Perforating necrobiosis lipoidica in a girl with type 1 diabetes mellitus: a new case reported

Necrobiosis lipoidica is an idiopathic dermatological condition that is strongly associated with diabetes mellitus. It is more commonly seen in women than men. The average age of onset is 30-40 years. Necrobiosis Lipoidica diabeticorum is an extremely rare finding in childhood diabetes. We describe the case of a 13-year-old girl who has had type 1 diabetes mellitus since she was 8 years old. The patient presented with 2 well-defined, persistent plaques with a depressed central area and elevated purple peripheral ring, one on the right thigh and the other over the lateral left leg. Histopathologic evaluation of the patient's biopsy confirmed the diagnosis of necrobiosis lipoidica with transfollicular elimination. Our patient is the second pediatric case described with perforating necrobiosis lipoidica. We review the literature and discuss clinical features, several complications, and the most recent treatment options for necrobiosis lipoidica in diabetic children.     

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.     

Expression of the human erythrocyte glucose transporter Glut-1 in areas of sclerotic collagen in necrobiosis lipoidica

BACKGROUND: Glut-1 is the human erythrocyte glucose transporter which mediates facilitative transport of glucose across epithelial and endothelial barrier tissues. A primary abnormality in glucose transport and Glut-1 cell-surface content has been observed in fibroblasts from NIDDM and obese individuals. With the strong correlation between necrobiosis lipoidica and diabetes mellitus, we investigated the expression of Glut-1 in diabetic individuals with necrobiosis lipoidica (NL). METHODS: A polyclonal anti-Glut-1 antibody was used with a standard immunoperoxidase technique to determine Glut-1 expression by fibroblasts in areas of sclerotic collagen from specimens taken from diabetic individuals with necrobiosis lipoidica,and non-diabetic individuals with scars and granuloma annulare (GA). RESULTS: Our results showed Glut-1 expression in the areas of sclerotic collagen in patients with NL, possibly contributing to insulin resistance in these tissues. CONCLUSIONS: Our findings raises the question as to whether abnormalities in glucose transport by fibroblasts in individuals with necrobiosis lipoidica contribute to the histopathologic changes.     

Focal endothelial cell degeneration and proliferative endarteritis in trauma-induced early lesions of necrobiosis lipoidica diabeticorum

Microangiopathy is an essential component in diabetic vascular pathology. We report ultrastructural observations of ballooning degeneration involving isolated endothelial cells of cutaneous capillaries, while leaving adjacent endothelial cells relatively intact in six diabetic patients with early lesions of necrobiosis lipoidica induced by trauma. Focal proliferation of endothelial cells encroaching upon the vascular lumina (obliterative endarteritis) was also observed. Lectin studies on biopsy specimens of older lesions of necrobiosis lipoidica revealed paucity of dermal blood vessels. These observations enable us to gain further insight into the pathophysiological mechanisms that underlie diabetic microvascular disease.     

Necrobiosis lipoidica of the glans penis

We report a 65-year-old diabetic man with necrobiosis lipoidica occurring on the glans of penis. He was initially seen with chronic ulcerative balanitis that eventually healed with strict control of diabetes mellitus, cystostomy, and pentoxifylline, leaving heavily depressed scars. Penile necrobiotic palisading granulomas include necrobiosis lipoidica and granuloma annulare. On the basis of the literature review, these 2 diseases manifest different skin lesions.     

Squamous cell carcinoma in an area of necrobiosis lipoidica diabeticorum: a case report

Ulceration in an area of necrobiosis lipoidica diabeticorum is a frequent complication but malignant transformation is rare. One such case is reported in a diabetic patient and the literature is reviewed.     

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.     

Necrobiosis lipoidica diabeticorum: platelet survival and response to platelet inhibitors

Results of an open trial of platelet inhibitor treatment for necrobiosis lipoidica diabeticorum suggest the possible importance of abnormal platelet function in this disease. In ten female patients with necrobiosis lipoidica diabeticorum (six who were diabetic and four who were not) platelet survival times were measured before and after treatment with aspirin and dipyridamole. Pretreatment platelet survival time was considerably shortened in 50 percent of the diabetic and nondiabetic patients. Platelet-inhibitor treatment prolonged platelet survival time toward normal in most of these patients. The clinical response to treatment varied from healing to no noticeable effect.     

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.     

Psoriasis, necrobiosis lipoidica, granuloma annulare, vitiligo and skin infections in the same diabetic patient

A diabetic patient is described presenting psoriasis, necrobiosis lipoidica diabeticorum, granuloma annulare, and vitiligo and with a history of recurrent erysipelas and mycotic infections. Scrupulous physical examination excluded further systemic or cutaneous involvement. The immunological workup revealed both phenotypic and functional defects in cellular immunity.     

Systemic corticosteroids for the outpatient treatment of necrobiosis lipoidica in a diabetic patient

Necrobiosis lipoidica (NL) remains a major therapeutic challenge for the dermatologist and diabetologist. Amongst many treatment modalities attempted, systemic corticosteroids remain controversial, not least because a proportion of NL patients are diabetic, and corticosteroids are likely to destabilize their diabetic control. Close supervision as an inpatient is often required. We report the ambulatory outpatient management of an insulin-dependent diabetic with ulcerated necrobiosis lipoidica complicated by activated protein C resistance.     

Necrobiosis lipoidica: treatment with systemic corticosteroids

In an attempt to find an effective therapy for necrobiosis lipoidica, we have treated six patients with this disease with a 5-week course of systemic corticosteroids. This treatment resulted in complete cessation of disease activity in all patients and no recurrence in a mean follow-up period of 7 months; however, restitution of atrophic skin lesions could not be achieved. The therapy was well tolerated and did not pose problems, even in diabetic patients. These results strongly suggest that short-course therapy of necrobiosis lipoidica with corticosteroids is of lasting benefit to these patients and should probably be considered early in the course of their skin disease.     

Necrobiosis lipoidica. Variants on a theme

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 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.     

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.     

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.     

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.     

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.     

Coexistence of sarcoidosis and necrobiosis lipoidica of the legs—A report of two cases

Two patients are reported with systemic sarcoidosis and necrobiosis lipoidica on the legs. There are no previous reports of this association. In view of the relative rarity of these two diseases, there is a clear suggestion of a pathogenetic link between sarcoidosis and necrobiosis lipoidica. Therefore, in patients with necrobiosis lipoidica the possibility of systemic sarcoidosis should be borne in mind and appropriate investigations should always be carried out.     

Ulceration in necrobiosis lipoidica—a case report and study

A case of aggressive ulceration of necrobiosis lipoidica was successfully treated with oral prednisolone. A retrospective study of 23 cases of necrobiosis lipoidica revealed a 13% incidence of ulceration. The pathogenesis, clinieal features and treatment of ulceration in necrohiosis lipoidica are discussed.