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PubMed2006

[Necrobiotic xanthogranuloma with scleroatrophic lichen associated with paraproteinemia].

Necrobiotic xanthogranuloma (Xn) with paraproteinemia is a histiocytoxanthomatosis (non-X histiocytosis) that affects the dermis and subcutaneous tissue of the face and less frequently the trunk and limbs. We present the case of a 58-year-old woman with a previous background of IgG (lambda) paraproteinemia and multiple autoimmune diseases, that associate clinically and histologically typical lesions of Xn on face, neck and limbs and of lichen sclerosus et atrophius (LEA) on skin and mucosae. The treatments performed were ineffective, the Xn lesions followed a chronic and progressive course with increased number, size and ulceration of them. The paraproteinemia has remained stable since it was diagnosed eight years ago. We have not found the association of Xn with paraproteinemia and SAL described in the literature. We review the characteristics of this rare disease and its possible pathogenic mechanisms.

Female↗
PubMed2006

[Necrobiotic xanthogranuloma without monoclonal gammopathy and with a rapidly fatal outcome].

BACKGROUND: Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. CASE REPORT: A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset ofcorticosteroid dependency, iatrogenic Cushing's syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient. DISCUSSION: Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.

Aged↗
PubMed1996

A clinicopathologic approach to granulomatous dermatoses.

Reaching a diagnosis or formulating a differential diagnosis in dermatopathology involves combining information from clinical and pathological sources. Traditionally, this process is presented as a chronologic progression from the patient's complaint, through the evaluation of findings, terminating in the microscopic examination of the biopsy specimen. However, dermatopathologists often find the sequence reversed. They must first form an impression of the diagnosis from the slide and then supplement it with clinical information. The purpose of this article is to present the spectrum of granulomatous dermatoses from a pathologic perspective. The dermatoses are categorized into five groups on the basis of histologic patterns.

Biopsy↗
PubMed1999

Necrobiotic cutaneous T-cell lymphoma.

We report 3 patients with granulomatous cutaneous T-cell lymphoma (CTCL) who showed necrobiosis histologically with palisading granulomas. Although granulomatous change may be present in up to 4% of cases of CTCL, necrobiosis is rare. Misdiagnosis may occur if epidermotropism is minimal or if atypical cells are masked by the granulomatous infiltrate. T-cell receptor gene analysis confirmed the presence of clonal T-cell populations in lesional skin from all 3 cases.

Adult↗
PubMed2006

[Angular pregnancy at eleven weeks gestation on a fibromyomatous uterus].

Reported cases of angular pregnancy, which by definition corresponds to pregnancy developing in a uterine horn, are rare. Etiologies as well as the diagnostic and therapeutic strategies are discussed. The anatomic modifications resulting from uterine fibromyoma and affecting embryo nidation in the uterine cavity of the uterus are the leading cause of angular pregnancy. The course of pregnancy depends on the evolution of the fibromyoma. Treatment therefore depends on the clinical course. Early diagnosis is essential for conservative treatment. In the case presented here, angular pregnancy was diagnosis following development of aseptic necrobiosis, the most common complication of fibromyosma.

Adult↗
PubMed2001

Recalcitrant necrobiotic xanthogranuloma responding to pulsed high-dose oral dexamethasone plus maintenance therapy with oral prednisolone.

A patient with the rare condition necrobiotic xanthogranuloma was treated from 1992 to 1999 with multiple therapies, none of which was effective. Her disease was progressive, causing significant disability. In September 1999 a trial of high-dose pulsed oral dexamethasone with maintenance corticosteroids led to a substantial improvement in her condition.

Aged↗
PubMed1998

Vaccine-induced necrobiotic granuloma.

We report two cases of necrobiotic palisaded granulomas which developed at the site of intradermal hepatitis B vaccination. To the best of our knowledge, this kind of reaction has not been reported previously.

Adolescent↗
PubMed1999

Necrobiotic xanthogranuloma with paraproteinaemia.

A 51-year-old woman developed multiple periorbital nodules. The subsequent demonstration of IgG lambda paraproteinaemia and the histological features of necrobiotic xanthogranulomatous inflammation confirmed the clinical diagnosis of necrobiotic xanthogranuloma with paraproteinaemia.

Female↗
PubMed2002

Wegener's granulomatosis is not a granulomatous disease.

The histologic diagnosis of Wegener's granulomatosis is usually challenging. It is made more so by the failure of most pathologists to realize that the disease is fundamentally neither a vasculitis nor a granulomatous reaction. This problem can contribute to delays in diagnosis and consequent adverse effects for patient prognosis. This article attempts to better describe the essence of Wegener's granulomatosis with the aim of enhancing diagnosis earlier in the course of the disease.

Diagnosis, Differential↗
PubMed2001

Panniculitis. Part I. Mostly septal panniculitis.

The panniculitides represent a group of heterogeneous inflammatory diseases that involve the subcutaneous fat. The specific diagnosis of these diseases requires histopathologic study because different panniculitides usually show the same clinical appearance, which consists of subcutaneous erythematous nodules on the lower extremities. However, the histopathologic study of panniculitis is difficult because of an inadequate clinicopathologic correlation, and the changing evolutionary nature of the lesions means that biopsy specimens are often taken from late-stage lesions, which results in nonspecific histopathologic findings. In addition, large-scalpel incisional biopsies are required. However, we believe that by obtaining appropriate biopsy specimens and with adequate clinicopathologic correlation, a specific diagnosis may be rendered in most cases of panniculitis. It must be accepted that all panniculitides are somewhat mixed because the inflammatory infiltrate involves both the septa and lobules; however, in general the differential diagnosis between a mostly septal and a mostly lobular panniculitis is straightforward at scanning magnification. Mostly septal panniculitides with vasculitis include leukocytoclastic vasculitis involving the small blood vessels of the septa; superficial thrombophlebitis resulting from inflammation and subsequent thrombosis of large veins of the septa; and cutaneous polyarteritis nodosa, which is a vasculitis involving arteries and arterioles of the septa of subcutaneous fat with few or no systemic manifestations. Often septal panniculitides with no vasculitis are the consequence of dermal inflammatory processes extending to the subcutaneous fat, such as necrobiosis lipoidica, scleroderma, subcutaneous granuloma annulare, rheumatoid nodule, and necrobiotic xanthogranuloma. However, in other cases, the inflammatory process is primarily located in the fibrous septa of the subcutis with or without involvement of the overlying dermis. The most frequently seen septal panniculitis is erythema nodosum, which, in fully developed lesions, is characterized histopathologically by Miescher's radial granulomas in the septa.

Adipose Tissue↗
PubMed2004

Upper and lower eyelid reconstruction for severe disfiguring necrobiotic xanthogranuloma.

BACKGROUND: Necrobiotic xanthogranuloma is a rare disease featuring generalized xanthomatous inflammatory skin lesions associated with paraproteinemia and possible lymphoproliferative diseases. Eyelid involvement can be unilateral or bilateral and ranges from minor xanthelasma-like lesions to severe ulcerative disease with consecutive keratitis and scleritis. CASE REPORT: The authors report the case of a 67-year-old woman with extensive necrobiotic xanthogranuloma involving the eyelids, head and neck, anterior chest, and both upper and lower extremities. Periorbital involvement caused severe upper and lower lid ectropium with chronic conjunctival inflammation and unilateral exposure keratitis. During a persistent period of low disease activity, granulomatous lesions and scars were widely excised, lids partially shortened and large full-thickness skin grafts applied. Uninvolved parts of the upper arms had to serve as donor sites, as other possible donor sites were not available. After successful reconstruction of the left side and no local recurrence of the disease, the right side was corrected in the same way. Full eyelid closure was achieved and skin grafts healed without complications. No recurrence of the disease appeared at the sites of operation, despite continuous new lesions elsewhere. CONCLUSION: Severe cicatricial eyelid deformation caused by necrobiotic xanthogranuloma can be treated with success by excision and free skin grafting. The mechanisms of recurrence at excision sites described by others remain unclear, but at least during phases of low activity, the described treatment is safe and recurrence is not to be expected.

Disease Progression↗
PubMed1997

Necrobiotic palisading suture granulomas involving bone and joint: report of two cases.

Biomaterial used in surgery is relatively inert and non-toxic; however, adverse reactions may follow implantation of such foreign material. We describe the first two cases of bone and joint destruction by necrobiotic palisading suture granulomas. The hypersensitivity reaction occurred years after shoulder repair using silk sutures. One patient received chemotherapy for a mistaken diagnosis of tuberculous arthritis. Although very rare, foreign material should be included in the differential diagnosis of necrotizing granulomas. A history of surgery and microscopic examination with polarized light should allow recognition of this entity.

Adult↗
PubMed2001

Conjunctival necrobiotic granuloma in xeroderma pigmentosum.

PURPOSE: We report clinical and histopathologic findings of a conjunctival lesion associated with xeroderma pigmentosum. METHODS: A Saudi girl with known xeroderma pigmentosum presented with pain and photophobia of the right eye and an elevated temporally located perilimbal mass. RESULTS: The mass was resected successfully and has not recurred during 1-year follow-up. Histopathologic examination of the tissue showed a necrobiotic granuloma with associated histiocytic infiltration. The patient had no systemic disease, lipid was not detected in the histiocytic lesion, no Touton giant cells were present, and there was no evidence of elastolysis. CONCLUSION: Ocular malignancies occur in association with xeroderma pigmentosum, but benign lesions that mimic a malignancy may occur.

Adolescent↗
PubMed2004

Collagenolytic (necrobiotic) granulomas: part II--the 'red' granulomas.

A collagenolytic or necrobiotic non-infectious granuloma is one in which a granulomatous infiltrate develops around a central area of altered collagen and elastic fibers. The altered fibers lose their distinct boundaries and exhibit new staining patterns, becoming either more basophilic or eosinophilic. Within the area of altered collagen, there may be deposition of acellular substances such as mucin (blue) or fibrin (red), or there may be neutrophils with nuclear dust (blue), eosinophils (red), or flame figures (red). These color distinctions can be used as a simple algorithm for the diagnosis of collagenolytic granulomas, i.e. 'blue' granulomas vs. 'red' granulomas. Eight diagnoses are included within these two groupings, which are discussed in this two-part article. In the previously published first part, the clinical presentation, pathogenesis and histologic features of the 'blue' collagenolytic granulomas were discussed. These are the lesions of granuloma annulare, Wegener's granulomatosis, and rheumatoid vasculitis. In this second half of the series, the 'red' collagenolytic granulomas are discussed; these are the lesions of necrobiosis lipoidica, necrobiotic xanthogranuloma, rheumatoid nodules, Churg-Strauss syndrome, and eosinophilic cellulitis (Well's Syndrome).

Churg-Strauss Syndrome↗