the University of Essen were examined. Our studies showed that local metabolic and vascular disturbances play an important factor in the histo- and pathogenesis of the necrobiosis lipoidica (diabeticorum).
Necrobiosis lipoidica diabeticorum is an unusual dermatologic condition with a characteristic clinical appearance and a clear association with diabetes mellitus. There is currently no treatment that reverses the atrophic changes associated with this lesion. We have carried out a clinicopathologic study on 15 subjects and, in addition, have reviewed 10 further biopsy specimens of necrobiosis lipoidica diabeticorum. We found a frequent association of necrobiosis lipoidica diabeticorum with other chronic complications of diabetes mellitus, including limited joint mobility. It is possible that nonenzymatic glucosylation or other changes in collagen may be important in the etiology of necrobiosis lipoidica diabeticorum and the limited joint mobility. We confirmed that cutaneous anesthesia is usually present in the necrobiosis lipoidica diabeticorum lesions. With the use of an antibody to S100 protein and an immunohistochemical method, there was an apparent decreased number of nerves in the skin lesions. We suggest that sensory loss results from local destruction of cutaneous nerves by the inflammatory process. Finally, in six elliptical biopsies extending into clinically normal skin, we demonstrated that the inflammatory infiltrate of necrobiosis lipoidica diabeticorum extended from the lesion into apparently normal skin surrounding clinically active lesions. Thus, intradermal steroids might be administered to perilesional areas surrounding active lesions in the hope of halting progression.
Necrobiosis lipoidica diabeticorum is a rare skin disorder, usually considered a marker for diabetes mellitus. More than half of the patients with necrobiosis lipoidica diabeticorum have diabetes mellitus, but less than one per cent of diabetes mellitus patients have necrobiosis lipoidica diabeticorum. In the diabetes and dermatology literature, we find the position that there is no effect of glucose control on either the appearance of necrobiosis lipoidica diabeticorum or the clinical course of the lesion. We base our challenge to this position on a critical review of the original data. And conclude on the contrary, that necrobiosis lipoidica diabeticorum is usually associated with poor glucose control and that tighter glucose control, as currently practised, might improve or prevent the disorder.
INTRODUCTION: Perforating necrobiosis lipoidica is a rare clinical form of necrobiosis lipoidica and is almost always associated with diabetes. Necrotized tissue is usually eliminated via transfollicular perforations. CASE REPORT: We report two cases of perforating necrobiosis lipoidica associated with type II diabetes mellitus. The characteristic clinical feature was the presence of keratotic plugs around the periphery of the lesions. Transfollicular perforation predominated in the first case. The second also presented epidermal perforation. DISCUSSION: There have been only 6 cases of perforating necrobiosis lipoidica in the literature. To our knowledge, this is the first case report of transepidermal elimination. We confirmed the constant association between this perforation type and diabetes mellitus.
Necrobiosis lipoidica diabeticorum is a chronic cutaneous complication of diabetes mellitus with microangiopathy as an important pathophysiologic factor. Because of the known success of hyperbaric oxygen in the treatment of chronic non-healing wounds, we used this mode of therapy to treat a diabetic patient with ulcerated necrobiosis lipoidica of 7 years' duration, refractory to medical and surgical treatment. The patient received daily sessions of hyperbaric oxygen therapy. There was considerable improvement during the course of the treatment, with complete closure of all the ulcerations after 98 sessions. The success of this treatment emphasizes the role of hypoxia in the pathogenesis of the lesion. This simple and safe treatment method may be a good solution for patients with chronic nonhealing necrobiosis lipoidica which fails to respond to other therapeutic approaches.
Necrobiosis lipoidica confined to the face and scalp is very rare. The clinical diagnosis is complicated by the granuloma anulare like aspect and in particular by the lack of typical localization of the extremities. Histologically prominent granulomatous dermal proliferation and less marked vascular changes can often be found in the involved areas of the scalp and/or in the absence of diabetes mellitus. For the microscopic classification the large variability of necrobiosis lipoidica must be considered. In our case with an exclusive localization in the face and capillitium, diabetes mellitus was excluded. The necrobiotic type of the necrobiosis lipoidica was predominant. The identity between necrobiosis lipoidica with other chronic granulomatous diseases and the possible treatment of this benign but long-term process is briefly discussed.
Necrobiosis lipoidica often fails to respond adequately to therapy with topical and intralesional corticosteroids, or to systemic medications like niacinamide and pentoxifylline (Trental). On the basis of unpublished work which showed a predominance of T helper cells in lesions of necrobiosis lipoidica, and recalling the case of a woman whose necrobiosis lipoidica improved after she was started on cyclosporine for a renal transplant, systemic cyclosporine was successfully used in the cases of two young women who had insulin-dependent diabetes and were disfigured by severe, ulcerating necrobiosis lipoidica on the anterior lower legs. Response to treatment was monitored with photographs. In both cases the ulcers resolved, and remained in remission after cyclosporine was stopped.
The most important morphological aspects and pathogenesis of necrobiosis lipoidica concerning histological and clinical aspects are reported. In case of diabetes we find more frequently necrobiosis lipoidica localized out of the shank than in cases of necrobiosis lipoidica without diabetes. Necrobiosis lipoidica must be differentiated from granulomatosis disciformis, localized scleroderma and atrophy of the skin of another origin. Beside normalization of diabetes and application of corticosteroids physical and surgical treatment is recommended.
Necrobiosis lipoidica occurs most commonly on the lower extremities in patients with diabetes; lesions typically occur in the pretibial region. Although the pathogenesis of necrobiosis lipoidica remains unclear, both external trauma and vascular damage have been proposed as contributing to the development of this disorder. We report polarizable foreign material in small blood vessels and multinucleated histiocytes in lesions of necrobiosis lipoidica. This phenomenon occurred in a patient who was both diabetic and an intravenous drug user.
Necrobiosis lipoidica is a skin disorders with an interesting predisposition for areas of trauma such as the anterior shins. In this report a patient with diabetes mellitus and generalized necrobiosis lipoidica diabeticorum with localization in surgical scars is described. A brief review of other skin disorders occurring in scars is also included.
The microradiogram of granuloma anulare was compared with that of necrobiosis lipoidica. In the areas of cellular infiltration, the amount of connective tissue missing depended on the degree of infiltration. Apparently the collagenous bundles were destroyed, not pushed aside. In the necrobiotic type of necrobiosis lipoidica, the damage to collagenous tissue was more severe than one would have expected from the stained sections. The collagenous bundles were generally swollen, and there was a complete lack of normal high-density bundles in the damaged areas, in contrast to granuloma anulare and to the granulomatous type of necrobiosis lipoidica, however, the damage to the collagenous tissue seemed less severe in the microradiograms than in the stained sections. Differences between granuloma anulare and necrobiosis lipoidica were to be found in the microradiogram (see tables), but in general a differential diagnosis is only possible in severe cases, in spite of the different clinical picture.
Necrobiosis lipoidica belongs to the inflammatory granulomatous skin disorders groups with palisade reaction. The association to diabetes mellitus is classical involving 80% of diabetics in its tibial localization. A retrospective study of all cases of necrobiosis lipoidica conducted in our dermatology department over a-15-year period, found 4 diabetic patients, 3 of whom were women known as diabetic patient with extremities involvement, and 1 man not known as diabetic patient at the time of the diagnosis. Necrobiosis lipoidica appears to be a rare disorder in diabetic people and this association seems to be over estimated.