[Role of medical treatment for symptomatic leiomyoma management in premenopausal women]. / Place des traitements médicaux : indication, durée, efficacité, chez la femme porteuse de fibromes utérins symptomatiques en période d'activité génitale.

The most frequent symptom with leiomyoma is menometrorrhagia. However, it can be responsible of pelvic pain, dysmenorrhea or urinary and digestive compression when it is particularly voluminous. These recommandations were made in order to review medical management of fibroids. If no therapy is able to have them disappear, various drugs may reduce their related symptoms. Tranexamic acid, non-steroidal anti-inflammatory drugs and high dose of oestrogen may be useful in the management of acute hemorrhagic disorders. Progestin, such as lynestrenol induces small reduction in leiomyoma volume and moderate increase in hemoglobin level before surgery. Pregnane and nor-pregnane may improve menstrual bleeding in short or mild delays. The use of Gonadotropin Releasing Hormone (GnRH) agonists can reduce menstrual bleeding with hemoglobin recovery. Add-back therapy using tibolone seems interesting since secondary effects encountered with GnRH agonists may be reduced. Levonorgestrel-releasing intrauterine system is proven to reduce increased menstrual bleeding and restore hemoglobin level. Aminoglutethimide and fadrozole have been underevaluated to conclude when letrozole seems as efficient as GnRH agonists to reduce leiomyoma volume and provide less hot flushes. Anastrozol is associated with reduction in leiomyomata volume, pain and menstrual bleeding. Mifepristone reduces the size of uterine leiomyomata, improves symptomatology, but could be associated with development of endometrial hyperplasia. SPRM evaluated in females have shown to improve leiomyoma related symptomatology. Danazol could be useful to reduce leiomyoma related symptoms in short terms. Tamoxifen and raloxifen show modest overall benefit. Because of insufficient data concerning fulvestrant, pirfenidone or interferon, their prescription cannot be recommended in patients with leiomyomata.

An unusual skin presentation of necrobiotic xanthogranuloma.

A well appearing 73-year-old Caucasian lady presented with a long-standing history of yellowish atrophic lesions on her limbs and trunk. The lesions were asymptomatic. These were found to be consistent with the diagnosis of Necrobiotic Xanthogranuloma (NXG) on histology. The reported patient did not have the characteristic skin changes and instead had unusual lesions reminiscent of morphoea not described in the literature. She reported no associated systemic symptoms.

Generalized necrobiotic xanthogranuloma successfully treated with lenalidomide.

Necrobiotic xanthogranuloma (NXG) presents a therapeutic challenge to clinicians. Generalized NXG has limited treatment options. A patient presented to the authors with generalized NXG associated with monoclonal gammopathy of unknown significance (MGUS), a plasma cell dyscrasia considered to be a precursor to multiple myeloma. The patient was treated with lenalidomide, a derivative of thalidomide with efficacy in treatment of multiple myeloma. Resolution of paraproteinemia was associated with resolution of NXG.

Necrobiotic xanthogranuloma without paraproteinaemia: marked improvement with psoralen ultraviolet A treatment.

Necrobiotic xanthogranuloma (NXG) is a rare granulomatous condition that is often associated with a paraproteinaemia and in some cases multiple myeloma. Treatment is therefore aimed at the underlying associated haematological abnormality. However, isolated NXG cases have been reported. We report a case of isolated NXG that responded very well to systemic psoralen ultraviolet A (PUVA) treatment. The rationale for this treatment was the successful use of PUVA treatment in other necrobiotic conditions such as granuloma annulare and necrobiosis lipoidica.

[Necrobiotic xanthogranuloma with scleroatrophic lichen associated with paraproteinemia]. / Xantogranuloma necrobiótico con paraproteinemia asociado a liquen escleroatrófico.

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.

Necrobiotic xanthogranuloma with paraproteinaemia.

Necrobiotic xanthogranuloma is a rare cutaneous condition that can be mistaken for atypical necrobiosis lipoidica. It has a strong association with a paraproteinaemia, which may progress to frank haematological malignancy. We describe four patients with variable cutaneous features, and their treatment response.

Necrobiotic xanthogranuloma associated with choroidal infiltration and syncytial giant cell hepatitis.

A 31-year-old woman developed necrobiotic xanthogranuloma (NXG), a thickened choroid, and syncytial giant cell hepatitis, a previously unreported association. NXG and syncytial giant cell hepatitis may have a common autoimmune pathogenesis.

Necrobiotic xanthogranuloma-rapid progression under treatment with melphalan.

Necrobiotic xanthogranuloma is a systemic disease associated in most cases with monoclonal paraproteinaemia. Although the causative role of the paraproteinaemia is supposed, the pathogenesis of this disease remains unknown. We report a 65-year-old woman with a 28-year history of disseminated indurated plaques involving her face, trunk and extremities. Since 1994 an IgG kappa paraproteinaemia was present. Current biopsies showed typical histological features of necrobiotic xanthogranuloma. We treated this extraordinary widespread condition with melphalan and prednisolone. Although the serum levels of IgG kappa light chains and gamma globulins decreased, the cutaneous plaques extended rapidly in size and number, which casts doubt on the causative significance of the paraproteinaemia in the pathogenesis of necrobiotic xanthogranuloma. The paraproteinaemia in patients with necrobiotic xanthogranuloma may rather reflect a secondary phenomenon than the originating cause. Pathogenetic and therapeutical concepts based thereon that have been proposed so far should be critically reconsidered.

Case of recalcitrant necrobiotic xanthogranuloma.

A 53-year-old woman with necrobiotic xanthogranuloma presented as infiltrated eyelid plaques, with later development of arm and thigh lesions. Clinical features, including association with immunoglobulin G-kappa paraprotein, and pathological findings were typical of this disorder. Treatment for 15 months with varying combinations of prednisone and multiple chemotherapeutic agents (melphalan, cyclophosphamide and chlorambucil) has led to minimal or no improvement in the clinical lesions or paraprotein. The case demonstrates some of the difficulties in managing this unusual disorder.

An unusual cause of refractive chronic bilateral leg ulceration.

BACKGROUND: Ulceration of the lower extremities is a common disorder that can be very painful. It occurs most frequently in the elderly population as a result of venous hypertension. We report an unusual case of a 32-year-old man with an 11-year history of extensive, painful, bilateral leg ulcers resistant to conventional treatment necessitating above-knee amputation of the left leg. Eventually, the patient was treated with prednisone, which led to almost complete healing of the ulcers of the right leg. OBJECTIVE: The objective of this report is to discuss a rare cause of chronic bilateral leg ulceration. METHODS: Detailed histopathologic examination showed a complex pattern of overlapping features of several specific dermatologic conditions, including necrobiosis lipoidica (NL), necrobiotic xanthogranuloma (NXG), and the destructive form of granuloma annulare (GA). CONCLUSION: The characteristics of our patient suggest a variant of a cutaneous necrobiotic disorder that has not been previously reported. When clinicians are confronted with recalcitrant ulcerations in the lower extremity, this group of disorders should be considered in the differential diagnosis.

[Degos atrophic malignant papulosis. Not always malignant!]. / Papulosis maligna atrophicans Degos. Nicht immer maligne!

A patient developed malignant atrophic papulosis with only cutaneous manifestation. Repeated coloscopy uncovered no malignant papules in the colon. Referring to the literature, the value of permanent anticoagulant therapy is discussed. In contrast to the term "malignant" atrophic papulosis, there also seems to be a variant with a benign clinical course.