[Uveitis and tattoos]. / Uvéites et tatouages.

The complications of tattoos are multiple and known for many years. However, their success explains their exponential multiplication in all countries. This confirmed the development of two new complications: a localized or remote skin sarcoidosis reaction, as well as the development of clinical and histological uveitis. This is in the context of a delayed hypersensitivity reaction, or a granulomatous reaction. Their prevention remains rather poorly known. It requires that each tattoo-carrying subject perform a systemic sarcoidosis check-up as well as an ophthalmological investigation in search of possible uveitis. If confirmed, preventive and therapeutic measures will be carried out in emergencies to avoid blindness. These new complications confirm the extreme severity of the use of uncontrolled inks, real toxic and sensitizing mixtures, especially apparently during extensive colorful tattoos. These particularly aggressive colored inks release multiple substances and nanoparticles into the body, not all of which are measured in the medium and long term.

[Rituximab induced remission of pemphigus vulgaris: 2 cases]. / Pemphigus vulgaire évolutif sous corticoïdes et immunosuppresseurs: rémission sous rituximab. Deux observations.

INTRODUCTION: Pemphigus vulgaris frequently requires corticoids and immuno-suppressive drugs. The disease and the side effects of the drugs severely affect the quality of life, and sometime the vital prognosis of the patients. Other treatments than corticosteroids and immunosuppressive drugs are needed. EXEGESIS: We report 2 additional cases of pemphigus vulgaris uncontrolled by corticoids and immuno-suppressive drugs that responded spectacularly to rituximab. One patient had a recently onset disease, that was active despite 1,5 mg/kg/day prednisone and 1,5 g/day mycophenolate. She had a complete remission during 15 months after rituximab treatment. At relapse, another rituximab cycle led to a prompt remission. The other patient had longstanding pemphigus vulgaris complicated by cutaneous infections on prednisone (20 mg/d), immunosuppressive drugs and intravenous immune globulins. She had a prompt and complete remission after rituximab. CONCLUSION: Rituximab seems to be a promising drug for refractory pemphigus vulgaris. The benefit to risk ratio of this drug in this new indication must be precisely documented.

[Nodules on localized scleroderma or morphea]. / Nodules sur sclérodermie en plaque ou morphée.

BACKGROUND: Localized scleroderma or morphea usually appears as flat or depressed lesions. OBSERVATIONS: We report 3 cases of morphea with atypical appearance, alternating pigmented and depigmented patches with nodules or sclerous bands, occurring in adult men. DISCUSSION: The occurrence of nodular elements on generalized or localized scleroderma, although rare, was first reported in the literature by Addisson in 1884. These nodules usually appear during evolution. These scleroderma are then described as being keloidal or nodular. We report 3 cases of nodules on localized scleroderma which appeared at the beginning of the dermatosis and where the scleroderma had a similar unusual irregularly pigmented appearance.

[Worsening of subacute lupus erythematosus induced by infliximab]. / Aggravation d'un lupus érythémateux subaigu sous infliximab.

BACKGROUND: Infliximab (Remicade) is an anti-TNF alpha indicated in the treatment of chronic inflammatory rheumatism, notably rheumatoid arthritis. CASE-REPORT: We report the case of a 56 year-old woman who developed severe worsening of an SSA-positive subacute lupus erythematosus on initiation of treatment with infliximab for rheumatoid arthritis. DISCUSSION: A review of the literature found 30 cases of drug-induced lupus and listed the autoimmune modifications induced by anti-TNF alpha. This first case of subacute lupus erythematosus, existing before the introduction of treatment and worsening during the latter, emphasizes the risk of developing a severe flare of an autoimmune disease during treatment with anti-TNF alpha. It raises the question of the relative contraindications of anti-TNF alpha in patients with lupus erythematosus.

Cutaneous metastasis from follicular carcinoma of the thyroid gland.

A 65-year-old man with a history of malignant melanoma presented a reddish nodule on the left temporal region of the scalp. Histologic features and immunoperoxidase staining on the skin biopsy specimen confirmed the diagnosis of metastatic thyroid follicular carcinoma. The patient presented recurrences of metastatic skin nodules, lung and bone metastases, and died after a 3-year follow-up.

[Anti-seborrhea effect of cimetidine]. / Effet anti-séborrhéique de la cimétidine.

Cimetidine, a histamine H2 antagonist, seems to have anti-androgenic activity. 22 young women were studied during follicular phase prior to and after a 3 month period of treatment with cimetidine (1,200 mg daily). Three month treatment with cimetidine resulted in a significant decrease of seborrheic index (p less than 10(-4)) and casual level (p less than 10(-4)). Testosterone level, TeBG binding capacity and free testosterone were unaffected by the treatment. Cimetidine decrease sebum secretion without change in androgen parameters.

Acne-like eruption induced by pseudoxanthoma elasticum: effectiveness of liquid nitrogen cryotherapy.

We report a case of pseudoxanthoma elasticum associated with an acneiform eruption involving the cervical area. When she was 16 years-old our patient was diagnosed with pseudoxanthoma elasticum, affecting the skin (flexural, cervical and neck areas) and the eyes (bilateral ocular angioid streaks). Ten years later, acneiform lesions (inflammatory and comedones) developed on these lesions: - the inflammatory lesions were characterized by phagocytosis of pathological elastic fibers inducing granuloma - the histological aspects of pseudoxanthoma elasticum were observed around large comedones. This association is rarely reported and we think that atypical, cervical acneiform lesions may be an indication of pseudoxanthoma elasticum. The mechanism for this association is not clearly understood. In our case, Von-Kossa staining was negative for the granulomatous lesions and positive for the comedones: calcification could protect elastopathic fibers from phagocytosis. Treatment is difficult: anti-acneic treatments are not effective except for tetracylines, the anti-inflammatory effects of which can improve granulomatous lesions. As its efficacy has been reported in elastosis perforans serpiginosa, we used liquid nitrogen cryotherapy on the inflammatory lesions with good results (separation of epidermis from dermis perhaps promoting transepithelial elimination of the abnormal dermal elastic tissue).

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.

[Celiac disease associated with cutaneous sarcoidosic granuloma]. / Maladie coeliaque associée à un granulome sarcoïdosique cutané.

Coeliac disease can be associated with numerous internal, skin and mucosa involvements: their physiopathology is often obscure. We report the case of a 14-year old female patient who suffered from a coeliac disease diagnosed in 1988 with considerable improvement with a gluten-free diet. Her two daughters also presented coeliac disease and her sister suffered from nevoid basal cell carcinoma syndrome. Four years later, she presented non pruriginous small nodules over both lower extremities. Skin biopsy revealed a non-caseating granuloma into the derm: we only could evocate sarcoidosis affecting the skin. The dermatological lesions improved during the following weeks with a gluten free diet and relapsed each time this diet was stopped. Many clinical associations with coeliac disease have been described with numerous visceral and skin-mucosa involvements. Eight cases of coeliac disease associated with sarcoidosis affecting the lung have been reported: in five cases, coeliac disease preceded sarcoidosis and in one case sarcoidosis relapsed each time gluten was reintroduced like in our case. This two diseases seem to share immunological and genetic disturbances.

[Sneddon-Wilkinson disease. Four cases report]. / Maladie de Sneddon-Wilkinson. A propos de quatre cas.

INTRODUCTION: We report four cases of subcorneal pustular dermatosis or Sneddon-Wilkinson disease. Clinical and histological lesions and immunofluorescence data were presented. This disease is classified among neutrophilic dermatitis. PATIENTS AND METHODS: All of four patients presented with clinical and histological lesions compatible with the diagnosis of Sneddon-Wilkinson disease. Indeed, direct and indirect immuno-testing were negative. We noted an association with a benign IgA monoclonal gammapathy in one case and with a seronegative polyarthritis in one other case. Three patients correctly responded to dapsone. One of them after transient improvement was resistant to dapsone and then dramatically responded to etretinate. CONCLUSION: Subcorneal pustular dermatosis is a chronic disease, rarely described in literature. It's a pustular eruption, involving the trunck, axillae and inguinal holds. It's often associated with monoclonal gammapathy, particulary IgA. Its nosological situation is still contested, especially with IgA pemphigus sharing with it the association with IgA monoclonal gammapathy and the same efficacy of dapsone. We discuss relationships between both diseases.

[Dermatology and polyposis of the gastro-intestinal tract (author's transl)]. / Dermatologie et polypose digestive.

The authors study three rare syndromes which are characterized by the association of cutaneous manifestations with an intestinal polyposis: Gardner's syndrome, Peutz-Jeghers-Touraine's syndrome, Cronkhite-Canada's syndrome. The Gardner's syndrome is transmitted with an autosomal prevalence, and its vital prognosis remains very porr. It is characterized by the association of various cutaneous manifestations such as fibromas, freckles, etc. with osteomas, neuro-fibromas and polyps of the large bowel. Its severity is related to the very early malignant degeneration of digestive polyps. The Peutz-Jeghers-Touraine's syndrome is transmitted in an autosomal prevalence and its vital prognosis is benign. The cutaneous manifestations are the very early occurrence of lentigines on the face, around the hiatus, and on the lips. The polyps are situated on the small bowel, and are the source of important functional phenomenons; their malignant change is rare. The Cronkhite-Canada's syndrome is rare. Its etiology is unknown and its prognosis is very poor. Its manifestation is the association of more or less wide-spread cutaneous pigmentations, alopecia, and onyxis with a digestive syndrome secondary to a pseudo-polyposis which is the origin for afecal and serous diarrhea, a cause for very severe denutrition. The diagnosis and the treatment of these three syndromes are discussed.

[Keratoderma palmoplantaris striata]. / Kératodermie palmo-plantaire striée.

Keratoderma palmoplantaris striata is a rare disease, with a well-defined clinical presentation which differentiates it from other cutaneous keratoses. Although there is a familial incidence, sporadic cases have been reported. Extracutaneous lesions have also been observed. We report a new case which responded well to retinoid therapy.

[Cutaneous and visceral sarcoidosis. Apropos of an exceptional form]. / Sarcoïdose cutanée et viscérale. A propos d'une forme exceptionnelle.

We present here an exceptional case of cutaneous and vascular sarcoidosis associated with lesions of the liver, spleen and lymph nodes. The disease began when this male patient was 27 years' old and gradually extended over 35 years, despite long-term systemic corticosteroid therapy. Clinically, the initial cutaneous lesions were atrophic, erythematous and squamous, resembling those of erythroderma; they were located on the skin of the right popliteal fossa, the left arm, the neck, the upper part of the chest and around the waist. Subsequently, they spread slowly to involve almost the entire skin, except for the face, right upper limb, left lower limb, hands and feet. The most ancient of these lesions were distinctly black. The skin was paper thin due to complete disappearance of the subcutaneous tissue. Left temporo-parietal alopecia of the cicatricial type developed towards the end of the patient's life. The nails remained normal throughout, and there were no other cutaneous signs of sarcoidosis. Initially, the venous network was clearly visible beneath the atrophic skin, but later on, the veins became grossly dilated and sacculated in those areas which had first been invaded by skin atrophy. Phlebography of the left upper limb confirmed the venous dilatation, while arteriography of the upper limbs showed multiple sacciform aneurysms of the subclavian, axillary and brachial arteries, with distal thrombosis of the latter vessel. Histology showed typical lesions of sarcoidosis in a vein; no arterial biopsy was performed. The muscles had a nodular and sclerous appearance; amyotrophy developed in the last stages of the disease; tendons and joints were normal.(ABSTRACT TRUNCATED AT 250 WORDS)

[Eccrine carcinoma with mucinous stroma of the face]. / Carcinoma eccrine à stroma mucineux de la face.

INTRODUCTION: We report a new case of eccrine carcinoma with mucinous stroma characterized by its voluminous exophytic growth. CASE REPORT: An 80-year-old patient was hospitalized for a voluminous vegetating tumor localized over the left temporal region and which had developed over the 10 preceding years. Wide exeresis was performed and histology confirmed the diagnosis of eccrine carcinoma. No locoregional recurrence was noted 18 months later. DISCUSSION: Eccrine carcinoma with mucinous stroma, also termed eccrine mucinous adenocarcinoma, is a rare adnexal skin carcinoma usually localized on the face or scalp. Abundant zones of mucine are characteristic dissociating a dermal cell proliferation with ruban architecture. The volume of the tumor in our case was particularly remarkable although outcome was favorable after surgical treatment.

[Hypersensitive urticarial vasculitis after natisedine intake]. / Vasculite urticarienne d'hypersensibilité après prise de Natisédine.

BACKGROUND: Various skin and mucosal reactions can be observed after administration of quinidine derivatives. CASE REPORT: A patient who was taking Natisédine (quinidine phenylethyl barbiturate) intermittently and at reintroduction developed a papulopurpuric eruption (without thrombopenia) producing extensive centrifugal annular infiltration and central healing which regressed approximately one week after drug withdrawal. This eruption was associated with moderate 24 h proteinuria. The clinical aspect was that of vasculitis purpura as confirmed histology. Direct immunofluorescence only demonstrated C3 deposits in the vessel walls of the superficial dermis. The quinidine moiety of this drug (currently removed from the formulation) appears to be the responsible agent (imputability score: 13 B3). DISCUSSION: Thrombopenic purpura by synthesis of anti-platelet antibodies induced by quinidine derivatives is well known. Inversely, cases of non-thrombopenic purpura imputable to these same derivatives is uncommon (7 reported cases). The pathophysiological mechanisms involved might be similar (antigenic similarity between the platelet surface and endothelium).

[Atypical manifestations in familial type 1 Waardenburg syndrome]. / Forme atypique au cours d'un syndrome de Waardenburg familial de type I.

BACKGROUND: Waardenburg syndrome is an uncommon genetic disorder. Four clinical types are recognized. Three responsible genes have been identified (PAX 3: for type I syndrome, MITF and EDN3 for types II and IV respectively). CASE REPORT: We report the case of a patient with Waardenburg type I morphotype who had atypical neurological manifestations. Decisive elements for diagnosis were the presence of Waardenburg syndrome in the family and, in affected kin, a mutation causing a shift in PAX 3 gene reading. DISCUSSION: This case confirms the variability of Waardenburg signs within one family. The association of unusual neurological manifestations in the proband suggested that Vogt Koyanagi Harada disease may have been associated and may show some relationship with familial Waardenburg syndrome.

[Cronkhite-Canada disease. Discussion apropos of a case and study of the pigmentation]. / Maladie de Cronkhite-Canada. Discussion à propos d'une observation et étude de la pigmentation.

In 1955, Cronkhite and Canada described two patients presenting abnormal skin pigmentation, alopecia, onychodystrophy and gastrointestinal polyposis. In the first French case reported here, the skin pigmentation has been the object of a special electron microscope study. M. E..., 48-year old, developed, in 1968, pigmented maculae with a metallic gloss around his pelvic girdle. The melanoderma rapidly expanded, associated with fall of hair, body hairs and eyebrows. Onyxis and perionyxis of the right thumb, milium-like epidermal cysts, tumoral lesions of the keratoacanthoma type on the nose and scrotum and, chiefly, generalized cockade-like bullous erythema associated with buccal erosions soon completed the clinical picture. The bullae were subepidermal, and direct and indirect immunofluorescence tests revealed the presence of antibodies directed against the basal membrane area. A few years later, a gastrointestinal syndrome developed progressively, consisting of liquid diarrhoea (8-10 stools per day) with deterioration of the patient's general condition and loss of weight leading to cachexia and, ultimately, death. During periods when the gastrointestinal symptoms regressed the general condition improved, hair and hairs started growing again and pigmentation was less pronounced. Examinations of the digestive tract discarded a malabsorption syndrome. Endoscopy revealed the presence of false polyps with paved appearance of the colonic and rectal mucosae. The mucosa was congested, inflamed and strewn with ulcerations. Histology showed signs of acute proctitis. The abnormal skin pigmentation was the object of histological and ultrastructural analysis. Under the light microscope the epidermis was thicker than normally with increased melanin content. There was marked pigment leakage with numerous melanophages. At electron microscopy the melanocytes, more numerous, showed increased melanogenic activity.(ABSTRACT TRUNCATED AT 250 WORDS)

[Acute generalized exanthematous pustulosis due to fluconazole]. / Pustulose exanthématique aiguë généralisée induite par le fluconazole.

INTRODUCTION: Fluconazole (Triflucan(R)), a systemic triazole antifungal agent is largely prescribed and some cutaneous side effects have already been described. We report the first case of acute generalized exanthematous pustulosis due to this molecule in a patient with cutaneous candidosis. CASE REPORT: A 65 year-old-woman was treated with fluconazole (200 mg/day) for a persistent cutaneous candidosis infection on the buttocks. After the third dose, the patient presented with a pustular eruption with erythema located on her trunk and in her large skin folds. The eruption was associated with fever at 39 degrees C, asthenia and neutrophilia (9,000/mm(3)). The histologic examination and the negativity of microbiological cultures were consistent with the diagnosis of acute generalized exanthematous pustulosis. The eruption cleared with local steroids in about ten days. Nineteen days later, the same pustular eruption occurred but without fever nor neutrophilia. DISCUSSION: Clinical, biological and histological manifestations were consistent with the diagnosis of acute generalized exanthematous pustulosis due to fluconazole. According to the imputability criteria of Begaud et al., intrinsic imputability of fluconazole was possible (I2). According to the classification of the EuroSCAR study, it was certain. No similar case of recurrence had already been described after the withdrawal of the molecule. We believe this is the first case of acute generalized exanthematous pustulosis due to fluconazole (extrinsic imputability: B0).

[Contact eczema induced by propylene glycol. Concentration and vehicle adapted for for patch tests]. / Eczéma de contact au propylène glycol. Concentration et véhicule adaptés pour les tests épicutanés.

INTRODUCTION: Contact dermatitis to propylene glycol, a widely used compound, is often difficult to evidence with skin tests. CASE REPORTS: We observed three cases of contact eczema to a dermal cream (Zovirax) used for labial herpes simplex. Patch-tests were positive in all three cases when the entire product was used but negative for each of the constituent components. The initial diagnosis could be an allergic reaction to the composition between the components as has been described elsewhere. Skin tests were completed with patch-tests using propylene glycol at concentrations over 5 p. 100 or with a vehicle other than vaseline (commercial tests use 5 p. 100 propylene glycol in vaseline). The results of these tests provided evidence allowing the diagnosis of contact dermatitis to the dermal cream due to allergic reaction to propylene glycol. DISCUSSION: Our three cases illustrate the frequency of false negative reactions to propylene glycol on commercial patch-tests. In agreement with data in the literature, these tests show that propylene glycol must be used at concentrations up to 10 to 20 p. 100 to identify allergic reactions with patch-tests.

[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.