Serum alpha 2 globulin levels in granuloma annulare and necrobiosis lipoidica

Alpha 2-macroglobulin, caeruloplasmin and haptoglobin were measured in the sera of patients with necrobiosis lipoidica, granuloma annulare and diabetes. Alpha 2 Macroglobulin and caeruloplasmin were significantly raised in diabetes, and caeruloplasmin was raised in necrobiosis lipoidica without diabetes. The ratio of alpha 2-globulin to serum albumin was significantly high for all three proteins in diabetes, and for haptoglobin and caeruloplasmin in necrobiosis lipoidica. None of these proteins was abnormally raised in non-diabetic patients with granuloma annulare. There is good evidence that the plasma protein changes in diabetes contribute to the development of microangiopathy by their influence on blood viscosity. The altered plasma protein profile in necrobiosis lipoidica may therefore be of relevance to the development of the vascular lesions in this disorder.     

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.     

Skin blood flow in necrobiosis lipoidica during treatment with low-dose acetylsalicylic acid

Skin blood flow was measured by the laser Doppler technique in lesional and clinically normal skin of 10 diabetic patients with necrobiosis lipoidica during and after treatment with 40 mg acetylsalicylic acid (ASA) daily. The measurements showed that the blood flow during ASA treatment was significantly decreased in the central lesional skin without changes in the peripheral part of the lesions and normal skin. In view of these findings we suggest that low-dose ASA may not be the best treatment for necrobiosis lipoidica.     

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. An immunofluorescence study

Biopsy specimens from 12 patients with necrobiosis lipoidica (diabeticorum) were studied by direct immunofluorescent microscopy. The immunoglobulin IgM was present in blood vessel walls of involved skin from six patients, and the third component of complement (C3) was present in the blood vessel walls of involved skin from seven patients. The immunoglobulin IgA was similarly observed in two patients. In addition, IgM, C3, or fibrinogen were observed at the dermal-epidermal junction of involved skin from seven patients. Necrobiotic areas invariably contained fibrinogen. These findings suggest that an immunecomplex vasculitis may be involved in the pathogenesis of necrobiosis lipoidica.     

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.     

Sweat gland disturbances in granuloma annulare and necrobiosis lipoidica

We studied sweat gland distribution, density and activity in thirteen cases of granuloma annulare and ten cases of necrobiosis lipoidica, using a combination of the plastic impression and starch-iodine methods. The pattern of sweat gland disturbance in the two diseases was entirely different. In necrobiosis lipoidica an intense and uniform hypohidrosis was detected throughout the lesion, whereas in granuloma annulare the disturbance followed the morphology of the lesion (the papular border showed complete anhidrosis, whereas the flat central part of the lesion showed only moderate hypohidrosis or normal sweating). The method assigns numerical values to the 'relative density' and the 'relative activity' of the functioning sweat glands compared with normal skin, thus permitting statistical evaluation of the results.     

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.     

SQUAMOUS CARCINOMAS DEVELOPING IN BILATERAL LESIONS OF NECROBIOSIS LIPOIDICA

Squamous cell carcinomas developed in bilateral pretibial lesions of necrobiosis lipoidica in a 39 year old non-diabetic male. The tumours and areas of necrobiosis were successfully excised and repaired with full thickness skin grafts. Despite the chronic scarring and at times ulcerative nature of necrobiosis lipoidica, complicating squamous cell carcinomas have infrequently been reported.     

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.     

Necrobiosis-lipoidica-like skin manifestation in lymphomatoid granulomatosis (Liebow)

A 58-year-old man developed 3 indurative erythematous lesions like necrobiosis lipoidica on the right lower leg. He had had similar cutaneous lesions 1.5 years previously. These had been surgically excised, and a histologic diagnosis of necrobiosis lipoidica was made at another hospital. He was diagnosed as having lymphomatoid granulomatosis by lung biopsy in our hospital. Nasal involvement was confirmed in later examinations and the skin lesions were also considered to be the cutaneous manifestation of lymphomatoid granulomatosis. This outlines that cutaneous manifestations may allow early diagnosis of lymphomatoid granulomatosis.     

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 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-like Skin Lesions in Systemic Sarcoidosis

A 62-year-old woman with systemic sarcoidosis developed erythematous plaques on her lower legs. Clinically, two kinds of skin lesions were distinguished; one type formed brownish-red plaques with induration suggesting plaque-type skin sarcoid, and the other formed purplish erythematous plaques with atrophic centers resembling necrobiosis lipoidica. In spite of this clinical appearance, a biopsy specimen from one of the latter lesions revealed typical skin sarcoid histology composed of discrete non-caseating granulomas, while that from one of the other lesions showed necrobiotic changes of collagen bundles surrounded by epitheloid histiocytes and foreign-body giant cells. Because cutaneous involvement of sarcoidosis may mimic necrobiosis lipoidica clinically and/or histologically, we diagnosed her skin lesions as necrobiosis-like skin sarcoid.     

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.     

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: 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.     

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 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 arising in long-standing necrobiosis lipoidica

Necrobiosis lipoidica (NL) is a disease of collagen. Squamous cell carcinomas developing in areas of chronic ulceration and scarring have been well documented in a variety of skin diseases but rarely in areas of necrobiosis lipoidica. The case history of a 76-year-old female is presented, whose squamous cell carcinoma appeared 30 years after the diagnosis of necrobiosis lipoidica. The clinical and histopathological picture is described, stressing the importance of the unusual association of the two pathologies in the prognostic.