[Severe drug hypersensitivity reaction (DRESS syndrome) to doxycycline]. / DRESS syndrome sous doxycycline.

BACKGROUND: While many cases of DRESS reaction to minocycline have been described, few of these involve doxycycline. CASE STUDY: A 59-year-old woman of African origin was repatriated after a journey to Ghana for hyperthermia with infiltrated maculopapular exanthema, facial oedema (no mucosal involvement) and polyadenopathy. Laboratory tests revealed hypereosinophilia, hepatic cytolysis and mononucleosis syndrome. Cutaneous histology was non-specific. The patient had been taking doxycycline as antimalarial prophylaxis for three weeks before the onset of symptoms. DRESS to doxycycline was diagnosed. Patch-tests with doxycycline three months later proved negative. The patient's HLA phenotype was A3/A30 and B39/B42. DISCUSSION: An intrinsic causal relationship with doxycycline was likely in this case (I3). Although patch-test sensitivity and specificity with doxycycline remains unknown in DRESS exploration, a negative result does not necessarily rule out the diagnosis. A number of cases of DRESS to doxycycline have been described recently, possibly as a result of more frequent prescription (malarial prophylaxis, acne). Subjects of African ethnicity or having specific HLA phenotypes are at higher risk of developing drug hypersensitivity. CONCLUSION: This patient is the third case of DRESS to doxycycline described in the literature. The originality of this case lies in the allergological investigation using patch-tests and HLA determination.

Panniculitis revealing qualitative alpha 1 antitrypsine deficiency (MS variant).

A 16-year-old girl presented painful, red, nodular lesions on the abdomen. A cutaneous biopsy showed inflammatory cell infiltrate and fibrosis in the dermis and in the septa with isolated adipocyte lobules. alpha1-antitrypsin level was found to be normal but M1S phenotype of alpha1-antitrypsin was determined by isoelectric focusing in polyacrylamide gel. alpha1-antitrypsin level was normal for her family but M2S phenotype was found in her father. Alpha 1-antitrypsin (alpha1 AT) deficiency is a common hereditary disorder of Caucasians. The locus is pleiomorphic and 75 alleles have been identified. Numerous pathological mutations can be classified by the mechanisms which cause the deficiency. The major clinical importance of this deficiency is emphysema and liver disease. Panniculitis is rarely reported and seems to occur principally for the ZZ or MZ phenotype and for low levels of alpha1 AT. MS phenotype has been more rarely reported and triggering agents such as trauma and infections must be present. However, normal levels of alpha1 AT in the serum have previously been reported as in our case, and we suggest the study of alpha1 AT phenotype even if the plasma level is normal.

[CD30 positive pilotropic lymphoma]. / Lymphome pilotrope CD30 positif.

BACKGROUND: We report an unusual case of cutaneous CD30-positive lymphoma with pilar tropism and circulating Sezary cells which had a rapidly fatal course. CASE REPORT: A 78-year-old man presented erythematous infiltration of the face, a pruriginous eruption on the trunk and proximal portions of the limbs with small erythematopurpuric follicular papulae, and node enlargement in the inguinal and axillary areas. The rest of the clinical examination was normal. Circulating Sezary cells were found in significant numbers on two different blood smears. Histologic and immunohistochemistry examination of a skin biopsy evidenced medium to large sized lymphoid cell infiltration in a perifollicular localization. A few small cells penetrated the pilar apparatus. There was no follicular mucinosis. The tumoral cells expressed CD2, CD3, CD4 and 75 p. 100 were positive for CD30. Node aspiration showed lymphomatous cells and CD3+ and CD30+ lymphomatous infiltration was found on marrow smears. A T clone was evidenced both in blood and bone marrow leading to the diagnosis of pilotropic CD30-positive lymphoma. Chlorambucil and prednisone were given. The patient died 5 months later. DISCUSSION: The cytology findings suggest medium to large cell pleomorphic lymphoma. The circulating Sezary cells, the pilotropic eruption, and the rapidly fatal outcome suggest transformation of a Sezary syndrome into CD30-positive large cell lymphoma which has been described in fungoid mycosis.

Localized lipoatrophia with persistent circulating autoantibodies and partial immunoglobulin A deficiency in a child.

A 7-year-old Caucasian girl developed lipoatrophic areas on the abdomen and left thigh. Laboratory tests showed a partial IgA deficiency, circulating autoantibodies (ANAs, double- and single-stranded anti-DNAs, rheumatoid factor). The clinical aspect resembles lipodystrophia centrifugalis abdominalis infantilis, but autoimmune abnormalities suggest connective tissue panniculitis which underlines the overlap between the different forms of localized lipoatrophias.

[Recurrent phototriggered summer light eruption of the axillary and inguinal area: an atypical topographic form of benign summer lucitis]. / Eruption estivale photodeclenchée récidivante des régions axillaires et inguinales: une forme topographique atypique de lucite estivale bénigne.

UNLABELLED: INTRODUCTION This case report of benign summer light eruption emphasizes the importance of phototests in the diagnosis of photosensitive dermatoses. CASE REPORT: A 25-year-old man, phototype II, had experienced a pruriginous papulovesicular erythematous eruption of the axillary and inguinal regions each summer for 12 years. A high-dose UV phototest (40 J/cm2 x 3 days) directed on the right posterior axillary area and a whole body exposure test (4 J/cm2 UVA, 20 mJ/cm2 UVB x 3 days) were positive both clinically and histologically on day 4. DEM B was normal at 26 mJ/cm2. Iterative polychromatic phototest (DEM x 3 days) in the area usually involved (left posterior axillary region) was negative. The simple UVA (13 J/cm2) and iterative phototests performed on the back were negative. The results of the phototests led to the diagnosis of benign light eruption despite the unusual localization. DISCUSSION: The diagnosis of benign light eruption is generally clinical. Phototests are unnecessary in most cases. Benign light eruption can be triggered by high-dose iterative UVA exposure of the susceptible area or whole body phototests (UVA-UVB). These specific phototests are indicated in atypical forms or localizations in order to determine the course of benign light eruption and to uncover simulations.

[Immunoglobulin G subclass distribution of anti-intercellular substance antibodies in pemphigus]. / Distribution en sous-classes d'immunoglobulines G des anticorps anti-substance intercellulaire du pemphigus.

INTRODUCTION: The purpose of this study was to analyze the IgG subclass distribution of pemphigus anti-epithelial cell surface (ECS) antibodies and to determine whether it differs according to clinical features. MATERIALS AND METHODS: 25 skin biopsies and 16 serum samples, obtained from 27 cases of pemphigus, were analyzed by direct and indirect IF staining, with mice anti-human IgG subclasses monoclonal antibodies. RESULTS: IgG1 deposits were observed in 21 of 25, IgG2 in 2, IgG3 in 0, and IgG4 in the 25 biopsies. IgG1 anti-ECS anti-ECS antibodies were detected in all 16 sera, IgG2 in 1, IgG3 in 1, and IgG4 in 15 sera. The anti-ECS IgG subclass distribution does not differ according to the clinical parameters studied. DISCUSSION: The isotypic restriction to IgG1 and IgG4 subclasses, observed in this study, is similar to previously reported results. The heterogenous distribution and the small number of the studied samples did not allow to put in evidence a correlation with the clinical parameters.