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PubMed1975

Actinic granuloma. An annular connective tissue disorder affecting sun- and heat-damaged (elastotic) skin.

Ring-shaped inflammatory lesions sometimes develop in the abnormal "elastotic" connective tissues of skin damaged by sun and heat. The lesions, which commence as papules and nodules, enlarge very slowly and may persist for years. Microscopical sections show that there is an infiltrate composed mainly of foreign-body giant cells, the cells being engaged in digesting and absorbing the abnormal elastotic fibers. The disorder, which occurs on several continents, should probably be regarded as a phenomenon of repair within damaged connective tissue. The name actinic granuloma indicates its external or environmental origin and distinguishes it from other granulomas with which it is constantly being confused. Actinic granuloma and granuloma annulare appear to be related. In granuloma annulare, a productive and resorptive process also occurs, but its nature remains obscure. Actinic granuloma may be misdiagnosed as "atypical necrobiosis lipoidica" or as a sarcoidosis. The subject of actinic damage to blood vessels deserves study.

Atrophy↗
PubMed1985

Eosinophils in the cellular infiltrate of granuloma annulare.

Eosinophils have been described in the infiltrates of granuloma annulare, but their frequency, distribution and extent are not well documented. We found eosinophils in 18/45 (40%) cases of granuloma annulare, without significant variation relating to histologic sub-pattern. Eosinophils were seen in over half the cases of deep granuloma annulare and in over one-third of the cases of superficial granuloma annulare. This study demonstrates the lack of specificity of eosinophils in differentiating superficial granuloma annulare from deep granuloma annulare, granuloma annulare from necrobiosis lipoidica, and granuloma annulare from occasional clinical simulants which histologically show eosinophils, such as arthropod bite reactions.

Diagnosis, Differential↗
PubMed1987

Sarcoidosis with extensive cutaneous ulceration. Unusual clinical presentation.

A 70-year-old white woman with sarcoidosis and insulin-resistant diabetes mellitus presented with extensive cutaneous ulcerations. Both the cutaneous lesions and the systemic features of sarcoidosis showed a dramatic improvement during oral corticosteroid therapy. When extensive cutaneous ulcerations are present, it is important to consider sarcoidosis, as these may be the only presenting sign of the disease. Unlike ulcerated necrobiosis lipoidica diabeticorum, sarcoidal ulcerations may respond well to treatment with oral corticosteroids.

Aged↗
PubMed1995

Subcutaneous granuloma annulare of the eyelid. A case report.

A rare case of subcutaneous granuloma annulare of the eyelid is reported. A diabetic patient developed a unilateral periorbital tumorous mass. Biopsy revealed subcutaneous palisading granuloma. By microscopy and immunohistology, other granulomatous reactions including necrobiosis lipoidica could be ruled out. Since treatment with dapsone was without effect, the tumor was surgically excised. The differential diagnosis and the literature are briefly discussed.

Adult↗
PubMed1995

[Skin symptoms in diabetic metabolism].

Diabetes mellitus is a common disease, and the cutaneous manifestations of it are frequently encountered. Some skin diseases have a strong association with diabetes, as necrobiosis lipoidica and diabetic bullae. They can be regarded as a cutaneous marker for diabetes. Other disorders--for example shin spots--also occur in the nondiabetic population. Cutaneous infections may be a sign of poorly controlled diabetes.

Acanthosis Nigricans↗
PubMed1996

Necrobiotic xanthogranuloma with paraproteinemia.

Necrobiotic xanthogranuloma (NXG) is a rare skin disease mimicking necrobiosis lipoidica diabeticorum clinically and histologically. However, its association with paraproteinemia, and the evolution of hematologic malignancies in some cases, established NXG as a separate clinicopathologic entity. A typical case with indolent course is described.

Aged↗
PubMed1981

Cutaneous manifestations of systemic disease.

The skin should not be thought of as an isolated organ system. When the skin is involved by disease, other organs may be affected as well. Cutaneous xanthomas commonly occur with plasma lipid abnormalities, necrobiosis lipoidica diabeticorum with diabetes mellitus, and the porphyrias with altered heme synthesis. Erythema multiforme and erythema nodosum may be considered hypersensitivity reactions, occurring in response to a wide range of inciting agents, including infections, drugs, neoplasms, and rarely, collagen vascular diseases.

Adult↗
PubMed1975

[Differential diagnosis of granulomatous skin diseases].

The case histories of two female patients with granulomatosis disciformis and necrobiosis lipoidica are reported. Widespread skin manifestations, also in face and neck, with peculiar anular lesions complicated the differential diagnostic discrimination from sarcoidosis. The aspects of the cases and their progress are discussed in regard to the necessity of permanent checking of diagnostic preconditions during therapy.

Aged↗
PubMed1989

Healing of necrobiotic ulcers with antiplatelet therapy. Correlation with plasma thromboxane levels.

Necrobiosis lipoidica in diabetics has been considered to be a cutaneous manifestation of diabetic microangiopathy. Seven diabetic patients with necrobiotic ulcers of recent onset that healed after administration of acetylsalicylic acid and dipyridamole had elevated thromboxane levels. Healing was associated with depression of the elevated thromboxane levels in all seven patients.

Adult↗
PubMed1985

T-zone histiocytes in granulomatous skin diseases.

We investigated the distribution of T-zone histiocytes by immunohistochemical demonstration of S-100 protein in granulomatous skin diseases (granuloma annulare, 8 cases; necrobiosis lipoidica, rheumatoid nodule, sarcoidosis, lupus vulgaris, and foreign-body granuloma; 5 cases each). T-zone histiocytes were regularly found in the lymphohistiocytic mantle, but also occasionally between epitheloid cells. Our results show that, besides the monocyte-macrophage system, T-zone histiocytes consistently contribute to the formation of cutaneous granulomas. These findings may indicate a role of delayed-type hypersensitivity.

Foreign-Body Reaction↗
PubMedDate not supplied

Becaplermin and necrobiosis lipoidicum diabeticorum: results of a case control pilot study.

Necrobiosis lipoidica diabeticorum (NLD) is a rare skin condition associated with diabetes, which characteristically occurs in the pretibial region of the lower limbs (Boulton et al., 1988). The lesions generally appear as well-circumscribed reddish plaques, which are most often asymptomatic, resulting primarily in cosmetic disability. Currently, there is no reliable form of treatment for NLD, although many regimens have been tried (Shall et al., 1990)

Administration, Topical↗
PubMed1976

Cutaneous signs of diabetes mellitus.

Cutaneous signs of diabetes mellitus are extremely valuable to both the clinician and the researcher. Lesions such as necrobiosis lipoidica diabeticorum, diabetic dermopathy, carotenemia idiopathic bullous eruptions of granuloma anulare alert the physician to consider the diagnosis of diabetes mellitus. Xanthomas reflect the status of glucose and lipid metabolism. Diabetic dermopathy might be a useful monitor of angiopathic changes in the heart, kidney and brain. Finally, the accessibility of the skin makes it an ideal organ for studying the pathogenesis of diabetes and the effect of medications on the vascular and neurological complications of diabetes.

Adolescent↗
PubMed2002

Necrobiotic xanthogranuloma with lambda paraproteinemia: case report of successful treatment with melphalan and prednisone.

BACKGROUND: Necrobiotic xanthogranuloma (NXG) is a rare non-X histiocytosis with conspicuous lesions on the periorbital skin. METHODS: A diabetic patient presented with NXG and a previous diagnosis of necrobiosis lipoidica on the legs over a period of almost 2 years before the development of the typical lesions of NXG on the periorbital regions, back, thighs and legs. The patient was found also to have developed lambda paraproteinemia. RESULTS: Treatment with melphalan and prednisone resulted in great improvement of cutaneous lesions and paraproteinemia remission. CONCLUSION: This case report details how melphalan and prednisone can be administered in the successful treatment of necrobiotic xanthogranuloma with lambda paraproteinemia.

Adult↗
PubMed1987

Immunohistochemical studies of major histocompatibility antigens in a case of the bare lymphocyte syndrome without immunodeficiency.

A case of the bare lymphocyte syndrome without apparent immunodeficiency was observed. The patient had, in addition, ichthyosis vulgaris and sinobronchial disease. A pustular lesion developed at first on the anterior aspect of lower part of the left leg. This lesion gradually increased in size and evolved into giant indurated, irregular adjoining plaques. On biopsy, the histologic findings were similar to necrobiosis lipoidica. No human lymphocyte antigen (HLA) class I antigens were detected on peripheral mononuclear cells; however, HLA-DR antigens were present on B lymphocytes. Immunohistochemical studies disclosed defective expression of class I antigens in the non-lesional skin, but positive expression was demonstrated in the lesional area. HLA-DR antigens were expressed on keratinocytes and on most infiltrating inflammatory cells in the affected skin. It is therefore speculated that class I antigen appearance and mononuclear cell infiltrate each induces the other and that together they play an important role in the formation and enlargement of the skin lesion.

Adult↗
PubMed2003

The management of hard-to-heal necrobiosis with PROMOGRAN.

Nearly one third of patients with diabetes mellitus have some sort of dermatologic complication. Necrobiosis lipoidica diabeticorum (NLD) is a degenerative disease of the collagen in the dermis occurring in 0.3-0.7% of the diabetic population. There is no standard therapy for patients with NLD. The lesions associated with the condition appear as well circumscribed, erythematous plaques, with a depressed, waxy telangiectatic centre. One third of these lesions may progress to ulcers if exposed to any trauma. Management of these compromised wounds is often difficult, as NLD often goes through stages of activity and inactivity. This case study clearly illustrates some of the problems associated with wound management in a 34-year-old woman with an unhealed ulcer of 3 years' duration. Treatment with a new protease modulating matrix resulted in complete healing of the ulcer after 8 weeks, where other dressing regimens had failed to affect healing over a period of 2.5 years.

Adult↗
PubMed2005

Therapy of noninfectious granulomatous skin diseases with fumaric acid esters.

BACKGROUND: Noninfectious granulomatous skin diseases are inflammatory disorders of unknown aetiology which are often recalcitrant to common anti-inflammatory treatment regimens. Recently, in several case reports, fumaric acid esters (FAE) have proved beneficial in granulomatous skin diseases, but studies on a larger collection of consecutive patients have not yet been performed. OBJECTIVES: To investigate the therapeutic efficacy of FAE for the treatment of granulomatous skin diseases. PATIENTS AND METHODS: The therapeutic efficacy and side-effects of FAE were analysed retrospectively in 32 patients with disseminated granuloma annulare (n = 13), annular elastolytic giant cell granuloma (n = 3), sarcoidosis (n = 11), necrobiosis lipoidica (n = 4), or granulomatous cheilitis (n = 1). RESULTS: Three patients discontinued treatment within 4 weeks because of side-effects. Of the remaining 29 patients, 18 patients responded to treatment with FAE. Marked improvement or complete clearance was seen in seven patients. We observed a slight to moderate improvement in 11 patients, and 11 patients did not respond. In patients showing a complete remission, the maximum effect was observed after 8.5 months (SD +/-6 months, range 3-20 months). In two patients with systemic sarcoidosis, the pulmonary changes improved in parallel with the skin. Side-effects were usually mild and resolved spontaneously upon dose reduction or discontinuation of the therapy. CONCLUSIONS: The data presented here indicate that FAE may be considered for the treatment of recalcitrant granulomatous skin disease.

Adolescent↗
PubMed1999

Enhanced expression of human metalloelastase (MMP-12) in cutaneous granulomas and macrophage migration.

Accumulation of inflammatory cells such as macrophages may lead to degeneration of connective tissue matrix in various skin diseases. Macrophage metalloelastase, is a matrix metalloproteinase (MMP-12) capable of degrading elastin as well as various basement membrane components. To investigate the role of human macrophage metalloelastase in skin, we assessed by in situ hybridization and immunohistochemistry 66 specimens representing skin diseases characterized either by changes in elastic fibers or by pronounced infiltrations of extravasating and migrating macrophages. CD68 immunostaining was performed to identify the human macrophage metalloelastase-positive cells and Weigert's Resorcin-Fuchsin staining to reveal the status of elastic fibers. We found abundant expression of human macrophage metalloelastase mRNA in macrophages in areas devoid of normal elastic fibers in granulomatous skin diseases sarcoidosis, necrobiosis lipoidica diabeticorum, and granuloma annulare. Positive cells for human macrophage metalloelastase protein could be detected in the same regions as well as positive immunostaining for urokinase plasminogen activator. Of the other matrix metalloproteinases capable of degrading elastin, 92 kDa gelatinase colocalized with human macrophage metalloelastase, while 72 kDa gelatinase was produced by surrounding fibroblast-like cells. Furthermore, human macrophage metalloelastase was expressed by macrophages in areas with disrupted basement membrane, as assessed by type IV collagen staining, in pityriasis lichenoides and dermatitis herpetiformis. Specimens of anetoderma, acrodermatitis chronica atrophicans and pseudoxanthoma elasticum showed no signal for human macrophage metalloelastase. Matrilysin was not detected in any of the samples investigated. Our study suggests that human macrophage metalloelastase may contribute to elastin degradation occurring in granulomatous skin diseases and may aid macrophage migration through the epidermal and vascular basement membranes in inflammatory disorders.

Cell Movement↗