1/67. "Neuro-sweet disease": benign recurrent encephalitis with neutrophilic dermatosis.OBJECTIVE: To describe benign recurrent encephalitis in a case of sweet syndrome that also showed clinical features of Behcet disease. CASE REPORT: A 37-year-old Japanese man developed relapsing and remitting encephalitis and mucocutaneous symptoms mimicking Behcet disease. Magnetic resonance images showed at least 5 episodes of transient abnormal signal intensity in various cerebral regions over a period of 5 years. A skin biopsy specimen of the cutaneous edematous erythematous plaques revealed neutrophilic dermatitis compatible with sweet syndrome. HLA typing showed B54, which is frequent in sweet syndrome but rare in Behcet disease. Oral prednisolone therapy (10-60 mg/d) was remarkably effective for the encephalitis as well as for the mucocutaneous symptoms. CONCLUSION: We propose that there is an entity that is like Sweet disease, but with recurrent encephalitis characterized by an association with HLA-B54 and a high responsiveness to corticosteroid therapy, which we have tentatively named neuro-Sweet disease, that is distinct from the classic central nervous system involvement of Behcet disease.- - - - - - - - - - ranking = 1keywords = neutrophilic dermatosis, neutrophilic, dermatosis, sweet (Clic here for more details about this article) |
2/67. Eosinophilic cellulitis following the lines of Blaschko.Eosinophilic cellulitis is an inflammatory dermatosis of unknown aetiology. We describe a case following the lines of Blaschko, which may be the expression of cutaneous mosaicism, suggesting a possible underlying genetic predisposition for the development of Wells' syndrome.- - - - - - - - - - ranking = 0.022717256403338keywords = dermatosis (Clic here for more details about this article) |
3/67. Neutrophilic eccrine hidradenitis secondary to infection with serratia marcescens.Neutrophilic eccrine hidradenitis (NEH) is a rare dermatosis which usually develops after administration of chemotherapeutic treatments. An infective origin is exceptional. We report a patient, previously operated on for ependymoma, who presented with an eruption typical of NEH even though he had not received chemotherapy. culture of a skin biopsy revealed serratia marcescens. The dermatosis improved after antibiotic therapy but recurred twice and culture again isolated S. marcescens; electron microscopy revealed cytoplasmic inclusions within neutrophils, suggestive of bacteria. The disease improved every time with appropriate antibiotic therapy. An infective aetiology for NEH is rare: three such cases have been reported, of which one was due to S. marcescens. The originality of our case is the recurrence of the disease on three occasions with the same bacterium isolated on each occasion, with disease remission after antibiotic therapy. This case confirms that infections may be a possible cause of NEH and underlines the necessity to search for infective agents, especially in patients immunocompromised by haematopoietic malignancies and/or chemotherapeutic treatments.- - - - - - - - - - ranking = 0.045434512806676keywords = dermatosis (Clic here for more details about this article) |
4/67. Recurrent neutrophilic eccrine hidradenitis in an hiv-1-infected patient.Most cases of neutrophilic eccrine hidradenitis (NEH) have been reported in patients receiving chemotherapy for acute myelogenous leukemia or other malignant diseases. We report the case of an hiv-1-infected patient who presented several and strikingly similar episodes of NEH without any medication as well as another one after the first course of chemotherapy for a B-cell non-Hodgkin's lymphoma. This case strengthens the hypothesis that NEH may occur in different situations, as reported for other neutrophilic dermatoses.- - - - - - - - - - ranking = 0.38224102348822keywords = neutrophilic (Clic here for more details about this article) |
5/67. Recurrent cholestatic jaundice associated with generalized pustular psoriasis: evidence for a neutrophilic cholangitis.Generalized pustular psoriasis can result in systemic complications. We report the case of a woman with relapsing generalized pustular psoriasis and recurring episodes of cholestatic jaundice. liver biopsy performed during an attack showed a neutrophilic infiltrate surrounding and invading portal triad bile ducts. Ultrasonographic exams and retrograde cholangiography ruled out biliary tract disease. This observation suggests that recurring cholestatic jaundice in pustular psoriasis is related to a neutrophilic cholangitis.- - - - - - - - - - ranking = 0.38224102348822keywords = neutrophilic (Clic here for more details about this article) |
6/67. Wells' syndrome associated with recurrent giardiasis.Wells' syndrome (WS) is a rare, recurrent, inflammatory dermatosis of uncertain pathogenesis, although a hypersensitivity response to different stimuli has been postulated. Several cases in association with parasitic infections have been described. We present a patient with WS associated with recurrent giardiasis. The course of the dermatosis was strictly related to the course of the parasitic infection. Both diseases disappeared with antiparasitic treatment. To our knowledge, this association is here reported for the first time.- - - - - - - - - - ranking = 0.045434512806676keywords = dermatosis (Clic here for more details about this article) |
7/67. Neutrophilic dermatosis of the dorsal hands: pustular vasculitis revisited.An entity termed "pustular vasculitis of the hands" was recently described. patients with this condition presented with low-grade fevers and erythematous plaques, pustules, and bullae limited to the dorsal hands and fingers, which were characterized histologically by a dense neutrophilic infiltrate and leukocytoclastic vasculitis. We describe patients with a similar clinical presentation, but who lacked vasculitis on biopsy findings. We describe 3 otherwise asymptomatic patients with hemorrhagic bullae, plaques, and pustules solely on the dorsal hands. biopsy specimens showed a neutrophilic infiltrate and leukocytoclasis, but no necrotizing vasculitis, and were reminiscent of Sweet's neutrophilic dermatoses. In our patients, corticosteroids or dapsone led to clearing of the lesions, and small maintenance doses of dapsone prevented their recurrence. Our 3 patients had clinical lesions similar to those termed pustular vasculitis of the hands, but which lacked leukocytoclastic vasculitis on biopsy findings. Because of histologic findings and a therapeutic response more characteristic of Sweet's syndrome, we propose the term neutrophilic dermatosis of the dorsal hands. In addition, low-dose dapsone is proposed as a possible first-line therapy in this condition, especially in those with recurrent disease.- - - - - - - - - - ranking = 0.50694769924151keywords = neutrophilic dermatosis, neutrophilic, dermatosis (Clic here for more details about this article) |
8/67. A case of recurrent impetigo herpetiformis with a positive family history.impetigo herpetiformis is a rare non-infectious pustular dermatosis of pregnancy. A few non-gestational cases due to oral contraceptive use have also been reported. Although the disorder is not thought to have a genetic background, there are some familial case reports in the literature. We describe a case of recurrent impetigo herpetiformis in an 18-year-old pregnant woman who had normal serum calcium levels and responded well to prednisolone therapy. Interestingly, the patient's mother had also experienced a generalised pustular dermatosis associated with hypocalcaemia during oral contraceptive use, which was diagnosed clinically and histologically as impetigo herpetiformis.- - - - - - - - - - ranking = 0.045434512806676keywords = dermatosis (Clic here for more details about this article) |
9/67. Pustular idiopathic recurrent palmoplantar hidradenitis: an unusual clinical feature.A 12-year-old boy had painful lesions on both soles. He had had a similar episode several months before that resolved spontaneously. physical examination showed erythematous, extremely tender nodules on both plantar surfaces and the toes. The second and third left toes had small pustules on top of the nodules. There was no palmar involvement. The clinical features, pathologic findings, and self-limited course suggested recurrent palmoplantar hidradenitis. This distinctive entity of unknown origin appears during childhood and is characterized histologically by a neutrophilic infiltrate affecting the eccrine glands. We report the case of a patient with unusual clinical features.- - - - - - - - - - ranking = 0.063706837248036keywords = neutrophilic (Clic here for more details about this article) |
10/67. Late recurrence of inflammatory first-stage lesions in incontinentia pigmenti: an unusual phenomenon and a fascinating pathologic mechanism.BACKGROUND: incontinentia pigmenti (IP) is an X-linked genodermatosis that is manifested by neonatal inflammatory vesicles localized along the lines of Blaschko. These lesions usually clear spontaneously within a few months, leaving hyperpigmentation. Ophthalmologic and neurologic symptoms can be associated with IP. Late recurrences of the first-stage inflammatory lesions after the initial rash are uncommon and have been reported infrequently. The mechanism involved in this phenomenon is unclear. However, the recent identification of NEMO/IKKgamma as the gene responsible for IP sheds new light on its pathophysiologic origins. OBSERVATIONS: We report 5 cases of children who experienced episodes of late reactivation of IP. In all cases, the recurrences occurred on the previously hyperpigmented streaks several months or years after resolution of the initial eruptions. In most cases, the recurrences were preceded by an infectious episode. CONCLUSIONS: These IP recurrences suggest that mutated cells can persist a long time in the epidermis. We theorize that infections trigger the reactivations. The NEMO/IKKgamma gene encodes a protein essential in nuclear factor kappaB activation, which is required for resistance to tumor necrosis factor alpha-induced apoptosis. We discuss the role of a proinflammatory cytokine such as tumor necrosis factor alpha as a triggering factor for the reactivation.- - - - - - - - - - ranking = 0.022717256403338keywords = dermatosis (Clic here for more details about this article) |
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