[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.

Myasthenia gravis with cutaneous polyarteritis nodosa.

Cutaneous polyarteritis nodosa presents with typical cutaneous lesions of polyarteritis nodosa without visceral involvement at the time of diagnosis. We report a 32-year-old woman with a 12-year history of myasthenia gravis under immunosuppressive treatment, who presented with tender, erythematous nodules 20 mm in diameter on her shins. A diagnosis of cutaneous polyarteritis nodosa was made, based on the clinical and histopathological findings. Myasthenia gravis, an antibody-mediated disease, is occasionally accompanied by other autoimmune diseases, but we found no previous report in the literature associating it with polyarteritis nodosa.

[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.

[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.

Pseudoxanthoma elasticum and nephrolithiasis.

We report the case of a 42-year-old man with pseudoxanthoma elasticum (PXE) and recurrent bilateral nephrolithiasis. Diagnosis of PXE was made by yellow papules on the neck and ophthalmologic angioid streaks. This diagnosis was confirmed by a skin biopsy (Von Kossa stain) and by genotyping analysis of ABCC6 (homozygous mutation R1138Q). Nephrolithiasis was recurrent and biological investigations showed hypophosphoraemia, hyperphosphaturia, hypercalciuria, normocalcaemia, normal serum parathyroid hormone value, high 1,25-dihydroxy vitamin D value and a renal calcium oxalate stone. ABCC6 encodes for MRP6, a multidrug resistant protein strongly expressed in the liver and kidney. The substrates of the MRP6 remain unknown. As PXE is characterized by calcification of elastic fibres and this patient presents important phosphocalcic anomalies, we discuss the possible implications of MRP6 in the phosphocalcic metabolism.

Lupus-like phototriggering in a young woman with benign summer light eruption.

We report the case of a young woman with a single history of benign summer light eruption (BSLE) who developed delayed onset annular lupus-like lesions triggered by a polychromatic phototest, 6 weeks after the irradiation. BSLE of French authors is an idiopathic photodermatosis that corresponds to the minor form of polymorphic light eruption (PLE) of Anglo-Saxon authors. This patient may develop a true lupus erythematosus in the future as indicated by this lupus-like phototriggering and in view of the high prevalence of PLE in lupus patients.

Sweet's syndrome revealing relapsing polychondritis.

Sweet's syndrome is extremely rare in relapsing polychondritis. We report a rare case of Sweet's syndrome revealing relapsing polychondritis. A 77-year-old man presented with fever, associated with respiratory symptoms and non pruritic, painful, erythematous, papules and plaques mainly on the arms and legs. Skin biopsy of the arm found a neutrophilic dermal infiltrate consistent with Sweet's syndrome. A month and a half after admission, the patient presented with ear chondritis and dysphonia. Biopsy of the ear cartilage found a significant perichondral inflammatory cells infiltrate. The diagnosis of relapsing polychondritis was established and a steroid treatment was instituted with a complete regression of the cutaneous signs and chondritis.

Multiple perforating and non perforating pilomatricomas in a patient with Churg-Strauss syndrome and Rubinstein-Taybi syndrome.

We report an unusual association of multiple perforating and non-perforating pilomatricomas with Churg-Strauss syndrome, and a dysmorphic syndrome evocative of Rubinstein-Taybi syndrome. These syndromes may be independent, but these rare diseases and genetic abnormalities may be linked together.

[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.

[Eccrine angiomatous hamartoma: an atypical case]. / Hamartome angio-eccrine. Un cas de présentation atypique.

BACKGROUND: We report a case of eccrine angiomatous hamartoma. This rare and benign skin disease is histologically characterized by the proliferation of both eccrine glands and vascular structures. CASE REPORT: A 65 year-old man had developed during the previous 8 years post-traumatic, angiomatous, extensive and plurifocal lesions of the left lower limb. There was neither pain or local hyperhidrosis. The diagnosis of eccrine angiomatous hamartoma was made on histological evidence. DISCUSSION: This observation is original because of the onset of the disease after 50 years of age, its widespread and plurifocal character and probable triggering-off by minor local trauma. The classical surgical treatment of eccrine angiomatous hamartoma was not suitable and a laser-CO2 therapy was undertaken instead. The results were very discreet.

[Cutaneous T-cell lymphoma, cutaneous hyperpigmentation and paraneoplastic pemphigus]. / Lymphome cutané T, hyperpigmentation cutanée et pemphigus paranéoplasique.

BACKGROUND: Paraneoplastic pemphigus is an autoimmune mucocutaneous disease usually associated with neoplasia as lymphoid proliferations. We report the original case of a patient who had developed a mycosis fongoide preceded by a paraneoplastic pemphigus. To our knowledge, this association has never been reported before. Cutaneous manifestations of mycosis fongoide as pigmentary change are known. This mycosis fongoide was particular in its progressive cutaneous hyperpigmentation. CASE REPORT: A 70-year-old male patient developed a mycosis fongoide with CD30 positive cells in the dermis several months after the diagnosis of a paraneoplastic pemphigus. Simultaneously, a cutaneous hyperpigmentation was predominantly noticed on photo-exposed areas, which improved after chemotherapy. DISCUSSION: Paraneoplastic pemphigus may precede the cancer, as is shown by our present case. This paraneoplastic pemphigus is singular because of the lack of oral erosions, patient's prolonged survival and its association with a mycosis fongoide. The diagnosis of mycosis fongoide with CD30 + cells was finally established together with its relationship to the cutaneous hyperpigmentation. Indeed, a few cases of pigmentary changes in mycosis fongoide have already been reported. The pathogenesis is still unknown, but the role of mast cell and stem cell factor in hyperpigmented mycosis fongoide has been proposed.