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Results for “NECROBIOSIS LIPOIDICA”

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PubMed1992

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.

Administration, Oral↗

PubMedDate not supplied

[Necrobiosis lipoidica over appendectomy scar in a patient with morphea of the breast].

Morphea is a sclerosing skin disease that may be clinically confused with a carcinoma when it involves the breast. It has been described in association with earlier radiotherapy and with silicone breast implants. We describe the case of a woman with morphea of the breast who developed necrobiosis lipoidica over the scar of an appendectomy performed 38 years earlier. Necrobiosis lipoidica over surgical scars is infrequently found in the literature. We review the published cases and discuss the association of necrobiosis lipoidica with morphea.

Appendectomy↗

PubMed2003

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.

Aged↗

PubMed2004

Healing of severe ulcerative necrobiosis lipoidica with cyclosporin.

A 55-year-old woman with severe, chronic, treatment-resistant ulcerating necrobiosis lipoidica of the shins was commenced on oral cyclosporin at a dose of 2.5 mg/kg/day. Improvement started within 1 week of commencing treatment, and the lesions healed completely over 8 months. Further ulceration occurred 3 months after discontinuing cyclosporin, with improvement on reintroduction of cyclosporin. There have been four previous case reports of healing of treatment-resistant ulcerating necrobiosis lipoidica with cyclosporin.

Cyclosporine↗

PubMed1998

Necrobiosis lipoidica: treatment by hyperbaric oxygen and local corticosteroids.

Necrobiosis lipoidica (NL) is closely associated with diabetes mellitus. Two-thirds to three-fourths of patients with NL have diabetes, although NL occurs in only 0.3% of diabetic patients. Typical lesions are found on pretibial skin, usually in young female diabetic patients whose disease is inadequately controlled. The cause of this dermopathy remains unknown. Multiple treatments have been described but have not led to consistent results. We report the case of a 28-year-old insulin-dependent diabetic woman with a disease duration of 23 years who spontaneously developed ulcerated NL on pretibial skin. NL progressively improved during 113 sessions of hyperbaric oxygen therapy and local corticosteroids.

Administration, Topical↗

PubMed1986

Necrobiosis lipoidica: clinical and immunofluorescent study.

Fourteen patients with necrobiosis lipoidica (NL) were carefully evaluated clinically and histologically. Ten of these patients (71%) had diabetes and most of them were treated with insulin (8 of 10 patients). Trauma was found as an immediate triggering factor in 6 patients (43%) and a possible preceding factor in 3 more cases. No immunoglobulins were found in the lesional skin of NL. Intradermal injections of histamine in healthy skin of legs led to deposition of immunoglobulins and complement (C3) in skin blood vessel walls in 4 of 12 patients studied (30%). These results suggest that immune complexes might be involved only in few cases of NL. Preceding trauma combined with metabolic and inflammatory changes could be an important triggering factor in the etiology of NL.

Adolescent↗

PubMed2006

UVA1 phototherapy for treatment of necrobiosis lipoidica.

The primary cause of collagen degeneration in necrobiosis lipoidica (NL) is proposed to be immunologically mediated vascular disease. Ultraviolet (UV)A1 has been used successfully to treat scleroderma in which both vascular damage and collagen dysregulation also occur. We treated six patients with NL [(five women; mean age of 32 years (range 22-70) and mean disease duration of 2.9 years (range 6 months to 5 years)] with a high-output ultraviolet (UV)A1 2-kW filtered metal halide source (Dr Hönle; Dermalight ultrA 1) having an emission spectrum of 340-440 nm. All patients had NL on the shins, which had been unresponsive to potent topical corticosteroid therapy (n = 6) and had responded minimally or not at all to TL-01 UVB (n = 2), topical psoralen plus UVA (PUVA) soaking (n = 2) or oral PUVA (n = 1) therapy. Patients received a variable number of total exposures (15-51), given 3-5 times weekly. NL resolved completely in one patient; this patient had minimal improvement after the first course of 16 exposures, but after a further 13 exposures, resolution occurred 6 months later. Two subjects obtained moderate improvement in their overall disease severity after 15 and 24 exposures, while two had only minimal improvement after 15 and 51 exposures. The remaining patient had no improvement after 16 treatments. Patients with the shortest disease duration had the greatest response. UVA1 therapy may be of benefit for the treatment of NL as an adjuvant therapy to topical corticosteroids or as a second-line alternative to other phototherapies, and may have a superior outcome in a proportion of patients.

Adult↗

PubMed2005

Fumaric acid esters in necrobiosis lipoidica: results of a prospective noncontrolled study.

BACKGROUND: Necrobiosis lipoidica (NL) is an uncommon granulomatous skin disease with association to diabetes mellitus. To date, no proven effective therapy for NL has been implemented. The standard treatment is topical application of corticosteroids, but numerous agents have been reported for NL, with varying degrees of success. In recent case reports, fumaric acid esters (FAE) have been reported to be effective in granulomatous skin diseases such as granuloma annulare, cutaneous sarcoidosis and NL. OBJECTIVES: We sought to investigate the efficacy of FAE in a larger number of patients with NL. METHODS: Eighteen patients with histopathologically proven NL were consecutively recruited into a prospective noncontrolled study. Dosage of FAE was given according to the standard therapy regimen for psoriasis. FAE were administered for at least 6 months. The treatment outcome was evaluated by means of clinical and histological scoring and 20-MHz ultrasound assessments. RESULTS: Three patients discontinued therapy with FAE, while the remaining 15 patients finished the study. After a mean +/- SD treatment period of 7.7 +/- 2.9 months, a significant (P < 0.001) decrease in the mean +/- SD clinical score, from 7.4 +/- 1.8 at the beginning to 2.5 +/- 1.3 at the end of therapy, was observed. Significant clinical improvement of NL was accompanied by significant (P = 0.019) increase of dermal density as assessed by means of 20-MHz ultrasound, and significant (P = 0.011) reduction of the histological score. Adverse effects were moderate and consisted mainly of gastrointestinal complaints and flushing. During follow-up of at least 6 months, clinical outcome remained stable in all patients. CONCLUSIONS: The results of this study demonstrate that FAE are beneficial and safe in the treatment of patients with NL.

Adult↗

PubMed1989

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.

Adult↗

PubMed1983

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.

Adolescent↗

PubMed1988

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.

Adult↗

PubMed1987

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.

Biopsy↗

PubMed1999

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.

Adult↗

PubMed1982

Necrobiosis lipoidica and granuloma annulare. Simultaneous occurrence in a patient.

Necrobiosis lipoidica (NL) and granuloma annulare (GA) occurred simultaneously in a patient with diabetes. Staining with colloidal iron showed deposition of acid mucopolysaccharides in the GA lesions and the absence of acid mucopolysaccharides in the NL lesions. If the two diseases have a common cause, that being the necrobiotic process, then one would expect to encounter their simultaneous occurrence much more frequently.

Adult↗

PubMed2005

Light-chain-restricted plasmacellular infiltrates in necrobiosis lipoidica -- a clue to an underlying monoclonal gammopathy.

BACKGROUND: Necrobiosis lipoidica (NL) is a member of the palisading granulomatous dermatitides that is associated, in most cases, with diabetes mellitus. However, there are an increasing number of cases of NL associated with other forms of systemic disease. We describe a novel case of NL associated with a light-chain-restricted plasmacellular infiltrate; subsequent investigations established an underlying monoclonal gammopathy. METHODS: Skin biopsy material was obtained and was processed in the usual fashion for hematoxylin and eosin (H and E) examination. Immunohistochemical staining was performed by utilizing kappa and lambda monoclonal antibodies (Dako Corporation, Carpentiera, CA, USA). Kappa and lambda in situ hybridization was also performed (Ventana Medical Systems, Tucson, AZ, USA). RESULTS: A 55-year-old woman with a 5-year history of bilateral thigh subcutaneous nodules underwent a skin biopsy, showing typical changes of NL; there was a concomitant prominent perivascular plasmacellular infiltrate. Kappa light chain restriction was observed amid the plasmacellular infiltrate. Bone marrow biopsy and immunophenotyping studies revealed a clonal plasmacytosis with kappa light chain restriction. CONCLUSIONS: Granulomatous inflammation, including NL, may be a cutaneous paraneoplastic expression of low-grade B-cell lymphoproliferative disease in the context of an underlying plasma cell dyscrasia.

Female↗

PubMed1995

Increased natural autoantibody activity to cytoskeleton proteins in sera from patients with necrobiosis lipoidica, with or without insulin-dependent diabetes mellitus.

Necrobiosis lipoidica (NL), a skin disease, is associated with insulin-dependent diabetes mellitus (IDDM). Natural autoantibody (NAb) activity in sera from 16 patients suffering from NL, with or without IDDM, was compared to that in sera from 41 patients with IDDM and 43 healthy controls. Isotype-specific enzyme-linked immunosorbent assays (ELISAs) were used to detect NAbs against actin, myosin, keratin, desmin, troponin, tropomyosin, thyroglobulin, insulin, single-stranded DNA and the hapten trinitrophenyl. NAb activity was significantly higher in sera from patients with NL (either with or without IDDM), compared with that detected in sera from patients with IDDM which was similar to that of healthy individuals. High proportion of NL sera exhibited increased IgG anti-tropomyosin (69%), anti-troponin, anti-desmin and anti-keratin (50% each), anti-insulin (44%) and anti-trinitrophenyl (31%) activities, as well as increased IgA and IgM anti-keratin activities (26% and 31%, respectively). The great majority (88%) of positive sera were polyreactive and contained NAbs, polyspecific and monospecific (as demonstrated by immunoadsorption studies), belonging to more than one isotype; there was no predominant serological reactivity pattern. In conclusion, increased NAb activity to cytoskeleton proteins is associated with the dermatological disease NL and not to the overlapping autoimmune disease (IDDM). The origin and significance of these NAbs is discussed.

Adult↗

PubMedDate not supplied

Maturity-onset diabetes of the young with necrobiosis lipoidica and granuloma annulare.

We describe a 12-year-old white girl with granuloma annulare localized to both ankles since she was five, necrobiosis lipoidica in the left pretibial region since she was ten, and a recent history of weakness, migraine, and weight loss. After initial evaluation, high fasting blood glucose levels and high hemoglobin A1c were found. The family history for non-insulin-dependent diabetes was suggestive of maturity-onset diabetes of the young. Coexistence of necrobiosis lipoidica and granuloma annulare, together with a family history of non-insulin-dependent diabetes, the age of onset, and the absence of ketosis, are specific features making possible, a clinical diagnosis. Genetic confirmation may not be so easily accessible or necessary.

Child↗

PubMed1975

Granuloma annulare and necrobiosis lipoidica treated by jet injector.

A Porto-jet injector was employed to treat forty-five cases of granuloma annulare and five cases of necrobiosis lipoidica with triamcinolone acetonide or sterile normal saline. Complete clearance of granuloma annulare lesions was achieved in nearly 70% of those receiving triamcinolone and in 44% with saline. Inadequate penetration of lesions was responsible for a number of failures. The rate of recurrence after treatment was high but retreatment was usually successful. In three cases of necrobiosis lipoidica complete resolution occurred, while in one, partial improvement was obtained. No serious complications of this type of treatment were observed.

Adolescent↗