[IgE mediated anaphylaxis in a patient with systemic mastocytosis]. / Anaphylaxie médiée par les IgE chez un patient atteint de mastocytose systémique.

BACKGROUND: Anaphylaxis is a severe, generalized, life-threatening reaction of rapid onset. We report the case of a patient presenting several systemic anaphylactic reactions over many years, initially ascribed to a cereals allergy but which finally proved to be due to systemic mastocytosis hidden for a long time. PATIENTS AND METHODS: A 53-year-old man consulted for an eruption consisting of monomorphic pigmented maculopapular lesions on the trunk associated with itching and urticaria. He was a farmer and presented severe sensitivity to cereals, with anaphylaxis, which continued despite withdrawal of these allergens. Skin and bone marrow infiltration, abnormal mast cells, positivity for c-kit 816 mutation and the persistent elevation of serum tryptase enabled a diagnosis of indolent systemic mastocytosis to be made. DISCUSSION: In systemic mastocytosis anaphylaxis is an expected complication relating to the proliferation of mast cells and a massive increase in mediator release (non-immunological mechanism). All patients with severe and recurrent anaphylaxis should be analyzed for underlying mastocytosis by careful physical examination and assay of baseline tryptase.

[Lymphogranuloma venereum: new serovariant L2b and old "groove sign"]. / Lymphogranulomatose vénérienne : nouveau sérovariant L2b et ancien "signe de la poulie".

An ongoing lymphogranuloma venereum (LGV) outbreak has been reported in several European countries since 2003, related to a new variant L2b. This serovar appears to affect men who have sex with men (MSM), most of them being co-infected with the Human Immunodeficiency Virus (HIV). The secondary stage of LGV may involve lymph nodes and the inguinal form has sometimes been described on each side of the inguinal ligament thus named the "groove sign". We report the case of LGV serovariant L2b acquired by an heterosexual intercourse in an HIV seronegative patient who presented with an inguinal lymph node and a "groove sign". This is an uncommon but suggestive sign of LGV and we suggest that the clinical presentation of L2b LGV might not be so different than other variants and than the 20th century authors' description. Such a new Chlamydia trachomatis variant may circulate in other populations than MSM in Europe and clinical awareness must prevail.

[Antidesmoglein antibodies in a patient with Hailey-Hailey disease]. / Détection d'anticorps antidesmogléines circulants chez un patient atteint de maladie de Hailey-Hailey.

BACKGROUND: Hailey-Hailey disease (HHD) is a rare hereditary disease in which the genetic defect is characterized by mutation in the ATP2C1 gene coding for a transmembrane calcium pump. It is generally considered a non-immunologic acantholytic dermatosis in which direct and indirect immunofluorescence studies are negative, unlike in autoimmune pemphigus. PATIENTS AND METHODS: We describe a case of HHD associated with antidesmoglein antibodies in a 53-year-old woman. The clinical symptoms and histology were typical of HHD. Antidesmoglein antibody tests were positive on several occasions and a difference was found between the two types of Elisa test performed (positive with the MBL kit, negative with the Euroimmun kit). DISCUSSION: The positive result for desmoglein antibodies could be due to unmasking of antigens by the mechanism of acantholysis. The specificity of the main desmoglein Elisa tests also requires discussion.

Contact photoallergy to isothipendyl chlorhydrate.

Isothipendyl chlorhydrate is an azaphenothiazine, an active ingredient of an antipruriginous gel, Apaisyl gel® (Merck Médication Familiale, Dijon, France). Although Apaisyl gel is registered and used worldwide, we present the first case of contact photoallergy to isothipendyl chlorhydrate to our knowledge. The diagnosis suspected on the basis of a positive UVA photopatch test to chlorpromazine was confirmed by a strongly positive UVA Apaisyl gel photopatch test and our photophysical studies. This case confirms the need to keep the phenothiazines in the photopatch test standard series as a diagnostic marker of phenothiazine photoallergy.

Significant delay in the introduction of systemic treatment of moderate to severe psoriasis: a prospective multicentre observational study in outpatients from hospital dermatology departments in France.

BACKGROUND: There is a low rate of systemic treatment usage in moderate to severe psoriasis. OBJECTIVES: The primary objective of the present study was to assess the time period between lack of control of moderate to severe psoriasis with topical treatment or phototherapy as perceived by patients and the medical decision to introduce a systemic treatment. METHODS: This was a prospective multicentre study, which included patients with moderate to severe psoriasis. A standardized questionnaire was completed by physicians and patients at the time the decision was taken to introduce a systemic treatment. The primary outcome was the duration of uncontrolled psoriasis, as estimated by the patient, prior to the introduction of systemic treatment. Factors associated with a delay in systemic treatment defined as > 2 years of uncontrolled psoriasis were assessed. The agreement between patients and physicians on the duration of uncontrolled psoriasis was estimated. RESULTS: The study included 142 patients. The mean age was 48 years, the mean Psoriasis Area and Severity index (PASI) was 18·5 and the mean Dermatology Life Quality Index (DLQI) was 12. The median duration of uncontrolled psoriasis estimated by patients and physicians was 3 years and 2 years, respectively. Factors associated with a delay in the introduction of systemic treatment as assessed by patients were fewer than three physician visits since psoriasis was uncontrolled [odds ratio (OR) 3·05; 95% confidence interval (CI) 1·29-7·21], Hospital Anxiety and Depression (HAD) scale < 10 (OR 2·83; 95% CI 1·19-6·71), continuous psoriasis evolution (OR 2·67; 95% CI 1·12-6·42), low consumption of topical treatment (OR 2·35; 95% CI 1·03-5·34). CONCLUSIONS: There is a significant delay in the introduction of systemic treatment in moderate to severe psoriasis. Patients with low level anxiety and limited use of healthcare resources appear to be at higher risk of experiencing long delays.

[Recurrent breast cellulitis associated with lymphangiectasia after tumorectomy for breast cancer]. / Érysipèle récidivant associé à des lymphangiectasies après traitement conservateur d'un cancer du sein.

BACKGROUND: Recurrent breast cellulitis has been described as a complication following breast conservation therapy. OBSERVATION: A 50-year-old woman undergoing tumour excision, postoperative radiotherapy and chemotherapy presented recurrent breast cellulitis in the same region. The presence of lymphangiectasia suggested a complication subsequent to lymph stasis. DISCUSSION: Conservative therapy for breast cancer, allowing the development of subclinical or patent lymphœdema, constitutes a prominent risk factor for recurrent cellulitis. This complication has also been considered in patients with lower extremity cellulitis following saphenous venectomy for coronary bypass surgery. The unusual presence of lymphangiectasia observed in our patient provides clear evidence that lymphœdema is the most prominent risk factor for the development of cellulitis after breast conservation therapy.

[Autochthonous filaria in south-west France caused by Dirofilaria repens]. / Dirofilariose autochtone à Dirofilaria repens dans le Sud-Ouest de la France.

BACKGROUND: Dirofilariasis is an autochthonous disease caused by Dirofilaria (Nochtiella) repens, a subcutaneous parasite of dogs and other carnivorous animals. Human infection leads to a self-limiting disease that generally presents clinically as a subcutaneous nodule. We describe a case occurring outside the classical endemic Mediterranean region. PATIENTS AND METHODS: A 38-year-old woman presented with a scalp nodule present for six months. Surgery revealed a thread-like parasite identified as a female Dirofilaria repens without microfilariae. DISCUSSION: This case points to the spread of this disease to temperate areas beyond the regions classically affected.

Herpes gestationis in mother and erythemato-pustulous rash in the newborn with positive direct immunofluorescence. A transfer of the disease?

A 24-year-old secundigravida presented at 38 weeks of amenorrhea with typical cutaneous lesions of herpes gestationis. The newborn developed a skin rash 3 days after birth. The rash clinically resembled erythema toxicum neonatorum with dermal infiltrate of lymphocytes and eosinophilic polymorphonuclear cells forming a few intraepidermal pustules. The complement-binding antibody test for herpes gestationis factor was negative, but direct immunofluorescence study on this biopsy demonstrated a linear deposit of IgG and complement on the basement membrane zone. The possible transmission of the disease to the newborn is discussed.

[A case of bullous dermatosis at the end of pregnancy transmitted to the newborn: herpes gestationis]. / Un cas de dermatose bulleuse de fin de grossesse "transmise" au nouveau-né: herpes gestationis.

The authors report a case of bullous dermatosis developing at 38 weeks of amenorrhoea in a para II. The newborn infant developed a skin rash 3 days after birth. Direct immunofluorescent study of skin biopsies showed the presence of a very small amount of IgG and a very large amount of complement fixed to the basal membrane. Tests for the presence of Herpes gestationis factor in maternal and child blood by immunofluorescence to anti-complement were negative. Tests for anti-epidermal antibodies by indirect immunofluorescence in the serum were also negative, for both anti-basal membrane antibodies as well as anti-intercellular substance antibodies.

[The Yellow-nails syndrome. Apropos of 2 cases. Review of the literature]. / Syndrome des ongles jaunes. A propos de 2 cas. Revue de la littérature.

The authors report 2 cases of yellow nails. These cases have evolved these last years, producing clinical aspects that reflect a typical association between the pulmonary and/or sinus reactions and the yellow nails. The authors discuss the scarce typical to the yellow nails syndrome and the different associations reported in the literature. The physiopathology is not known but many hypothesis have been discussed.