Necrobiosis lipoidica

A 69-year-old patient presented with different skin lesions all of which belonged to group of necrobiosis lipoidica. The initial histologic diagnosis was actinic granuloma O??Brien. A subsequent biopsy was interpreted as granulomatous necrobiosis lipoidica. The history of these necrobiotic variants is reviewed and exemplarily depicted with this case. Necrobiosis lipoidica is part of the spectrum of granulomatous skin disorders. Although its etiology is unclear, an association with diabetes mellitus is often discussed. Multiple therapeutic options exist, but standardized guidelines for treatment are missing.     

Necrobiosis lipoidica and sarcoidosis

We report the case of a female patient with necrobiosis lipoidica of the lower legs and coexistent systemic and cutaneous sarcoidosis. We review the six previously reported patients with coexisting necrobiosis lipoidica and sarcoidosis. The associations between the granulomatous disorders of the skin, especially necrobiosis lipoidica, sarcoidosis and granuloma annulare, are discussed. The common pathogenetic features of these disorders are reviewed.     

Erfolgreiche Therapie einer exulzerierten Necrobiosis lipoidica non diabeticorum mit Ciclosporin

Necrobiosis lipoidica is an inflammatory granulomatous skin disease of unknown etiology which is associated with diabetes mellitus in about 60% of the patients. In 15-35% of the affected patients painful ulcerations may occur after minimal trauma which can be extremely refractory to therapy. Because of the unknown pathomechanisms, current therapeutic options are limited. We report on a 68-year-old patient with an 18 year history of ulcerated necrobiosis lipoidica non diabeticorum of both lower limbs, which responded to systemic cyclosporine A. Based on this case, we discuss the role of cyclosporine A in patients with necrobiosis lipoidica in the context of the disease etiology.     

Ulcerative necrobiosis lipoidica responsive to colchicine

Necrobiosis lipoidica is an uncommon granulomatous disease of unknown aetiology. Few treatments have emerged with consistent efficacy and the ulcerated form of necrobiosis lipoidica can be particularly difficult to treat. A 56‐year‐old non‐diabetic woman with chronic ulcerative necrobiosis lipoidica unresponsive to other therapies was commenced on colchicine treatment. Complete resolution of the ulcers was observed after 2 months therapy with colchicine 500 µg twice daily.     

Systemic sarcoidosis with necrobiosis lipoidica-like scalp lesions.

A 78-year-old woman is described, having systemic sarcoidosis for 15 years, involving the lungs, lymph nodes and the skin. Over the last 6 years she developed a progressive cicatricial alopecia with histologic changes of the granulomatous type of necrobiosis lipoidica surrounded by sarcoid granulomas.     

Erfolgreiche Behandlung einer Patientin mit therapierefraktärer, exulzerierter Necrobiosis lipoidica non diabeticorum mit Adalimumab

Necrobiosis lipoidica is a rare inflammatory granulomatous skin disease of unknown etiology which is associated with diabetes mellitus in about 60?% of the patients. In up to 30?% of these patients extremely painful and often hard-to-heal ulcerations occur in the course of the disease. We present a new therapeutic option using adalimumab to treat refractory ulcerated necrobiosis lipoidica non diabeticorum. The clinical efficacy of adalimumab probably reflects an immunomodulatory effect through the specific TNF-α inhibition which is one central aspect of the underlying inflammation. Thus, adalimumab could represent promising new treatment option, especially for patients with otherwise therapy-refractory ulcerated necrobiosis lipoidica.     

Epidermal dendritic S100 positive cells in necrobiosis lipoidica and granuloma annulare

Using an antibody to S100 protein, the number of dendritic cells above the basal layer in the epidermis was assessed in necrobiosis lipoidica and granuloma annulare. A statistically significantly higher number of these cells was found within the epidermis in necrobiosis lipoidica compared with granuloma annulare and normal skin. The numbers were similar to those seen in sarcoidosis and tuberculous reactions in the skin, which raises the possibility of an immune pathogenesis for necrobiosis lipoidica.     

Fractional CO2 laser: a novel therapeutic device for refractory necrobiosis lipoidica

Necrobiosis lipoidica diabeticorum is a granulomatous skin disease of uncertain pathogenesis. Many therapeutic approaches have been reported in the literature, but none of them can be considered the gold standard. Fractional CO₂ laser treatment shows peculiar effects in the skin, mainly due to its ability of modulating cytokine pathways of tissue‐repairing mechanisms. Thus, we propose fractional CO₂ laser in the management of refractory necrobiosis lipoidica in selected recalcitrant patients.     

Seltene ulzerierende kutane und systemische Sarkoidose

Sarcoidosis is a granulomatous multisystemic disease of unclear etiology, which can affect any organ. The cutaneous manifestations are variable, but ulcerative cutaneous sarcoidosis is very rare. One must rule out other granulomatous skin diseases, especially necrobiosis lipoidica. There is no standarized therapy; usually an interdisciplinary approach over years taking multiple side effects into consideration is needed. A 58-year-old woman with a long history of cutaneous, nodal and pulmonary sarcoidosis suddenly developed ulcerations within the disseminated skin lesions on her legs. The combination of systemic hydroxychloroquine and modern wound management lead to complete healing of the ulcers and a significant improvement in the remaining skin lesions.     

Necrobiosis lipoidica and sarcoidosis

The case is described of a female patient with necrobiosis lipoidica of the legs, and a previous history of acute sarcoidosis. There has been only one previous report of an association between necrobiosis lipoidica and sarcoidosis.¹ The relationship between these disorders may be suggested by the known association of sarcoidosis and other granulomatous and necrobiotic disorders.     

Alterations of connective tissue in granuloma anulare and in necrobiosis lipoidica. Microradiographic studies]

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 diabeticorum: a clinicopathologic study

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

A 68-year-old woman presented with a 3-year history of asymptomatic, yellow-brown plaques on the lower extremities with trauma-related superficial ulcers. A biopsy specimen was consistent with necrobiosis lipoidica. Necrobiosis lipoidica is a chronic granulomatous dermatitis that is associated with diabetes mellitus; however, its pathogenesis remains unclear. Topical glucocorticoids are first-line therapy; however, no treatment has proven efficacy in double-blind, placebo-controlled studies.     

Pulsed dye laser treatment of necrobiosis lipoidica: report of a case

A 23-year-old woman with long-standing insulin dependent diabetes presented with necrobiosis lipoidica. She was particularly troubled by the telangiectatic element of the lesion and therefore underwent test treatment with the pulsed dye laser. At low fluences minimal therapeutic effect was achieved, and at higher fluences skin breakdown occurred. This resolved with conservative management. It would appear that caution is required when attempting to treat necrobiosis lipoidica with laser.     

Transcutaneous measurement of oxygen and carbon dioxide pressure in necrobiosis lipoidica

Transcutaneous measurement of oxygen pressure (PcO2) and carbon dioxide pressure (PcCO2) was performed in nine patients with histologically confirmed necrobiosis lipoidica. None of the patients had diabetes mellitus. All measurements were taken at the lower leg. In each case, the atrophic center, the inflamed border, and the surrounding clinically normal skin of necrobiosis lipoidica were examined at 44 degrees C sensor temperature (maximal vasodilatation). Statistically significant hypoxia was found in the area of necrobiosis lipoidica, which was even more pronounced in the inflamed border. Inhalation of 100% oxygen provoked a marked increase in the PcO2 in the lesion, but the values were still significantly lower than in the normal skin. At the edge of the lesions the PcCO2 was significantly elevated. These findings support a vascular origin of necrobiosis lipoidica, involving reduced vascular perfusion combined with diffusion block.     

Pulsed dye laser treatment of necrobiosis lipoidica: report of a case

A 23-year-old woman with long-standing insulin dependent diabetes presented with necrobiosis lipoidica. She was particularly troubled by the telangiectatic element of the lesion and therefore underwent test treatment with the pulsed dye laser. At low fluences minimal therapeutic effect was achieved, and at higher fluences skin breakdown occurred. This resolved with conservative management. It would appear that caution is required when attempting to treat necrobiosis lipoidica with laser.     

Necrobiosis lipoidica diabeticorum with cholesterol clefts in the differential diagnosis of necrobiotic xanthogranuloma

The histopathologic findings in 331 cases of necrobiosis lipoidica diabeticorum seen during a 50-year period were reviewed. Three cases showing cholesterol cleft formation were found. All 3 cases were associated with severe diabetes mellitus. The differential diagnosis of importance is necrobiotic xanthogranuloma. Common features included extensive hyaline necrobiosis and foreign-body giant cells. Atypical and Touton-type giant cells are more common in necrobiotic xanthogranuloma. Vascular changes in necrobiotic xanthogranuloma may include granulomatous involvement of muscular walls with thrombosis. Explanations for cholesterol cleft formation are offered. When cholesterol clefts are seen in biopsy specimens of necrobiosis, necrobiotic xanthogranuloma must be ruled out. In addition, when found in necrobiosis lipoidica diabeticorum, these clefts may imply diabetes mellitus with complications.     

Defective neutrophil migration in granuloma annulare, necrobiosis lipoidica, and sarcoidosis.

The neutrophil mobility in patients with granuloma annulare, necrobiosis lipoidica, and sarcoidosis was tested using both in vivo and in vitro techniques. Use of a skin window chamber for measurement of chemotaxis in vivo demonstrated defective neutrophil migration in each group. This contrasted with the finding that, in vitro, chemotaxis toward casein and endotoxin-activated sera was normal. The importance of this dissociation is discussed. Abnormal neutrophil accumulation at sites of inflammation may be of importance in the pathogenesis of these granulomatous disorders.     

Squamous cell carcinoma arising in an area of long-standing necrobiosis lipoidica

BACKGROUND: Squamous cell carcinoma in an area of chronic ulceration is a well-documented phenomenon. However, its occurrence arising de novo in an area of necrobiosis lipoidica is rare. METHOD: We report a case in a 53-year-old female who presented with a 2 month history of an erythematous nodule occurring in a plaque of necrobiosis lipoidica on the medical aspect of the right lower leg. She had a background of poorly controlled Type 1 diabetes. RESULTS: Histopathological findings revealed a well-differentiated squamous cell carcinoma overlying an area of necrobiosis lipoidica. Treatment was by excision and split-thickness skin graft. CONCLUSION: Clinicians should be aware of malignant transformation within a plaque of necrobiosis lipoidica. Early detection will allow conservative treatment.     

Cutaneous Sarcoidosis Clinically Mimicking Necrobiosis Lipoidica in a Patient with Systemic Sarcoidosis

A 70-year-old woman with an 8-year history of systemic sarcoidosis developed round, red-brown eruptions, with central atrophic lesions on her lower legs. The features of the biopsy specimen resembled those of necrobiosis lipoidica (NL), but although necrobiosis was present there were well-formed non-necrotizing granulomas in the dermis. The histological diagnosis was cutaneous sarcoidosis. Systemic sarcoidosis presenting with NL has rarely been reported. The histological features of cutaneous sarcoidosis sometimes mimic those of other granulomatous diseases, including NL and granuloma annulare, which are difficult to distinguish. We discuss the novel association between sarcoidosis and other granulomatous diseases.