Chronic linear ulcerations of the inguino-crural and buttocks folds.

Vulvo-perineal Crohn's disease is a rare condition either when it is isolated or associated with digestive manifestations. In the former condition named metastatic Crohn's disease, it may constitute a diagnostic challenge and may be confused especially with other infectious or inflammatory disorders. We report a case of vulvo-perineal Crohn's disease in a 46-year-old woman. A 46-year-old woman was diagnosed with a vulvo-perineal Crohn's disease without digestive involvement. There was a chronic edema of the vulva with linear ulcerations on the inguino-crural regions and the buttocks fold, of 3 years. Treatment with metronidazole (1 g/day for 6 months) led to almost complete healing of the ulcerations with a sustained result. Physicians must be aware of the diverse manifestations and confusing presentations of vulvo-perineal Crohn's disease.

Vulvoperineal Crohn's disease: response to metronidazole.

A 46-year-old woman with a medical history of chronic juvenile arthritis with bilateral prosthetic hips presented with vulvoperineal ulcerations of 3 years' duration. There was no diarrhea or recent weight loss. Cutaneous examination showed asymmetrical vulvar edema of the labia minora and labia majora with deep and linear ulcerations having verrucous borders located on the inguinocrural regions and the buttocks fold (Figure 1). On physical examination there was bilateral limited mobilization of the hips. A biopsy specimen was taken from the border of the vulvar ulceration and histologic examination showed under a hyperplasic epidermis an epithelioid granuloma with multinucleated giant cells of the dermis without caseification. Laboratory analyses and results from chest x-ray were normal. Results for Koch bacilla in the spittle, microbiologic studies (staining for microorganisms and cultures), and tuberculin intradermoreaction were negative. There was no Crohn's disease aspect on colonoscopy, and there was normal small bowel enterography. Systematic intestinal biopsies were also with normal aspect. Based on the clinical data and granulomatous histologic characteristics, the diagnosis of metastatic Crohn's disease without digestive involvement was obtained. The patient was started on metronidazole 1 g/d. After 6 months of treatment, there was an almost-complete healing of ulcerations (Figure 2). Treatment was well-tolerated.

[Side effects of meglumine antimoniate in cutaneous leishmaniasis: 15 cases]. / Les effets indésirables du Glucantime par voie générale, dans le traitement de la leishmaniose cutanée: à propos de 15 cas.

BACKGROUND: Leishmaniasis is wide spread parasitic disease considered to be endemic in 88 countries in both old and new world. The standard treatment remains Meglumine antimoniate. AIM: We study the side effects of systemic meglumine antimoniate in cutaneous leishmaniasis. METHODS: We conduct a retrospective study covering 3-year period (2002- 2005). All medical reports of cutaneous leishmaniasis treated by systemic Meglumine antimoniate are reviewed. RESULTS: The study comprise 63 patients all treated by systemic meglumine antimoniate at the dose of 60 mg/kg/day for 10-15 days. Side effects were noted in 15 cases (12 females and 3 males). The subject's age range from 11 to 78 years. Stibio-intolerance (fever, rash, arthralgia, abdominal pain) was observed in 12 cases and stibiotoxicity in 3 cases: precordialgies 1 case, hyperamylasemia and increase liver enzyme: 1 case, pancytopenia, renal and hepatic failure leading to death: 1 case, skin eruption: 7 cases, pruritus and erythema in the site of injection: 5 cases, urticaria: 1 case. Meglumine antimoniate was stopped in 13 cases. CONCLUSION: Meglumine antimoniate is the generally recommended treatment of cutaneous leishmaniasis. In spite of the rarity of Glucantime's side effects, we recommend a careful survey especially in older patients.

[Anhidrotic/hypohidrotic ectodermal dysplasia: ten cases]. / Dysplasie ectodermique hypo/anhidrotique: 10 observations.

BACKGROUND: Ectodermal dysplasias are rare hereditary diseases characterised by congenital absence of ectodermally derived structures and classified according to four symptoms: trichodysplasia, hypodontia, onychodysplasia and hypohidrosis. AIM: The objective of our study is to precise the epidemioclinical characteristics, the diagnostic tools, the evolution and the treatments of this rare disease through a 10-case series of hypohidrotic ectodermal dysplasia (HED). METHODS: The present report is a retrospective study of all cases of an/hypohidrotic ectodermal dysplasia collected from 1977 to 2006. We have specified age, sex, parental consanguinity, similar familial cases, clinical and histological features, dental, oto-rhino-laryngologic, ophthalmologic and respiratory examinations. RESULTS: Ten cases of HED were collected (average age: 14 years, sex ratio 9/1). The mean duration diagnostic period was of 14 years. Parental consanguinity was registered in 3 cases but only one patient had similar familial cases. All patients had facial dysmorphy, hypotrichosis and hypo/anodontia (respectively 8/10 and 2/10). All patients had clinically and histologically documented hypoplastic (6/10) or aplastic sweat glands (4/10). Extra-cutaneous manifestations were noted in 8 patients (recurrent rhinitis 6/10, recurrent pneumopathies 3/10, xerophtalmy 3/10). CONCLUSION: Our series deals with 10 cases of HED, consisting in Chris-Siemens Touraine syndrome. It highlights the delayed diagnosis of this disease (mean: 14 years) with a diagnosis made at an adult age in four patients. Our study confirm the X-linked heredity (9/10) with a possible autosomal transmission (one female-case). HED is rarely life-threatening, but early diagnosis allows a better quality of life to patients and genetic counselling to parents. Our series illustrates the rarity of HED which is also probably due to its underestimation by clinicians.

[Systemic corticosteroids and their place in the management of hemorrhagic erysipelas]. / Place de la corticothérapie générale dans le traitement de l'érysipèle hémorragique.

BACKGROUND: Haemorrhagic erysipelas is a recently described clinical condition. AIM: Our aim is to study the clinical and evolutive particularities of haemorrhagic erysipelas treated with adjuvant corticotherapy. METHODS: It's a retrospective study of 6 cases of erysipelas of the lower limbs with serious local signs (purpura, bullae, petechia). RESULTS: Surgical examination as well as evaluation of muscular enzymes blood level were achieved in all cases. Adequate antibiotherapy and rapidly degressive systemic corticosteroids (0.5 mg/Kg daily) were prescribed. Fever, pain and cutaneous signs regressed rapidly. Haemorrhagic erysipelas is different from common erysipelas by the presence of serious local signs (bullae, purpura), by its bad response to adequate antibiotics and by its response to systemic steroids.

[Erosive buccal lichen planus: nine case reports]. / Le lichen érosif buccal: à propos de 9 cas.

Erosive oral lichen planus (EOLP) is uncommon. We present nine cases of EOLP in patients aged from 39 to 77 years. The aim of our retrospective study was to determine the clinical particularities and the treatment difficulties of this disease.