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.
Adhesives ; Aged ; Chin ; Diagnosis ; Eosinophils ; Female ; Follow-Up Studies ; Giant Cells ; Hematologic Diseases ; Hematologic Neoplasms ; Histiocytosis ; Humans ; Inflammation ; Lymphocytes ; Macrophages ; Neck Dissection ; Necrobiotic Disorders ; Necrobiotic Xanthogranuloma ; Neutrophils ; Plasma Cells ; Postoperative Period ; Salivary Glands ; Sialadenitis ; Skin ; Submandibular Gland

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.
Biopsy ; Cicatrix ; Collagen ; Dermis ; Diabetes Mellitus ; Giant Cells ; Granuloma ; Humans ; Lacerations ; Lower Extremity ; Lymphocytes ; Necrobiosis Lipoidica ; Necrobiotic Disorders ; Plasma Cells ; Skin Diseases ; Sutures ; Thigh

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.
Colchicine ; Dapsone ; Dermis ; Diabetes Mellitus ; Dilatation, Pathologic ; Female ; Granuloma ; Granulomatous Disease, Chronic ; Herpes Zoster ; Humans ; Leg ; Leg Ulcer ; Myocardial Ischemia ; Necrobiosis Lipoidica ; Necrobiotic Disorders ; Pancreatic Neoplasms ; Skin ; Ulcer ; Vasculitis

No abstract available.
Cyclosporine ; Necrobiosis Lipoidica ; Necrobiotic Disorders

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.
Humans ; Hyalin ; Hypertension ; Multiple Myeloma ; Necrobiotic Disorders ; Necrobiotic Xanthogranuloma ; Necrosis ; Paraproteinemias

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.
Bacterial Infections ; Cicatrix ; Diabetes Mellitus ; Humans ; Hydroxychloroquine ; Necrobiosis Lipoidica ; Necrobiotic Disorders ; Skin ; Skin Diseases ; Ulcer ; Young Adult

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.
Aged ; Biopsy ; Dermis ; Female ; Granuloma ; Granuloma Annulare ; Humans ; Hydrazines ; Leg ; Necrobiosis Lipoidica ; Necrobiotic Disorders ; Sarcoidosis

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.
Agammaglobulinemia ; Bacterial Infections ; Cell Count ; Common Variable Immunodeficiency ; Granuloma ; Hemoglobins ; Immunoglobulin A ; Immunoglobulin G ; Leg ; Necrobiotic Disorders ; Preschool Child ; Skin ; Skin Manifestations

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.
Aged ; Arthritis ; Arthritis, Rheumatoid ; Connective Tissue Diseases ; Dermatitis ; Female ; Granuloma ; Humans ; Inflammation ; Necrobiotic Disorders ; Neutrophils ; Skin

Necrobiotic xanthogranuloma (NXG) is a rare multisystemic disease that manifests as cutaneous inflammatory lesions and has a strong association with paraproteinemia and an increased risk of plasma cell dyscrasias and other lymphoproliferative disorders. Its general skin manifestation is multiple, asymptomatic, indurated nodules or plaques with a yellowish hue which frequently affect the periorbital regions. This case is unusual in that cutaneous lesions developed only at the extremities without periorbital involvement, accompanied by multiple myeloma.
Extremities ; Lymphoproliferative Disorders ; Multiple Myeloma* ; Necrobiotic Xanthogranuloma* ; Paraproteinemias ; Skin Manifestations