[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.

[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.

[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.

[Spontaneous cholecystocutaneous fistula]. / Fistule cholécysto-cutanée spontanée.

INTRODUCTION: Spontaneous cholecystocutaneous fistula is now a rare entity due to the advent of antibiotics, ultrasonography, and safe and early surgical treatment of biliary tract diseases. Such a case is reporting here, revealed by the systematic histological examination of the skin biopsy. OBSERVATION: A 65 year-old male presented with an inflammatory and ulcerated lesion located on his right flank, with a long-standing but asymptomatic course. Biological tests and biliary tract ultrasonography were not very contributive. Histopathological findings consisted in a granulomatous dermal reaction enclosing biliary fragments. Per-operative data were in favour of a compound biliocutaneous fistula complicating an inflammatory process of the gall-bladder. DISCUSSION: Spontaneous cholecystocutaneous fistula is unusual. Diagnosis might be difficult because of the lack of clinical specificity and a occasionally insidious evolution. Consequently, systemic histological examination is fundamental.

[Cutaneous Scedosporium infection revealed by bullous-necrotic purpura]. / Scédosporiose cutanée révélée par un purpura bullo-nécrotique.

BACKGROUND: Non-mycetomatous cutaneous scedosprium is an uncommon mycosis observed in immunodepressed subjects. We report a case with an inaugural presentation of bullous and necrotic purpura. CASE REPORT: A 79-year-old man on intermittent corticosteroid therapy for bronchospasm was admitted for bullous and necrotic purpura and fever. Subcutaneous nodules with a sporotrichoid aspect developed despite wide-spectrum antibiotics. Microbiology samples cultured on Sabouraud medium evidenced Scedosporium apiospermum. The pathogenic nature of the infection was proven on a skin biopsy showing numerous myceleal filaments with Gomori-Grocott staining despite negative PAS. No pulmonary involvement was evidenced. The patient was treated unsuccessfully with itraconazole. A Pseudomonas lung infection was fatal. DISCUSSION: Scedosporium apiospermum, an ubiquitous ascomycetes anamorphous to Pseudallescheria boydii, is the cause of a growing number of human infections due to widespread use of immunosuppressors. Skin and lung localizations predominate. The inaugural bullous and necrotic purpural skin manifestations in this case are unusual. In addition, the patient was only minimally immunodepressed and despite demonstrated in vitro sensitivity, itraconazole was ineffective clinically. Treatment is not well defined, but surgery is essential in combination with empirically chosen antifungals.

Keratinocyte-lymphocyte interaction in cutaneous T-cell lymphoma. Modulation of keratinocyte antigen My7 by a soluble factor produced by T lymphocytes.

OBJECTIVE: To test the hypothesis that the modulation of My7 antigen in the basal keratinocytes is directly related to the effect of dermal lymphocyte infiltrate of epidermotropic cutaneous T-cell lymphoma (CTCL). DESIGN: In vitro study with reconstituted skin model. SETTING: Department of Dermatology of University Hospital, Nantes, France. PATIENTS: Lymphocytes extracted from 11 skin samples with lesions of epidermotropic CTCL (mycosis fungoides, stages IIa to IV) and 6 skin samples with lesions of atopic dermatitis (control population) together with the supernatants of these infiltrating lymphocytes were incubated with normal reconstituted skin samples either alone or in the presence of interferon alfa-2a (10(2) IU/ mL). Moreover, normal peripheral blood mononuclear cells of 7 patients and 4 controls were incubated with reconstituted skin. INTERVENTION: None MAIN OUTCOME MEASURES: None. RESULTS: Ten of 11 samples of lymphocytes extracted from CTCL and 7 of 11 of their supernatants inhibited partially or completely My7 expression by basal cells. NO inhibition was noted for lymphocytes extracted from inflammatory skin or their supernatants. Addition of interferon alfa-2a in a culture medium of extracted lymphocytes or their supernatants blocked inhibition of My7 expression by keratinocytes in 8 of 10 reconstituted skin samples. No abrogation of My7 expression was noted with peripheral mononuclear cells. CONCLUSIONS: Our in vitro study demonstrated a direct and specific interaction between the tumor infiltrate of CTCL and keratinocytes. Moreover, this interaction appeared to be closely associated with a soluble factor produced by the tumor T-cell infiltrate and was at least partially blocked by interferon alfa-2a.

Long-term interferon-alpha therapy induces autoantibodies against epidermis.

BACKGROUND: While interferon (IFN) is known for its immunoregulatory properties, it has also been shown to induce autoimmune disorders such as lupus erythematosus, hypothyroidism, antiphospholipid syndrome and, recently, bullous pemphigus-like eruptions. OBJECTIVE: The purpose of this study was to determine the percentage of antibodies against epidermis induced by IFN-alpha therapy, as detected by indirect immunofluorescence and Western blotting (WB). METHOD: We have studied the sera of 47 patients treated with low doses of IFN-alpha 2a for malignant melanoma or cutaneous T cell lymphoma for a period of 12 months. These sera were tested by standard IIF and WB. RESULTS: 32% of patient sera were positive after 6 months of treatment. Antibodies against epidermis were most often of the pemphigus type as confirmed by WB. Two types of labeling were noted for anti-basement membrane antibodies: basal cytoplasmic pattern in 4 sera and along the dermal side of the basal keratinocytes in 2 sera. CONCLUSION: This study brings biological arguments to confirm the direct role of IFN-alpha in the development of pemphigus-like eruption, and emphasizes the need for clinical follow-up of patients treated with IFN-alpha over a long period of time.