Long-term successful healing of ulcerated necrobiosis lipoidica after topical therapy with becaplermin.

We report a case of chronic leg ulceration occurring in a patient with necrobiosis lipoidica (NL). After many topical treatments had failed to achieve healing, treatment with topical becaplermin was started, which resulted in rapid improvement and ultimately complete healing of the ulceration. Treatment of ulcerated NL is often disappointing. Many topical and systemic drugs have been tried. Becaplermin is a platelet-derived growth factor indicated for the treatment of neuropathic diabetic ulcers measuring < 50 mm in size. To our knowledge, this is only the second case reported in the literature of ulcerated NL successfully treated with becaplermin.

[Eosinophilic fasciitis associated with Borrelia burgdorferi infection]. / Fasciite à éosinophiles survenue secondairement à une infection par Borrelia burgdorferi.

BACKGROUND: Eosinophilic fasciitis (Shulman syndrome) is defined by the association of sclerodermatous skin changes involving underlying fascia and hypereosinophilia. While the aetiology is unknown, some observations suggest an infectious origin. We report the association of eosinophilic fasciitis with an infection involving Borrelia burgdorferi. PATIENTS AND METHODS: A 54 year-old man consulted for a hardened oedema and stiffness of the calves associated with an oedema of the left hand evolving for 4 months. Routine blood tests showed hypereosinophilia at 1.01 G/l and moderate inflammatory syndrome. Diagnosis of eosinophilic fasciitis was confirmed by MRI and muscle biopsy. Since the patient had reported previous tick bites some months before onset, he was tested for Lyme disease. An ELISA test revealed IgG directed against Borrelia burgdorferi and this was confirmed by Western blot analysis. DISCUSSION: The association of eosinophilic fasciitis with Lyme disease raises the question of a real link or a fortuitous association between the two conditions. Similar cases have been described in the literature with or without isolation of the spirochete from skin or fascia lesions. The incidence of eosinophilic fasciitis remains low compared to the prevalence of the infection in endemic areas. We suggest that in some patients, perhaps genetically predisposed, infection with B. burgdorferi may be at the origin of fasciitis.

[Urbach-Wiethe disease (lipoid proteinosis) with neurological involvement]. / Maladie d'Urbach-Wiethe (lipoïdo-protéinose) avec manifestations neurologiques.

INTRODUCTION: Lipoid proteinosis is a rare autosomal recessive disease that has recently been shown to result from mutations in the ECM1 gene. Some cases are associated with a more severe mucocutaneous phenotype. OBSERVATION: We report the case of a 38-year-old woman who had clinical and histological skin features typical of Urbach-Wiethe disease. Physical examination revealed neurological abnormalities including dizziness, ataxia, slight psychomotor retardation and amnesic impairment. The patient reported sudden left brachiofacial paralysis one month earlier that regressed spontaneously after one week. CT scan and MRI were normal. Cerebral scintigraphy displayed bilateral hypoperfusion of the frontal areas, of the anterior and internal right temporal lobe (which includes amygdala), and of the left thalamic core. DISCUSSION: We considered these abnormalities as neurological features of lipoid proteinosis in the absence of evidence of any other cause. Our observation as well as other recent reports suggests the need for neurological and neuropsychological investigations in patients with Urbach-Wiethe disease.

[Necrobiotic xanthogranuloma without monoclonal gammopathy and with a rapidly fatal outcome]. / Xanthogranulome nécrobiotique sans gammapathie monoclonale d'évolution rapidement fatale.

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.

Regression of cutis calcinosis with diltiazem in adult dermatomyositis.

Calcinosis cutis is common in several connective tissue diseases such as dermatomyositis, scleroderma or lupus erythematous. In dermatomyositis, it is more likely to concern children than adults but it is not exceptional in adults. Several treatments have been used empirically with inconsistent success. We report a case of adult cutis calcinosis associated with dermatomyositis which responded dramatically to treatment with diltiazem.

[Agranular CD4+ CD56+ CD123+ hematodermic neoplasm (blastic NK-cell lymphoma) revealed by cutaneous localization: 2 cases]. / Localisations cutanées révélatrices d'une hématodermie CD4+ CD56+ CD123+: 2 cas.

BACKGROUND: Agranular CD4+ CD56+ hematodermic neoplasm (blastic NK-cell lymphoma) has been recently described. The skin is often the first organ involved. OBSERVATIONS: Two old men of respectively 70 and 77 years consulted for infiltrated cutaneous lesions. Preliminary histological examination of cutaneous biopsy taken in both patients showed a malignant proliferation suggesting a cutaneous lymphoma, and the patients were referred. Histological examination of new biopsies showed a very similar proliferation in the 2 cases of monotonous medium-sized mononuclear cells without expression of the common antigens CD3 and CD20 and the expression of CD4, CD56, and CD123. No rearrangement of the T-cell receptor gene or the immunoglobulin heavy chain gene were evidenced. No extracutaneous involvement was initially detected in the first patient. Thrombocytopenia associated with the abnormal presence of 15 p. 100 of circulating CD4+ CD56+ cells was initially found in the second patient. The first patient was treated with chemotherapy, with complete remission. A cutaneous relapse promptly occurred, followed by bone and cerebral localizations. The patient died one year after the diagnosis of the disease, in spite of intensification of the treatment. Treatment is still ongoing in the second patient. COMMENTS: The histological presentation of these two patients was very similar with an unusual phenotype of tumor cells expressing CD4, CD56, CD123, but not expressing CD3 and CD20. Some cases have been published under the "term of blastic NK lymphoma" which is the actual term for the disease in the WHO classification. However, the tumor cells derive from the dendritic plasmacytoid cells, also called type 2 dendritic cells, and perhaps from a common precursor to lymphocyte T and dendritic plasmacytoid cells. In spite of complete cutaneous response in the 2 cases presented, as in other reports, extra-cutaneous involvement occurs quickly. Overall survival is usually poor since nearly all the patients died in less than 3 years. This justifies attempting aggressive protocols, with bone marrow allograft in the younger patients.