Primary Necrobiotic Xanthogranulomatous Sialadenitis with Submandibular Gland Localization without Skin Involvement

Necrobiotic xanthogranulomatous reaction is a multiorgan, non-Langerhans cell histiocytosis with an unknown etiology. Occurrence in the salivary gland is extremely rare. We recently identified a case of necrobiotic xanthogranulomatous sialadenitis in a 73-year-old Korean woman who presented with a painless palpable lesion in the chin. There was no accompanying cutaneous lesion. Partial resection and subsequent wide excision with neck dissection were performed. Pathological examination showed a severe inflammatory lesion that included foamy macrophages centrally admixed with neutrophils, eosinophils, lymphocytes, plasma cells, and scattered giant cells, as well as necrobiosis. During the 12-month postoperative period, no grossly remarkable change in size was noted. Necrobiotic xanthogranulomatous inflammation may be preceded by or combined with hematologic malignancy. Although rare, clinicians and radiologists should be aware that an adhesive necrobiotic xanthogranuloma in the salivary gland may present with a mass-like lesion. Further evaluation for hematologic disease and close follow-up are needed when a pathologic diagnosis is made.

Extensive papulonodular lesion in a child - granuloma annulare presenting as a diagnostic dilemma

Granuloma annulare is an idiopathic, palisaded, granulomatous disorder. There are four main clinical varieties of GA of which the subcutaneous type is less commonly encountered. Here, we report a case of generalized subcutaneous granuloma annulare in a 10-year-old boy. This variety is common in children but its generalized form is a rare presentation.

Necrobiosis Lipoidica Over Surgical Scar in a Patient without Diabetes Mellitus / 대한피부과학회지

Necrobiosis lipoidica (NL) is a chronic granulomatous skin disease typified by indurated plaques on the shin. Although this condition is strongly associated with diabetes mellitus, its etiology and pathogenesis remains unknown. Localization of NL to the lower extremities suggests that local injury may be a contributing factor to the disease. A healthy 38-year-old man presented with localized erythematous to yellowish plaques on his right thigh that developed over several years. The lesion developed in the scar where suture had been done for laceration repair 25 years ago. A biopsy specimen showed degenerated collagen with surrounding palisading granulomas. Numerous lymphocytes and multinucleated giant cells were infiltrated throughout the reticular dermis. Plasma cells aggregation was found at the dermal subcutaneous junction.

Necrobiosis Lipoidica Presenting as Unilateral Papulovesicular Eruptions on the Lower Leg / 대한피부과학회지

Necrobiosis lipoidica is a chronic granulomatous disease that has a well-known association with diabetes mellitus. It usually affects the bilateral lower legs of middle-aged women. Over time, the clinical features vary from red-brown papules to sharply demarcated yellow-brown plaque with atrophic center. Some of the lesions may become ulcerated, especially in diabetic patients. A 61-year-old Korean female with a history of diabetes, ischemic heart disease, and pancreatic cancer was referred to our clinic for evaluation of the painful papulovesicles on her left lower leg. Under the assumptive diagnosis of herpes zoster, we immediately started an antiviral agent. However, her skin lesions rapidly increased in size and in number, and progressed to the multiple ulcerations in 9 days. Histopathologic examination showed several poorly circumscribed necrobiotic granulomas in the deep dermis and subcutis levels of the skin. In addition, prominent vascular ectasia and vasculitis were observed. We diagnosed the patient as having necrobiotic lipoidica, and treated her with oral dapsone, colchicine, phentoxifylline and antiplatelets. Her leg ulcerations healed after four weeks of treatment.

A Case of Necrobiosis Lipoidica Treated with Cyclosporine / 대한피부과학회지

No abstract available.

[Fibroid and pregnancy: the complications]

To study the maternal and fetal morbidity in the association fibroid and pregnancy and the management in this case. A retrospective study of 80 cases of fibroids associated to pregnancy. These cases were taken from the department "C" of gynecology and obstetrics in the center of maternity and neonatology of Tunis. We studied 80 cases of fibroid associated to pregnancy in our study. The mean age of the patients was 32 years old. 45% of the patients were primipares. The interstitial fibroids [68%] are the most frequent. The average number of fibroid is 1, 7 in each pregnancy. The aseptic necrobiosis is the most frequent complication of the fibroid whereas for the mother the main complications are the premature delivery, the premature rupture of membranes and the placenta praevia during the third term of pregnancy. The dystocic presentations are more frequent than in the general population, responsible of a higher rate of caesarian sections. The delivery hemorrhage constitutes the most frequent complication of the post partum. The fetal prognosis is globally good with a morbidity dominated by growth restrictions but with no superior mortality rate. The myomectomy was practiced during the caesarian section in 3 cases, the abstention being the rule for the other patients. The association fibroid and pregnancy is not rare, the complications are frequent that is why it is considered as a high risk pregnancy. An early detection of the complications and a prevention of delivery hemorrhage would reduce the maternal and fetal morbidity

Necrobiotic Xanthogranuloma with Monoclonal Gammopathy / 대한피부과학회지

Necrobiotic xanthogranuloma (NXG) is a rare, progressive, histiocytic disease characterized by destructive cutaneous lesions, a close association with paraproteinemia, multiple myeloma, hypertension, and multiple extracutaneous manifestations. Multiple indurated yellow-red plaques or nodules are commonly present in this disorder. The pathogenesis of NXG is poorly understood. Histopathologically, NXG features with hyaline necrosis or necrobiosis are present. We describe a patient with typical cutaneous and histologic findings of NXG with an associated monoclonal gammopathy.

Cutaneous Sarcoidosis Clinically Mimicking Necrobiosis Lipoidica in a Patient with Systemic Sarcoidosis

A 70-year-old woman with an 8-year history of systemic sarcoidosis developed round, red-brown eruptions, with central atrophic lesions on her lower legs. The features of the biopsy specimen resembled those of necrobiosis lipoidica (NL), but although necrobiosis was present there were well-formed non-necrotizing granulomas in the dermis. The histological diagnosis was cutaneous sarcoidosis. Systemic sarcoidosis presenting with NL has rarely been reported. The histological features of cutaneous sarcoidosis sometimes mimic those of other granulomatous diseases, including NL and granuloma annulare, which are difficult to distinguish. We discuss the novel association between sarcoidosis and other granulomatous diseases.

Ulcerative Necrobiosis Lipoidica Improved by Hydroxychloroquine / 대한피부과학회지

Necrobiosis lipoidica (NL) is a chronic granulomatous skin disease of unknown etiology, which can be seen in patients with diabetes mellitus. Typical lesions of NL appear on the pretibial skin as painful yellow-brown inflammatory plaques with raised borders and atrophic centers. Ulceration occurs in approximately 35% of cases, leading to increased risk of secondary bacterial infection and scarring. Although there have been many proposed therapies for ulcerative NL, the response to treatment is often met with limited success. Here, we report a case of a 19-year-old girl with ulcerative NL, which showed improvements with oral hydroxychloroquine, an antimalarial agent.

Cutaneous Granulomas Associated with Common Variable Immunodeficiency / 대한피부과학회지

Common variable immunodeficiency (CVID) is one of the primary immunodeficiency disorders, which is characterized by hypogammaglobulinemia, defect in antibody synthesis and recurrent sinorespiratory bacterial infections. Granuloma is an uncommon skin manifestation in primary immunodeficiency disorders. Here we present a 5-year-old girl who had asymptomatic erythematous plaques on the face and legs. She had a past medical history of recurrent sinorespiratory and gastrointestical bacterial infections since 2 years old. The histopathologic findings of the skin lesions showed sarcoid-like granulomas with focal necrobiosis. No specific organism was found with evaluation. Cells forming granulomas were strongly positive for CD3 or CD68. CD4/CD8 ratio was estimated less than 1. Laboratory examination showed low hemoglobin, low B cell and normal T cell number, low IgA and IgG levels.

A Case of Palisaded Neutrophilic and Granulomatous Dermatitis / 대한피부과학회지

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an uncommon skin eruption associated with immune-mediated connective tissue diseases such as rheumatoid arthritis, lupus erythematosus, and Behcet's disease. This condition is also described as Winkelmann's granuloma, linear subcutaneous bands, rheumatoid papules, rheumatoid neutrophilic dermatitis, interstitial granulomatous dermatitis with cutaneous cords and arthritis, or Churg-Strauss disease. PNGD shows variable clinical manifestations and is characterized histopathologically by step pattern of early leukocytoclastic vasculititis, progressing to form palisaded granulomatous inflammation, and surrounding zones of necrobiosis in the later stages. We report a 66-year-old woman with multiple skin nodules, who had concomitant rheumatoid arthritis. Histopathologic findings revealed palisaded neutrophilic and granulomatous dermatitis.

Testicular cellular toxicity of cadmium : transmission electron microscopy examination.

It is clear that environmental heavy metals influence life systems and reproductive system. In the present study histological investigation revealed that cadmium was testicular toxicant in mice. Here we compared the fine-structure of spermatogenesis in two groups of mice (SWR), experimental and control. The experimental group underwent cadmium ingestion at 1 mg/kg daily for 4 weeks. The control group underwent ingestion of distilled water with equal dosages, using the same type of injectors, for 4-weeks. After cadmium exposure period both control and experimental groups were killed and samples of the testes were processed for microscopic examination. Ultra sections were examined and photographed by Transmission Electron Microscope (JEOL- 100SX) at 80KV. Ultrastructure examination revealed, vascular endothelial, interstitial, and sertoli cells damages. Early impairments of germinal cellular differentiation resulted in deformations in all parts of late spermatid. There were dislocation of accrosomal granules, nuclear damage associated with chromatin heterogeneity, detached spermatid from the apical process of sertoli cell, disarrangement of the mitochondria, abnormal oriented tail piece, and abnormal microtubules complex. These ultra morphological abnormalities relate to cell injury and to the resulting physiological abnormality, necrobiosis. Based on the results of this investigation it can be concluded that cadmium ingestion at 1000 microg/kg caused testicular toxicity and abnormalities in early sperm development.

Psychiatric disturbances in rheumatoid arthritis

To study the pattern of psychiatric syndromes that may accompany Rheumatoid arthritis [RA] and their relationship to disease activity, severity, pain and disability. Also, to find out the impact of stressful life events on the same parameters of the disease. The study comprised forty RA patients and 20 apparently normal controls. They were subjected to thorough medical history taking, clinical examination, laboratory investigations, disease activity, spread severity index, functional assessment with the Health Assessment Questionnaire Score, the Social Readjustment Rating Scale [SRRS] and identification of psychiatric disorders using the International Classification of Diseases [ICD,-10] symptom checklist for mental disorders. The prevalence of psychiatric disorders was significantly higher in RA patients than in the control group [p<0.05]. Depressive disorders were the most prevalent of these disorders. The Social Readjustment Rating Scale was significantly higher in RA patients than in the control group [p<0.001]. Also RA patients with psychiatric disorders were found to have higher SRRS than those not suffering from psychiatric disorders [p<0.05]. The stressful life events were found to have a strong impact on disease activity and pain in RA patients. Depression was the most common psychological disturbance associated with RA. This study highlights the role that the rheumatologist can play in the early detection of psychiatric disorders and psychiatric consultation in the proper time. In addition, it should be emphasized that the psychosocial effects of RA should always be put in mind and treated as a part of the illness

A Case of Necrobiosis Lipoidica with Prominent Cholesterol Cleft / 대한피부과학회지

We report an unusual case of necrobiosis lipoidica with prominent cholesterol clefts in a 36-year-old man who had developed multiple, 0.5~5cm in diameter, round to oval-shaped erythematous plaques on both shins for over 3 years. Laboratory findings showed the following; cholesterol, 250mg/dl; triglyceride, 208mg/dl; blood sugar level fasting/2-h postprandial, 101/92mg/dl; and other findings were within normal limits or negative. There was no paraproteinemia. The biopsy specimen showed various lymphohistiocytic granuloma with many giants cell in the mid and deep dermis, and septal fibrosis in the subcutaneous tissue. There were also extensive areas of necrobiosis with prominent cholesterol clefts mimicking necrobiotic xanthogranuloma.

A Case of Necrobiosis Lipoidica Diabeticorum with Koebner Phenomenon / 대한피부과학회지

The isomorphic response of Koebner is a well-known phenomenon commonly associated with psoriasis, but it has been also described in conjuction with a host of other disorders. However it has seldom been associated with necrobiosis lipoidica diabeticorum, a rare skin manifestation of diabetes mellicuts. In this report, we describe a 59-year-old woman who developed necrobiosis lipoidica diabeticorum on the left shin where a mosquito had bitten her 2 months ago. Four months ago, she was diagnosed as necrobiosis lipoidica diabeticorum on the right forearm and shin by biopsy and the lesions were improved with corticosteroids intralesional injection. The lesions were asymptomatic and had grown slowly. Histopathological findings showed a large palisading granulomas composed of histiocytes, epithelioid cells and areas of necrobiosis of collagen with multinucleated giant cells. Van Gieson stain showed decreased and fragmented elastic fibers and there were no deposits of mucin in the necrobiotic areas. The lesions were improved with triamcinolone acetonide intralesional injection and there was no recurrence during 4 months.

A case of Generalized Actinic Granuloma with Giant cell Pattern and Necrobiotic Pattern / 대한피부과학회지

We report a case of actinic granuloma in a 70-year-old woman with diabetes mellitus. Several annular lesions developed in both shins and persisted for five years, and similar lesions developed later mainly in sun-exposed areas. Histopathological study from the annular plaques of the shoulder showed actinic granuloma composed of numerous giant cells with disappearance of elastic fibers. Histopatholoc finding from the shin showed actinic granuloma of several foci of necrobiosis. From this case, we observed two different patterns of actinic granuloma in the same patient.

A case of perforating granuloma annulare / 대한피부과학회지

A 29-year-old woman had multiple, umbilicated papules on her fingers for 2 months. Skin biopsy specimen showed transepidermal perforation filled with plug of cellular debris, degenerated collagen and mucinous material. In the dermis, there were necrobiosis and peripheral palisading of mononuclear cells. Special stainings including masson's trichrome, alcian blue, acid orcein, and PAS showed necrobiotic collagen fibers and acid mucopolysaccharides on the necrobiotic zone and trasepidermal elimination canal. Topical application of 0.05% Clobetasol-17-propionate ointment improved her lesion.

A Case of Malignant Atrophic Papulosis (Degos' Disease) / 대한피부과학회지

A 31-year-old male patient had malignant atrophic papulosis that afected the skin only. He had multiple typical porcelain-white skin lesion on the face and trunk without abdominal symptoms. Histopathologic studies showed moderate inflammatory infiltration in addition to the typical cone-shaped region of necrobiosis, There are no satisfactory treatment modes, and in the present case, the patient's condition improved transiently on oral medication of aspirin, but we lost the contact with him afterwards.

Necrobiosis Lipoidica: Report of a Case / 대한피부과학회지

Necrobiosis Lipoidica is a skin disorder of unknown cause, which shows characteristic clinical and histological findings. With histochemical studies, we report of a case which oecurred on the both pretibial surfaces in a 5]-year-old female. Having had treatment with aspirin and dipyridarnole for 9 months. The results were successful.

A Case of Granuloma Annulare / 대한피부과학회지

Granuloma, annulare is a chronic dermatosis characterized by skin colored, firm intradermal papules or subutaneous nodules, which may be discrete or arranged in an annular configuration. Histologically the dermis shows well define8 areas of necrobiosis of collagen tissue surrounded. by palisading histiocytes and lymphocytes. There may be lobules of epithelioid cells ani multinucleated giant ce.1s surrounding the necrobiotic areas. Many factors have been implicated in the genesis of this reaction. Recent studies have indicated that delayed hypersensitivity reactions rnay be involved in the pathogenesis of granuloma annulare. We present a typical granulorna annulare on a 16 year-old boy who had flesh colored, hyperkeratotic small nodules arranged in ring fashion on the dorsum of hands and right ear auricle.