[Port-wine stains treated by pulsed-dye laser with sedation: A retrospective observational efficacy and safety in 18 children]. / Étude observationnelle rétrospective du traitement des angiomes plans par laser colorant pulsé sous sédation : efficacité et tolérance chez 18 enfants.

BACKGROUND: Pulsed-dye laser (PDL) is the gold standard treatment for port-wine stains but it is painful. To limit pain in small children, sedation may be given. OBJECTIVE: We sought to determine the efficacy of this approach and the risks of sedation, as well as the level of satisfaction of parents. PATIENTS AND METHODS: A retrospective study was conducted in our dermatology department in children treated with PDL while under sedation. The efficacy of treatment was evaluated by comparing pictures of lesions before and after treatment and using a questionnaire sent to the parents. RESULTS: 18 children were included between 2003 and 2011. In all, 111 laser sessions were performed with sedation. Comparison of photographs showed an improvement rate of 6.2/10 (in terms of colour and area). The mean parent satisfaction score was 6.6/10. Adverse events were reported in 27.8 % of children and for 4.5 % of PDL sessions, with one case of severe hypoxemia that resolved without sequelae. DISCUSSION: In our study, PDL for port-wine stains was effective, with good improvement of lesions. Pain was controlled thanks to sedation with one serious adverse event. The parents indicated a positive impression. This study suggests that the procedure may be proposed for small children in order to reduce pain, with a good risk-benefit ratio for sedation. More studies are needed to better qualify pain management for children under PDL treatment.

[An atypical skin rash in a setting of antiphospholipid syndrome]. / Éruption cutanée atypique au cours d'un syndrome des antiphospholipides.

BACKGROUND: Antiphospholipid syndrome (APS) is characterised by arterial or venous thrombosis combined with the presence of specific antibodies known as antiphospholipids. It is commonly associated with cutaneous signs. Herein we report a case of atypical cutaneous eruption occurring during the course of APS and we discuss the possible mechanism. PATIENTS AND METHODS: A 45-year-old woman consulted twice within six months for an erythematosus papular eruption around the neckline. She was being followed-up for antiphospholipid syndrome treated with fluindione (Préviscan®), and her International Normalized Ratio (INR) was consistent with the prescribed anticoagulation target. Blood tests confirmed the presence of anticardiolipin and antiphospholipid antibodies, but no laboratory evidence of lupus was seen. Histopathological examination of a skin biopsy demonstrated the presence within the vascular lumen of a weakly eosinophilic anhistic substance positive for PAS stain. The patient was given acetone salicylic acid (Kardégic®) combined with fluindione, and four years later, she had presented no relapses. DISCUSSION: Despite a clinically evocative appearance, the diagnosis of lupus tumidus was ruled out in our patient by histopathological features, and associated systemic lupus erythematosus was repeatedly refuted on the basis of clinical and laboratory data. We suggest that the specific histological images of intraluminal deposits within the dermal vessels seen in this patient, although not typical of thrombosis, are associated with APS. The clinical remission seen from the start of antiplatelet treatment could be due to the action of these drugs against the obstruction of small-calibre dermal vessels.

[Bullous drug eruption with Nigella sativa oil]. / Éruption bulleuse à l'huile de nigelle.

BACKGROUND: Nigella sativa is classically used for its various therapeutic properties, particularly in the field of allergic diseases. We report a case of bullous eruption following application of Nigella sativa oil to the skin, possibly involving an associated systemic mechanism linked to ingestion of the oil. PATIENTS AND METHODS: A 53-year-old woman was hospitalized for febrile rash consisting of erythematous plaques with vesicles and bullous lesions. She had Nigella sativa oil applied to her skin and ingested it for two weeks. Cutaneous histopathology revealed sub-epidermal detachment and necrosis of the epidermal surface consistent with toxic epidermal necrolysis. The lesions healed slowly, but depigmented macules persisted three months later. Patch tests for Nigella sativa oil were strongly positive. DISCUSSION: Erythema multiforme due to contact with Nigella sativa oil was initially diagnosed on the basis of lesions on skin where the oil was applied, as well as on cutaneous histopathology and positive patch tests. However, a systemic reaction such as toxic epidermal necrolysis could not be ruled out since the patient had also ingested the oil. The most likely diagnosis was thus systemic and contact bullous drug eruption. The discovery of new therapeutic properties of Nigella sativa in other clinical domains underscores the need for particular vigilance regarding future use of this substance on account of the risk of severe drug eruptions.

[Syringotropic cutaneous T-cell lymphoma mimicking dermatomycosis]. / Lymphome cutané T syringotrope simulant une dermatomycose.

INTRODUCTION: Cutaneous syringotropic T-cell lymphoma is a rare form of lymphoma. We report a case involving a misleading cutaneous presentation on the sole of the foot. PATIENTS AND METHODS: A 55-year-old woman presented discrete coalescent papules on her left foot, having an anhidrotic appearance, for which a number of antifungal treatments had been given without success. The skin biopsy revealed CD4+ T lymphocytic dermal infiltrate, mainly near the sweat glands, with syringotropism. The diagnosis of syringotropic T-cell lymphoma was reinforced by the presence of dominant cutaneous T-lymphocyte clone in the skin biopsy. Staging tests were negative. Treatment was initiated with an extremely potent (class IV) dermal corticosteroid. DISCUSSION: Syringotropic T-cell lymphoma is an extremely rare form of cutaneous lymphoma similar in presentation to mycosis fungoides, characterised by the mainly perisudoral and syringotropic nature of the lymphocytic infiltrate. The value of this case report lies in the extremely mild nature of the misleading skin lesions, which could only be diagnosed through biopsy. Treatment for this condition is not as yet codified due to the extremely low number of cases reported in the literature.

Lupus attributable to anti-TNF therapy and revealed by interstitial granulomatous dermatitis.

Interstitial granulomatous dermatitis belongs to the group of aseptic cutaneous granulomas. It is a histopathological entity encountered in various pathological situations, such as polyarthritis including rheumatoid arthritis, but also systemic lupus erythematosus. It may also occur after systemic administration of medication, thus representing a drug-induced, interstitial granulomatous outbreak. This has recently been described after anti-TNF therapy was taken. We are reporting the case of a patient treated using adalimumab for rheumatoid arthritis and having developed interstitial granulomatous dermatitis during treatment, which revealed lupus erythematosus attributable to the biotherapy. The clinical appearance of interstitial granulomatous dermatitis can vary, and the diagnosis is confirmed by anatomo-pathological examination. Drug-induced interstitial granulomatous outbreaks have specific histological criteria, and secondary cases involving anti-TNF medication have been described. Cases of lupus attributable to anti-TNF therapy have also been described, and they have specific biological characteristics. Like idiopathic lupus, they may be associated with interstitial granulomatous dermatitis, but the association of an anti-TNF-induced lupus and this type of granulomatous has not, to our knowledge, been described before. We are reporting one case, which emphasises the importance of carrying out a complete and systematic aetiological assessment for all cases of interstitial granulomatous dermatitis, including where there is systemic disease or following medical treatment, either of which may provide an evident cause for the granulomatosis. In particular, the outbreak of interstitial granulomatous dermatitis during anti-TNF treatment should lead to screening for a drug-induced lupus, which would require the patient to stop such treatment.

[Multiple cutaneous osteomas of the face in a setting of chronic acne]. / Ostéomes cutanés multiples de la face dans un contexte d'acné chronique.

BACKGROUND: Multiple cutaneous osteomas are a rare complication of chronic inflammatory acne that often goes unrecognized. We report a case concerning a 35-year-old woman. PATIENTS AND METHODS: A 35-year-old woman had been treated for acne since the age of 22 years, as part of which she received two courses of oral isotretinoin. We noted the secondary appearance of several microcysts on the face for which the excision was very difficult. Curiously, these small formations did not contain keratin but were very callous. Histological examination revealed foci of osseous metaplasia, probably of postinflammatory origin. Treatment consisted solely of excision of the lesions. DISCUSSION: Osteoma cutis comprises two distinct groups (primary and secondary). In our case, there were multiple cutaneous osteomas of the face resulting from chronic acne. The differential diagnosis was idiopathic miliary osteomatosis of the face, but this was ruled out by the young age of the patient, the improvement of the acneiform lesions under isotretinoin (confirming the initial diagnosis of acne) and the subsequent appearance of microcysts. Although there are as yet no codified treatments, excision appears to yield good results.

[Therapeutic value of moderate-dose oxybutynin in extensive hyperhidrosis]. / Intérêt de l'oxybutynine à dose modérée dans l'hyperhidrose étendue.

BACKGROUND: Hyperhidrosis is a functional disorder characterized by increased sweating that can greatly impair quality of life due to psychosocial factors. It is most often localized (underarms, hands, feet) but can also be generalized. In this case, after eliminating an underlying cause, systemic treatment may be proposed. In this retrospective study, we report the benefits and adverse effects of the anticholinergic drug oxybutynin. PATIENTS AND METHODS: All patients treated with oxybutynin between February 2008 and October 2010 for troublesome generalized hyperhidrosis were recruited after ruling out underlying disease. Oxybutynin (Ditropan(®)) was started at a dose of 1.25mg then increased in increments of 1.25mg every 4 days up to the lowest effective dose, which did not exceed 2.5mg three times per day in order to avoid serious adverse effects. After 2 months of treatment, patients were asked to rate their satisfaction on a scale of 0 to 10, as well as degree of efficacy, time to efficacy, type of adverse effects and whether or not treatment was continued. RESULTS: Thirty patients were included in this study: 24 women and 6 men aged 19 to 64 years (mean=33 years). The treatment was rated by 24 patients (80%) as very effective and by three patients (10%) as moderately effective. Three patients (10%) stopped the treatment due to adverse effects such as faintness and ocular accommodation disorders, and for these patients, the degree of satisfaction ranged from 1 to 3/10. For the other 27 patients, satisfaction was 6 to 10 with a median of 8.5. The onset of action was between 10 and 21 days with an average of 15.4 days. Adverse effects were present in 10 of 27 patients (37%) comprising dry mouth, which was well tolerated (grade 1). Mean follow-up was 14.9 months (2 months to 32 months), with two patients being lost to follow-up. DISCUSSION: The treatment of primary extended or generalized hyperhidrosis is not currently codified. Our study shows the efficacy of oxybutynin in this indication at a moderate dose, with good safety. The satisfaction index was high, with 80% of patients considering the drug very effective and for 10% considering it moderately effective. To our knowledge, this is the first retrospective study in a large population. A placebo-controlled study could provide the grounds for a therapeutic consensus concerning a drug widely known for many years in the treatment of extensive hyperhidrosis.

[Annular elastolytic giant-cell granuloma]. / Granulome annulaire élastolytique à cellules géantes.

BACKGROUND: Annular elastolytic giant-cell granuloma (AEGCG) is a rare form of granulomatous dermatosis characterized by annular plaques with central atrophy and raised erythematous margins and is usually located on the facial and neck areas. It is characterized histologically by loss of elastic fibre and elastophagocytosis. We report a case of AEGCG. CASE REPORT: A 72-year-old man consulted for annular plaques, some of which were atrophic, and papules that had been present for 2 years. The lesions involved sun-exposed and non-sun-exposed skin. The biopsies showed granulomatous infiltrates and discrete elastophagocytosis. After ruling out various differential clinical and histological diagnoses, the patient was diagnosed with AEGCG. DISCUSSION: We report a case of AEGCG. Diagnosis was not easy. The differential diagnoses of this entity were discussed and we ruled out actinic granuloma, sarcoidosis, leprosy, and granuloma annulare. Our patient presented the classical annular variant combined with a papular variant. We report the first case involving response to isotretinoin.

[Efficacy and risks of topical 5-fluorouracil in Darier's disease]. / Traitement de la maladie de Darier par 5-fluorouracile topique: quelle efficacité, quels risques?

BACKGROUND: Darier's disease, an autosomal dominant disorder, is often resistant to therapy. There have been few encouraging reports in recent years of treatment using topical 5-fluorouracil (5FU) in this indication. We describe three cases of Darier's disease treated with Efudix: though it initially proved very effective, after several months this treatment became inefficacious. PATIENTS AND METHODS: Case 1: a 16-year-old girl with Darier's disease refractory to conventional treatments. Treatment with topical 5FU was initiated. After 3 weeks, her cutaneous lesions had practically disappeared, leaving only pigmented scars. At 6 months, worsening of the disease was controlled by increasing 5FU. At 11 months, the disease worsened and 5FU, which proved inefficient, was stopped. Case 2: a 59-year-old woman presenting treatment-resistant Darier's disease was given 5FU. After 1 week, improvement began and was evident at 4 months. However, after 13 months of treatment, the disease progressed, and increased 5FU proved completely ineffective. Case 3: a 29-year-old woman was hospitalized for inflammatory Darier's disease. Topical 5FU was applied to the left half of the body and a short course of oral corticosteroids was prescribed. After 10 days of treatment, there was a clear improvement. After 6 weeks, the patient showed episodes of healthy skin. However after 3 months, the patient stopped taking her treatment due to inefficacy. DISCUSSION: While topical 5FU seems to be effective initially in treating Darier's disease, this efficacy subsides over time. Although no adverse effects were noted in our patients, use of 5FU can result in serious adverse reactions. Because of the loss of efficacy of this treatment following initial success, coupled with its poorly evaluated safety, caution must be exercised when prescribing it, particularly in patients with dihydropyriminidine dehydrogenase (DPD) deficiency or for use on damaged skin.

Management and outcome of metastatic melanoma during pregnancy.

BACKGROUND: Although metastatic melanoma occurrence during pregnancy challenges the physician in several ways, only a few studies have been published. OBJECTIVES: Our aim was to investigate therapeutic management together with maternal and fetal outcomes in pregnant women with advanced melanoma. METHODS: A French national retrospective study was conducted in 34 departments of Dermatology or Oncology. All patients with American Joint Committee on Cancer (AJCC) stage III/IV melanoma diagnosed during pregnancy were included. Data regarding melanoma history, pregnancy, treatment, delivery, maternal and infant outcomes were collected. RESULTS: Twenty-two women were included: 10 AJCC stage III and 12 stage IV. Abortion was performed in three patients. Therapeutic abstention during pregnancy was observed in three cases, 14 patients underwent surgery, four patients received chemotherapy and one patient was treated with brain radiotherapy alone. The median gestational age was 36 weeks amenorrhoea. Neither neonatal metastases nor deformities were observed. Placenta metastases were found in one case. Among 18 newborns, 17 are currently alive (median follow up, 17 months); one died of sudden infant death. The 2-year maternal survival rates were 56% (stage III) and 17% (stage IV). CONCLUSIONS: Faced with metastatic melanoma, a majority of women chose to continue with pregnancy, giving birth, based on our samples, to healthy, frequently premature infants. Except during the first trimester of pregnancy, conventional melanoma treatment was applied. No serious side effect was reported, except one case of miscarriage after surgery. Mortality rates do not suggest a worsened prognosis due to pregnancy but larger prospective controlled studies are necessary to assess this specific point.

[Congenital histiocytoma: a benign disease]. / Histiocytome congénital: une lésion bénigne.

BACKGROUND: We report on a newborn presenting a solitary congenital skin nodule due to Langerhans cell histiocytosis. This benign lesion is rare and has been described in the literature under a variety of names; there is no consensus regarding treatment. CASE REPORT: A 28-day-old newborn presented with a solitary congenital blue-brown nodule measuring 1cm on the left iliac fossa. Histological examination of a skin biopsy showed a proliferation of histiocytes throughout the dermis with an immunohistochemical profile of Langerhans cells. The lesion resolved spontaneously, with rapid reduction of the infiltration, and at 16 weeks only a pigmented scar remained. DISCUSSION: Thirty-eight similar cases have been reported in the literature under a variety of different names. The lesions noted consisted of a solitary brownish nodule measuring 1cm which was congenital in almost all cases, often ulcerated and exhibited no predilection for any particular body site. Histological examination revealed dermal proliferation of Langerhans cells. Electronic microscopy revealed dense intracytoplasmic bodies and Birbeck's granules. Laboratory and radiological tests did not show systemic involvement in any cases. All lesions other than those surgically removed regressed spontaneously within a mean 8 weeks. It appears necessary to differentiate between congenital and other forms of histiocytoma since therapeutic strategies differ. Spontaneous resolution of lesions and lack of systemic involvement militate in favour of simple clinical follow-up, with surgical excision and further tests required only for cases lasting 6 months and beyond.

[Bursitis due to Mycobacterium intracellulare in an immunocompetent patient]. / Bursite fistulisée à Mycobacterium intracellulare chez un adulte immunocompétent.

BACKGROUND: Cutaneous infections due to Mycobacterium intracellulare are infrequent. We report a case of bursitis in an immunocompetent patient. CASE-REPORT: A 80-year-old patient with a history of tuberculosis reported swelling of the elbow for six months with effusion due to olecranon bursitis, progressing to an abscess and skin ulceration. Examination of the synovial fluid revealed the presence of M. intracellulare. Histologic investigations showed epithelioid and gigantocellular granulations. Screening for immunodepression was negative. Treatment with clarithromycin and rifabutin proved effective, with partial healing at three months, although hepatic and ocular side effects occurred. DISCUSSION: While osteomyelitis, arthritis and cutaneous infections due to M. intracellulare have been described in immunocompromised patients, there are very few reported cases concerning immunocompetent individuals, and these suggest the possibility of special individual sensitivity to mycobacterial infections. Genetic mutations of the interleukin-12-interferon-gamma pathway involved in the immune response to mycobacterial infections have been demonstrated. Autoantibodies against interferon-gamma have also been described. Mycobacterium avium-intracellulare is resistant to the classical antibiotics used in tuberculosis. As in pulmonary infections, antibiotics with proven in vivo and in vitro efficacy must be used, taking into account the antibiogram and potential side effects, particularly for combined clarithromycin and rifabutin. CONCLUSION: This case report emphasizes the benefits of routine mycobacterial investigation of synovial fluid samples.

[Efficacy of hypnosis in the treatment of palmar hyperhidrosis with botulinum toxin type A]. / Intérêt de l'hypnose dans les injections palmaires de toxine botulique A.

BACKGROUND: Injections of botulinum toxin type A are extremely effective in treating palmar hyperhidrosis. The main problem is pain during injections in the palms and pulp of the fingers. Anaesthesia of the hands using Emla and analgesia with an equimolar mixture of oxygen/nitrous oxide are occasionally disappointing. We analysed the value of hypnosis in reducing injection pain. PATIENTS AND METHODS: In a prospective study performed between September 2005 and September 2006, we offered a full session of hypnosis in order to relieve pain for patients presenting troublesome bilateral and palmar hyperhidrosis and requiring treatment by palmar injection of botulinum toxin. At the end of the hypnosis session, patients evaluated their pain on a scale of 0 to 10 and indicated whether or not the injections had been bearable. RESULTS: Of the 15 patients included, 13 underwent a hypnosis session. The mean pain scores following hypnosis were 2.52 and all patients considered their injections bearable. Six patients with a pain score of between 7 and 8 out of 10 during previous injections without analgesia or using Kalinox evaluated their pain as between 1 and 3 following hypnosis. DISCUSSION: Palmar injection of botulinum toxin is increasingly being used for hyperhidrosis patients. In dermatology, access to operating theatres or the presence of a trained anaesthetist is normally extremely difficult, and hypnosis appears to offer an extremely effective method of reducing the pain associated with such injections.

[Granuloma annulare of the eyelid in a child: an atypical localization]. / Granulome annulaire des paupières: une localisation atypique.

BACKGROUND: Granuloma annulare is a common form of dermatosis in children and young adults. Lesions are typically found on the hands, the feet and the extensor surfaces of the limbs, and occasionally on the trunk. We report a case original in terms of its palpebral localization. CASE-REPORT: A 5 year-old girl consulted for papular lesions on the eyelids. The clinical examination revealed papules on the right lower eyelid measuring 8 mm, on the left lower eyelid measuring 5 mm and on the right upper eyelid measuring 3 mm. Laboratory tests including serum glucose, lipids and calcium as well as a complete blood count proved normal. Biopsy showed granulomatous lesions: a region of central necrosis surrounded by a palisade of inflammatory cells confirmed the diagnosis of granuloma annulare. The lesions disappeared in a few weeks without treatment. DISCUSSION: To our knowledge, 44 cases of granuloma annulare of the periorbital area have been reported, of which 19 concerned children. This presentation represents an atypical localization of granuloma annulare which must not be confused with many other palpebral disorders. Biopsy is necessary to confirm the diagnosis.

[Cutaneous calciphylaxis treated by autologous keratinocytes graft and subtotal parathyroidectomy]. / Nécrose cutanée par calciphylaxie traitée par greffe de kératinocytes autologues et parathyroïdectomie partielle.

BACKGROUND: Cutaneous calciphylaxis, seen most often in dialysis patients, is characterised by skin necrosis, and is a disabling and lifethreatening disease. Despite intensive topical treatment, recourse to parathyroidectomy is often necessary. We report the case of a female patient with skin necrosis due to calciphylaxis: pain and necrotic lesions were controlled by grafting of cultured autologous keratinocytes (Epibase). CASE REPORT: A 75-year-old woman with a 5-year history of dialysis-dependent chronic renal failure secondary to nephroangiosclerosis presented a very painful necrotic ulceration on her left leg. In spite of an autologous patch grafts, the lesions rapidly deteriorated. Laboratory data showed high levels of calcium, phosphate and parathyroid hormone and imaging suggested parathyroidal adenoma. Although cutaneous biopsy was not performed, the diagnosis of cutaneous necrosis due to calciphylaxis with tertiary hyperparathyroidism was established. Treatment consisted of a low-calcium and low-phosphorus diet with autologous keratinocytes grafts (Epibase). After the third application of keratinocytes, the pain disappeared, necrosis ceased and wound healing began. Subtotal parathyroidectomy was performed two months after the start of grafts. At three months, the patient was cured. DISCUSSION: . Calciphylaxis is an obstructive vascular disease secondary to calcification of the arterioles leading to ischemic tissue necrosis. Prompt diagnosis is essential since this disease is disabling and life-threatening due to sepsis and ischemic complications. In our case, autologous keratinocyte grafts allowed pain relief to be achieved within large expanses of cutaneous necrosis after correction of calcium and phosphorus levels, thereby allowing parathyroidectomy to be performed under optimal conditions.