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PubMed1998

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.

Collagen↗
PubMed1977

Systemic sarcoidosis with necrobiosis lipoidica-like scalp lesions.

A 78-year-old woman is described, having systemic sarcoidosis for 15 years, involving the lungs, lymph nodes and the skin. Over the last 6 years she developed a progressive cicatricial alopecia with histologic changes of the granulomatous type of necrobiosis lipoidica surrounded by sarcoid granulomas.

Aged↗
PubMed1983

Differential staining pattern of lysozyme in palisading granulomas: an immunoperoxidase study.

Biopsies from palisading granulomas of granuloma annulare, necrobiosis lipoidica, and rheumatoid nodules were examined for the presence of lysozyme (muramidase). This enzyme was identified in paraffin-embedded tissues using a primary antibody to lysozyme and the peroxidase-antiperoxidase technic. Some inflammatory cells in the infiltrate of granuloma annulare stained abundantly for lysozyme, whereas those of necrobiosis lipoidica and rheumatoid nodule stained minimally and negatively, respectively. This pattern of staining may be of diagnostic value and suggests that the histiocytoid cells constituting the infiltrate of granuloma annulare are in some way different from the similar-appearing cells of necrobiosis lipoidica and rheumatoid nodule.

Granuloma↗
PubMed1996

Lipomembranous (membranocystic) fat necrosis. Clinicopathologic correlation of 38 cases.

Clinicopathologic correlation of cutaneous biopsy specimens demonstrating typical lipomembranous fat necrosis was performed. Material from 732 biopsies of various subcutaneous inflammatory disorders seen at our institution in the past 5 years was screened for typical lipomembranous (membranocystic) changes in the panniculus, and 39 specimens from 38 patients with these changes were identified. The most common clinical context in which this condition was observed was in chronic sclerotic plaques of the lower legs associated with venous insufficiency (37% of the total cases). All patients were women, and the majority were obese. Typical lipomembranous fat necrosis was also observed in eight cases (21%) of erythema nodosum, three (8%) of morphea or subcutaneous morphea (or both), two (5%) of lupus panniculitis, two (5%) of necrobiosis lipoidica, and in single cases of polyarteritis nodosa, necrotizing vasculitis, and erysipelas. Six cases (16%) had no definite underlying disease. The mean age of all patients was 57 years (range 32-86 years), and 34 patients (89%) were women. Of the five major categories identified, lipomembranes lining macrocysts and microcysts were most prominent in the venous insufficiency- and morphea-related cases and were much less prominent in erythema nodosum, lupus panniculitis, and necrobiosis lipoidica, which generally showed histopathologic findings typical of these disorders. In addition to lining the macrocystic and microcystic cavities formed in the fat lobules, lipomembranes were prominent in areas of septal fibrosis in all cases associated with morphea and necrobiosis lipoidica and in 35% and 25% of venous insufficiency- and erythema nodosum-related cases, respectively. In lupus panniculitis, lipomembranes were most prominent in areas of hyaline necrosis. We conclude that lipomembranous fat necrosis is most likely a nonspecific form of ischemic fat degeneration that may be induced by various clinical entities. This change is most often seen in venous insufficiency-associated chronic sclerotic plaques typically observed in middle-aged obese women, and we propose the term stasis-associated lipomembranous panniculitis (SALP) to describe this most common form of lipomembranous fat necrosis.

Adipose Tissue↗
PubMed1985

[Ultrastructural study of a case of Miescher-Leder granulomatosis disciformis chronica et progressiva].

Ultrastructural study of a case of granulomatosis disciformis occurring on the legs of a 62-year-old woman. The diagnosis is based upon the contrast between the clinical aspect of necrobiosis lipoidica and the histologic aspect of inflammatory granulomatous infiltrate of dermis without necrobiosis nor palisading. Electron microscopic findings are: perivascular mixed cell infiltrate and histiocytic granuloma with lysosomial and phagocytic activity; degenerative and necrotic cellular alterations; modifications of collagen fibres with two types of fibres: the thinnest are the most numerous and have a mean diameter of 50 nm, the thickest of 110 nm; no significant lesions of capillary walls. These findings suggest the following succession of pathological events: cellular necrosis, lysosomial and phagocytic activity, scarring. Some descriptions of necrobiosis lipoidica and granuloma annulare seem to be quite similar and our findings are in favour of the unity of these three illnesses.

Capillaries↗
PubMed1997

Necrobiotic xanthogranuloma with paraproteinemia: an evolving presentation.

Necrobiotic xanthogranuloma with paraproteinemia is a progressive and destructive process that is often confused both clinically and histologically with other granulomatous and xanthomatous entities. It was first described by Kossard and Winkelmann in 1980. Prior to this, the entity was reported under a variety of names such as atypical multicentric reticulohistiocytosis with paraproteinemia, atypical xanthoma disseminatum, and atypical necrobiosis lipoidica. A 69-year-old woman experienced slightly pruritic and painful papules, plaques, and nodules. Initial biopsy specimens showed a granulomatous process consistent with granuloma annulare. Later biopsy specimens demonstrated histologic changes indicative of necrobiosis lipoidica. The most recent histologic findings are those of necrobiotic xanthogranuloma, with results of laboratory studies revealing a coexistent IgG kappa paraproteinemia. Patients with necrobiotic xanthogranuloma who demonstrate a benign monoclonal proteinemia and are evaluated for several years show a 9 to 11 percent risk of myeloma, amyloidosis, or macroglobulinemia. Multiple treatment regimens have been attempted, none of which are curative. We propose that this entity may be part of an evolutionary process that may start as a granulomatous entity and culminate in a xanthogranulomatous process with an accompanying paraproteinemia.

Aged↗
PubMed1998

[Granulomatosis disciformis Miescher].

We describe granulomatosis disciformis Miescher in a 57-year old female patient with typical clinical manifestation: disc-shaped plaques with slight central sclerosis and lesions resembling necrobiosis lipoidica and granuloma anulare. Histology revealed an infiltrate mainly composed of macrophages and lymphocytes and small foci of necrobiotic collagen and of new collagen formation. We review the literature on granulomatosis disciformis and nosology, concluding--as do most authors--that granulomatosis disciformis is a characteristic variant within the spectrum of cutaneous noninfectious granulomatous disease lying in between generalized granuloma anulare and necrobiosis lipoidica.

Adult↗
PubMed1951

The topical and systemic use of cortisone in dermatology.

Part I of this report deals with the topical use of cortisone in a variety of skin diseases. Fifteen patients with chronic discoid lupus erythematosus, four patients with necrobiosis lipoidica diabeticorum, four with psoriasis, one with lichen planus and one with granuloma annulare were treated with cortisone ointment. All the patients with chronic discoid lupus erythematosus had some degree of improvement. In two patients with chronic lupus erythematosus, complete clearing of the eruption occurred. In four patients with necrobiosis lipoidica diabeticorum remarkable involution resulted. Patients with psoriasis, lichen planus and granuloma annulare were not benefited. Part II deals with the systemic use of cortisone. Eight patients with severe serum sickness-like penicillin reaction responded dramatically to parenterally administered cortisone. In two cases of pemphigus vulgaris and one case of Sulzberger-Garbe disease, the disease was kept in remission with cortisone administered intramuscularly as well as orally. Partial improvement resulted in a case of localized myxedema associated with malignant exophthalmus. Two patients with exfoliative dermatitis due to therapy with heavy metals responded dramatically to cortisone. No beneficial effects were noted in patients with chronic urticaria and atopic dermatitis.The systemic use of ACTH and cortisone in dermatology at present should be confined to patients with known fatal or hopelessly incapacitating diseases and to patients with extreme hypersensitivity reactions which may be protracted or life-endangering, and which can be controlled or cured with a relatively small total dosage of the agents in a short time.

Administration, Topical↗
PubMed1979

[The clinical picture of "circumscribed granuloma diseases"].

Characteristic findings, differential diagnosis and clinical course of the so-called "circumscript granulomas" (Granuloma anulare, necrobiosis lipoidica, Miescher's granulomatosis, disciformis, necrobiosis maculosa, granuloma multiforme, actinic granuloma, granuloma faciale and lethal midline granuloma etc.) are represented in a condensed clinical survey.

Diagnosis, Differential↗
PubMed1976

Lymphokines (MIF) in the serum of patients with sarcoidosis and cutaneous granuloma annulare.

Serum MIF activity was studied in ten patients with sarcoidosis, fourteen with granuloma annulare, four with necrobiosis lipoidica, and nine with various dermatological diseases. Positive MIF activity was found in the sera of nine of the ten patients with sarcoidosis and eleven of the fourteen patients with granuloma annulare. The delayed hypersensitivity tests were negative in all nine of the patients with sarcoidosis who had serum MIF activity and were positive in only three patients with cutaneous sarcoid lesions. One of four patients with necrobiosis lipoidica demonstrated minimal serum MIF activity. Data on serum lymphokine activity in sarcoidosis and granuloma annulare suggest that these two diseases are related to delayed hypersensitivity mechanisms.

Granuloma↗
PubMed2004

The histologic spectrum of cutaneous sarcoidosis: a study of twenty-eight cases.

BACKGROUND: Naked sarcoidal granulomas (NSGs) are the characteristic histologic finding in sarcoidosis. This descriptive study was designed to identify the frequency of other histologic changes in cutaneous sarcoidosis. METHODS: The slides from 28 sequential biopsies previously diagnosed as sarcoidosis in patients with known systemic sarcoidosis were reviewed. RESULTS: Classic NSGs were identified in 25 biopsies (89%). Four biopsies contained tuberculoid granulomas, two with neutrophils suggesting infection (cultures negative). Five biopsies contained interstitial granulomas that resembled granuloma annulare and necrobiosis lipoidica in one case each. Additional histologic findings included birefringent foreign material in 14 biopsies (50%), focal necrosis (43%), elastophagocytosis (39%), linear peri-neural granulomas resembling leprosy (25%), increased dermal mucin (18%) and lichenoid inflammation (14%) [two with plasma cells resembling syphilis (7%)]. In all but three cases, the clinical morphology of the lesions suggested sarcoidosis. Special stains for mycobacteria and fungi were negative. CONCLUSIONS: The histologic changes in cutaneous sarcoidosis are more diverse than previously recognized. In sarcoidosis, foreign material may be a frequent nidus for cutaneous granuloma formation. Histologic examination without the clinical history could lead to a misdiagnosis of leprosy, syphilis, other infectious granulomas, rosacea, granuloma annulare, necrobiosis lipoidica, and foreign body reaction in selected cases from this series.

Adult↗
PubMed1981

[Granulomatosis disciformis chronica et progressiva (Miescher-Leder)].

A case of granulomatosis disciformis chronica et progressiva in a 75-year-old diabetic is reported. The dermatosis which was successfully treated with a high-potent local steroid preparation, today is regarded as a granulomatous variety of necrobiosis lipoidica. The present case shows, as originally described by Miescher and Leder, that the clinical findings as well as the histopathology represent an entity which may differ markedly from the common type of necrobiosis lipodica.

Aged↗
PubMed1985

Superficial ulcerating rheumatoid necrobiosis: a perforating rheumatoid nodule.

We report a case of superficial ulcerating rheumatoid necrobiosis (SURN), which is a recently described cutaneous manifestation of severe rheumatoid arthritis. As with classic rheumatoid nodule, there is evidence that this lesion may result from a vasculitis. SURN appears to represent a form of "perforating" rheumatoid nodule, and completes a triad of major necrobiotic processes (granuloma annulare, necrobiosis lipoidica, rheumatoid nodule) that have been reported to show epidermal perforation.

Arthritis, Rheumatoid↗
PubMed1989

[Skin lesions in diabetes mellitus].

The characteristics of cutaneous manifestations in diabetes mellitus are presented. The clinical features of the skin lesions are described in relation to the severity of metabolic disorders. The clinical and biochemical features of Necrobiosis lipoidica, its classification and methods of treatment are detailed.

Adolescent↗
PubMed1986

Necrobiotic xanthogranuloma: a report of four cases.

Four patients are described who demonstrate the range of clinical and pathological features seen in necrobiotic xanthogranuloma with paraproteinaemia (NXG). Each patient had the typical periorbital yellow plaques with numerous well demarcated yellow indurated plaques on the trunk. All four patients had evidence of paraproteinaemia. The histopathology showed diagnostic features of intense necrobiosis with xanthomatization, including touton giant cells and the bizarre angulated giant cells of NXG. Previously, the patients had been diagnosed as atypical necrobiosis lipoidica, and the distinction between NXG and other necrobiotic conditions is discussed.

Aged↗