Post-traumatic ulcerated and chronic necrobiosis lipoidica of the elbow: A new entity?

BACKGROUND: Necrobiosis lipoidica (NL) is a chronic granulomatous dermatosis usually affecting the lower limbs, although less common sites have been described. Herein we report a series of cases of NL located on the elbow, with an unusual presentation and occurring after trauma or surgery. OBSERVATIONS: Our series includes three men and one woman, with a mean age of 64 years. Three had undergone surgery for elbow bursitis and one had had trauma after a fall from a horse, with exposure of subcutaneous tissue prior to healing. Within 5 years, they had all developed an atrophic erythematous annular plaque with papular and telangiectatic edges, with recurrent episodes of ulceration and scarring. Repeated tests for infectious agents were negative. Histological examinations showed granulomas and necrobiosis with palisading or early-stage palisading. Partial healing was achieved in two patients after 6 months of doxycycline. Treatment with adalimumab resulted in disappearance of the ulcers at 6 months in one patient. DISCUSSION: Unusual sites of NL impose consideration of other types of palisading granuloma or mycobacterial infections, which we were able to rule out. Two other cases of NL of the elbow similar to ours are reported in the literature. These cases, involving multiple ulcerations over a very long period of time, probably constitute a distinct entity because of the very distinct character of these 6 cases. Tetracyclines are partially active and tumour necrosis factor alpha (TNF)-alpha inhibitors may offer an option.

Necrobiosis Lipoidica in a Patient with ß-Thalassemia Major: A Case Report and Review of the Literature.

Necrobiosis lipoidica (NL) is a rare granulomatous disease that predominantly affects middle-aged women and is often associated with diabetes mellitus (DM), rheumatoid arthritis (RA) and other metabolic disorders. Thalassemias are the most common hereditary hemoglobin (Hb) disorders worldwide. A few studies investigated dermatologic problems that coexist with ß-thalassemia major (ß-TM). The most common skin disorders in patients with ß-TM are xerosis, urticaria, pseudoxanthoma, hyperpigmentation, leg ulcers and small-vessel vasculitis. Necrobiosis lipoidica has only been occasionally reported in patients with ß-TM. Herein, we present a female with ß-TM and NL. Furthermore, a brief review of the literature was performed.

Risk factors for necrobiosis lipoidica in Type 1 diabetes mellitus.

AIMS: To compare the clinical and metabolic characteristics of patients with Type 1 diabetes and necrobiosis lipoidica with those of patients with Type 1 diabetes who do not have necrobiosis lipoidica. A multicentre analysis was performed. METHODS: Clinical and laboratory data were obtained from 64 133 patients (aged 0-25 years) with Type 1 diabetes with and without necrobiosis lipoidica who were registered in the German/Austrian Diabetes Prospective Documentation Initiative registry. Data were analysed using multivariable regression modelling. Age, diabetes duration, treatment year and sex were considered as confounding factors. RESULTS: Results adjusted for demographic variables are presented. In patients with necrobiosis lipoidica, metabolic control was worse (HbA1c 72 vs. 67 mmol/mol, 8.7% vs. 8.3%; P = 0.0065) and the duration of diabetes was longer [6.24 (3.28-9.97) vs. 5.11 (2.08-8.83) years; P = 0.014; not adjusted]. Patients with necrobiosis lipoidica required higher insulin doses than those without (1.02 vs. 0.92 U/kg/day; P < 0.0001). There was no significant difference in the frequency of microvascular complications (microalbuminuria and retinopathy) between the groups. Furthermore, 24.8% and 17.5% of patients with Type 1 diabetes with and without necrobiosis lipoidica, respectively, had elevated thyroid antibodies (P = 0.051). Necrobiosis lipoidica was correlated with coeliac disease in patients with Type 1 diabetes (3.4% vs. 1.0%; P = 0.0035). CONCLUSIONS: Our data indicate a strong correlation between hyperglycaemia and the development of necrobiosis lipoidica. We postulate that the underlying pathogenic processes differ from those leading to microalbuminuria and retinopathy, and additional immunological mechanisms may play a role.

A granulomatous conundrum: Concurrent necrobiosis lipoidica, cutaneous sarcoidosis and erythema nodosum in a nondiabetic patient.

Necrobiosis lipoidica (NL) and cutaneous sarcoidosis are granulomatous disorders with a largely unknown aetiopathogenesis. Evidence of co-existing NL and sarcoidosis in the same patient may suggest a degree of overlap between these entities through shared granulomatous inflammatory pathways. Occasionally, one condition can mimic the other, making their distinction difficult. We report a novel case of a non-diabetic woman who presented with concurrent NL, cutaneous sarcoidosis and erythema nodosum. We discuss some of the complexities distinguishing these entities and propose that they may represent different stages of the same granulomatous process linked through yet unknown pathomechanisms.

Refractory Ulcerated Necrobiosis Lipoidica: Closure of a Difficult Wound with Topical Tacrolimus.

OBJECTIVE: To report a case of refractory ulcerated necrobiosis lipoidica (NL) with significant response to treatment with topical tacrolimus. SUBJECT: A 55-year-old woman without diabetes and with a previous history of NL presented to the Helen L. and Martin S. Kimmel Hyperbaric and Advanced Wound Healing Center of NYU Langone Medical Center, New York, with bilateral lower-leg ulcerations resistant to wound healing techniques at other institutions. MATERIALS AND METHODS: Repeat biopsy performed at the author's institution confirmed the diagnosis of NL. Initial therapy was based on reports of other successful treatment methods, which included collagen wound grafts and collagen-based dressings coupled with compression. These methods initially showed promising results; however, the wounds reulcerated, and any gains in wound healing were lost. Alternative options were initiated, including topical clobetasol and narrowband ultraviolet B; however, no significant improvement was observed. The patient's lower-extremity wounds began to deteriorate. The patient also refused systemic therapy. Treatment was changed to topical 0.1% tacrolimus ointment and was applied daily for 10 months with multilayer compression wraps. RESULTS: Both lower-extremity ulcerations began to show significant improvement, with the ulcers progressing toward closure except for 1 very small area on the left lower extremity. CONCLUSIONS: Topical tacrolimus seems to be an effective treatment option for patients with refractory chronic ulcerated NL who do not want systemic oral therapy. The authors found that successful wound closure may require a multimodal approach, which promotes wound healing, but also concurrently addresses the underlying disease process.

Ustekinumab to target granulomatous dermatitis in recalcitrant ulcerative necrobiosis lipoidica: case report and proposed mechanism.

We present a 42-year-old woman with no history of diabetes or glucose intolerance who had a 5-year history of ulcerative necrobiosis lipoidica (NL). Despite failure of multiple medications, she experienced clearing of her ulcers after her treatment was changed to ustekinumab. We discuss our patient's disease course and elaborate upon mechanistic reasons for her improvement related to ustekinumab therapy.

Ulcerated necrobiosis lipoidica as a rare cause for chronic leg ulcers: case report series of ten patients.

Necrobiosis lipoidica is a rare granulomatous disorder of the skin. In up to 30% of the affected patients it can lead to ulcerations, which can impair the quality of life and are also very difficult to treat. Its pathogenesis is not fully understood. Only few studies focussing on necrobiosis lipoidica can be found, but none of them focus on ulcerated necrobiosis lipoidica. Therefore, we collected demographic data and comorbidities and assessed treatment options for patients with ulcerated necrobiosis lipoidica. Data of patients who were treated in the wound care centre of the University Hospital of Essen for ulcerated necrobiosis lipoidica over the past 10 years were retrospectively analysed. Hence, data of altogether ten patients (nine women and one man) with ulcerated necrobiosis lipoidica were collected. Of these, 70% of the patients had diabetes mellitus of which 30% had type I diabetes and 40% had type II diabetes; 60% of the patients suffered from arterial hypertension, obesity and hypercholesterolaemia; 40% of the patients suffered from psychiatric disorders such as depression and borderline disorder. Our clinical data demonstrate an association of ulcerated necrobiosis lipoidica and aspects of metabolic syndrome. This leads to a conclusion that ulcerating necrobiosis lipoidica can be seen as part of a generalised inflammatory reaction similar to the inflammatory reaction already known in the pathophysiology of rheumatoid diseases or psoriasis. In patients with clinical atypical painful ulcerations, necrobiosis lipoidica should be considered as a possible differential diagnosis. Therapists should be aware of associated aspects in patients with ulcerated necrobiosis lipoidica who besides diabetes often suffer from other aspects of a metabolic syndrome with increased cardiovascular risk factors. Therefore, these related comorbidities should also be diagnosed and treated.

Necrobiosis lipoidica: a descriptive study of 35 cases.

INTRODUCTION AND OBJECTIVES: Necrobiosis lipoidica (NL) is a chronic idiopathic granulomatous disease considered to occur in association with diabetes mellitus. Data on the frequency of this association, however, are inconsistent. Our aim was to retrospectively analyze the clinical characteristics of patients diagnosed with NL at our hospital and to investigate the association with diabetes mellitus and other diseases. MATERIAL AND METHODS: We performed a chart review of all patients with a clinical and histologic diagnosis of NL treated and followed in the dermatology department of Hospital de Bellvitge in Barcelona, Spain between 1987 and 2013. RESULTS: Thirty-five patients (6 men and 29 women with a mean age of 47.20 years) were diagnosed with NL in the study period. At the time of diagnosis, 31 patients had pretibial lesions. Thirteen patients (37%) had a single lesion at diagnosis, and the mean number of lesions was 3.37. Twenty-three patients (65.71%) had diabetes mellitus (type 1 in 10 cases and type 2 in 13). In 20 patients, onset of diabetes preceded that of NL by a mean of 135.70 months. The 2 conditions were diagnosed simultaneously in 3 patients. None of the 35 patients developed diabetes mellitus during follow-up. Six patients had hypothyroidism, and 4 of these also had type 1 diabetes. CONCLUSIONS: NL is frequently associated with type 1 and 2 diabetes. Although diabetes tends to develop before NL, it can occur simultaneously.

Necrobiosis lipoidica.

BACKGROUND: Necrobiosis lipoidica presents with a distinctive appearance making it an important clinical diagnosis. OBJECTIVE: To describe a case of necrobiosis lipoidica in a patient with type 1 diabetes mellitus, and to discuss differential diagnoses and management. DISCUSSION: Necrobiosis lipoidica is most commonly found on the shins, presenting as a well-defined plaque. Management is challenging and options are discussed. Avoiding ulceration is a key concern.

[Successful treatment of a patient with ulcerated necrobiosis lipoidica non diabeticorum with adalimumab]. / 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.

Atypical Ulcerative Necrobiosis Lipoidica Diabeticorum: A Case Study.

Necrobiosis lipoidica is a granulomatous skin condition commonly associated with diabetes. When associated with diabetes mellitus, the name expands to necrobiosis lipoidica diabeticorum (NLD). In these patients, this rare condition has an incidence rate of 0.3% to 1.6%. The cause of NLD remains unknown even though many speculations exist in the medical literature. The treatment of this condition has most researchers agreeing on the use of topical steroids for the anti-inflammatory effect on NLD; however, the role of glucose control in the treatment of this disorder has been debatable. The following case presents a patient who, despite better glucose control, did not improve until the introduction of doxycycline.

Necrobiosis lipoidica of the breast associated with Crohn's disease: a case report.

BACKGROUND: Necrobiosis lipoidica located to the breast; without evidence of glucose intolerance, is extremely rare, and its association to Crohn's disease is not usual. CASE PRESENTATION: We report an interesting case of an association of necrobiosis lipoidica of the breast and Crohn's disease in a 54-year-old Moroccan woman. Skin necrobiotic changes are a characteristic feature in necrobiosis lipoidica, but they are exceptional in metastatic Crohn's disease, since there are only three published cases of necrobiotic skin lesions on the lower leg resembling erythema nodosum in metastatic Crohn's disease. CONCLUSIONS: On the basis of this rare observation, necrobiosis lipoidica without evidence of glucose intolerance should be recognized as a possible cutaneous manifestation or association of Crohn's disease.