[Mucinous nevus of late onset]. / Nævus mucineux de révélation tardive.

INTRODUCTION: Mucinous nevus is a rare disease characterized by mucin deposits in the dermis, with only 23 cases reported to date. It belongs to the connective tissue nevus family and is characterized by dermal mucin deposits. Two histological subtypes have been described: pure mucinous dermal nevus (connective tissue nevus of the proteoglycan [CTNP]) and combined mucinous nevus (combined CTNP) associating epidermal nevus with dermal mucin deposits. Lesions generally appear in childhood or early adulthood. We report herein the case of a 59-year-old man with a symptomatic lesion. OBSERVATION: A 59-year-old man consulted for unilateral lesions occurring linearly along the Blaschko line on the right lower limb, composed of brown verrucous papules, which started at the age of 50. The patient complained of itching caused by friction from clothes. Histological examination of a papule highlighted an acanthotic epidermis, with elongation of rete ridges and mucin deposits in the dermis showing uptake of alcian blue stain. The clinicopathological analysis enabled diagnosis of combined CTNP. We treated this nevus by surgical dermabrasion and observed a slight and cosmetically acceptable scar. DISCUSSION: To our knowledge, our patient is the oldest reported to date and the only one to have complained of symptoms. Our review of the literature highlights the importance of skin biopsy to guide aesthetic or symptomatic treatment. CO2 laser and dermabrasion are recommended for combined CTNP as they treat the epidermal component with low risk of secondary scarring. However, these techniques should be avoided in pure dermal CTNP, where surgical excision alone is acceptable in order to achieve optimal cosmetic results.

[Livedo-like cutaneous sarcoidosis]. / Sarcoïdose cutanée à type de livédo.

BACKGROUND: The cutaneous signs of sarcoidosis are extremely polymorphous and may be classified under several different headings. PATIENTS AND METHODS: Herein, we report the case of a 51-year-old female patient presenting bilateral livedo of the knees accompanied by systemic signs with polyarthralgia, impaired general state of health, weight loss, and a sensation of dyspnoea. Skin biopsy revealed giant-cell granuloma around the dermal vessels, with no caseous necrosis. Histopathological examination of the patient's blood vessels revealed no abnormalities. Laboratory tests showed high levels of angiotensin converting enzyme (1.5×ULN), bilateral mediastinal adenomegaly and incipient pulmonary fibrosis. Systemic sarcoidosis was diagnosed on the basis of the overall clinical and laboratory picture. The patient subsequently presented hepatic involvement and hypercalcaemia associated with the sarcoidosis; systemic corticosteroid therapy (prednisone) was initiated, with slow dose-reduction. The outcome was favourable with a return to normal laboratory values, regression of the adenomegaly and subsidence of the livedo. DISCUSSION: The literature contains reports of a number of cases of livedo heralding sarcoidosis. The majority of such cases involved young or middle-aged female patients of Asian origin presenting systemic sarcoidosis, with a high frequency of ocular and neural involvement. Livedo could be accounted for by the specific disposition of granulomas around the arterioles, resulting in disturbance of local blood flow, which was probably associated with the mechanical compression of vessels and with the micro-thrombi noted at histology. CONCLUSION: Livedo may be considered a clinical sign of cutaneous sarcoidosis. Laboratory tests to screen for sarcoidosis may be proposed in the exploration of atypical livedo.

[Adult kwashiorkor: a rare complication of bariatric surgery]. / Kwashiorkor chez l'adulte : une complication rare de la chirurgie bariatrique.

INTRODUCTION: Kwashiorkor is a clinical manifestation of global protein-calorie malnutrition that is described mainly in children in developing countries initially presenting eczema-like skin disease associated with hydrops. OBSERVATION: We report the case of a 44-year-old woman with dark skin who had undergone bariatric surgery for treatment of stage-III obesity without regular monitoring and who was hospitalized in our dermatology department four years later. She had diffuse oedema and asteatotic eczema-like cutaneous lesions on pigmented skin that had developed for two months despite daily application of topical corticosteroids. Clinical signs, the collapse of serum albumin and histopathological analysis of skin tissue were evocative of kwashiorkor. Her symptoms subsided after several weeks of a nutritional programme. DISCUSSION: Kwashiorkor is a clinical entity described chiefly in children in developing countries. Cutaneous involvement comprising asteatotic eczema-like skin associated with diffuse oedema is prominent. Treatment with protein supplementation alone is effective and avoids many complications such as infections and haemodynamic disorders. Obesity is a serious and common disease in Western countries affecting one third of the world's population, and for which treatment with bariatric surgery may be indicated. Without regular monitoring, this surgery can cause severe protein deficiency. Consequently, in the future there will seemingly be a greater likelihood of kwashiorkor in adults as an iatrogenic complication of obesity treatment. Its semiology needs to be underlined because of the simplicity and efficacy of treatment.

[Dermato-neuro syndrome during scleromyxedema: efficacy of plasmapheresis and intravenous immunoglobulin]. / Dermatoneuro syndrome au cours d'un scléromyxœdème: efficacité des plasmaphérèses et des immunoglobulines intraveineuses.

BACKGROUND: Dermato-neuro syndrome is a specific neurological complication of scleromyxedema presenting with fever, coma, seizures and flu-like syndrome. To our knowledge, it has only been reported about twenty times in the literature. Its outcome is uncertain. We describe the case of a patient in whom a favorable outcome was achieved using a combination of plasmapheresis and intravenous immunoglobulin (IVIG). PATIENTS AND METHODS: A 57-year-old woman was diagnosed 14 years ago with scleromyxedema resistant to multiple lines of treatment. In November 2011, she presented an initial episode of epileptic seizure followed by post-seizure coma, and later, confusional state with visual hallucinations. She recovered spontaneously within a few days. CT scan, MRI, EEG and screening for infection were perfectly normal, resulting in suspicion of neurological involvement associated with her scleromyxedema. In December 2012 and August 2013, she presented two further episodes of status epilepticus, followed once more by a confusional state, with etiological explorations again proving unfruitful. On this occasion, her confusional state persisted for two months until the initiation of plasmapheresis and IVIG. This combination therapy led to rapid regression of all neurological symptoms and an improvement in her general condition. DISCUSSION: The dermato-neuro syndrome is a rare neurological complication of scleromyxedema. Its pathophysiology is unknown. The monoclonal gammopathy induced by the scleromyxedema could account for the patient's hypercoagulable state and for the formation of neutrophilic aggregates leading to impaired microcirculation. Treatment is empirical and poorly codified. The course of the disease is unpredictable and may be lethal.

[Evaluation of hypnosis in pain management during photodynamic therapy: a pilot study]. / Évaluation de l'hypnose à visée antalgique dans la photothérapie dynamique : étude pilote.

BACKGROUND: Pain is the main adverse effect of photodynamic therapy (PDT) and few effective analgesic methods are currently available. Our aim was to evaluate the efficacy of hypnoanalgesia with the use of PDT. PATIENTS AND METHODS: Between August 2011 and February 2013, a hypnoanalgesia session was proposed to patients requiring PTD for the treatment of (pre)carcinomatous lesions. At the end of the hypnosis session, patients evaluated their pain on a numeric pain scale (NPS) of 0 to 10. RESULTS: Twelve patients of average age 74.6 years were included. The indication for PDT was actinic keratosis (AK) in 9 patients, 1 Bowen's disease of the penis, 1 mammary Paget's disease and 1 bowenoid papulosis of the penis. Hypnoanalgesia was effective in 8 patients with a mean pain evaluation score of 2.9/10 on the NPS. Six of these 8 patients had previously undergone treatment by PDT without hypnosis and with an average pain score of 8.3/10. DISCUSSION: Hypnoanalgesia appears to be of value for pain management with PTD. This method is simple, inexpensive and devoid of side effects, and it is active on both pain and anxiety. To improve the use of hypnoanalgesia in PDT, it would be necessary to have better knowledge of the predictive factors for pain in PDT, to determine how to best select patients "sensitive" to hypnosis, and to encourage the training of nurses and doctors in this method.

[Endogenous endophthalmitis as a complication in erysipelas]. / Endophtalmie endogène compliquant un érysipèle.

BACKGROUND: Endogenous endophthalmitis is a devastating infection of the eye that leads to blindness in about two-thirds of patients. It results from the haematogenous spread of a microorganism from a focus of sepsis, mainly gastro-intestinal, genitourinary or cardiac. PATIENTS AND METHODS: We describe the case of a diabetic subject presenting endogenous endophthalmitis following erysipelas of the leg due to Streptococcus agalactiae. The outcome was favourable thanks to prompt initiation of appropriate antibiotic treatment. DISCUSSION: Endogenous endophthalmitis as a complication of a skin infection is a rare entity, with only about 30 reported cases in the literature. Awareness of this condition among dermatologists would allow prompt intervention, which is essential for sparing of the patient's eyesight.

[An uncommon cutaneous presentation of cat scratch disease]. / Présentation cutanée atypique d'une maladie des griffes du chat.

BACKGROUND: Herein we report a case of cat scratch disease on account of its atypical presentation. PATIENTS AND METHODS: A 21-year-old woman presented erythema nodosum associated with painful bilateral inguinal adenopathy, odynophagia, joint pain and evening urticaria in a setting of impaired general condition. Initial serological testing for Bartonella henselae was negative. PCR for Bartonella henselae performed on an adenectomy fragment was positive. A favourable outcome was achieved with azithromycin. COMMENTS: This case shows an atypical and severe presentation of cat scratch disease and raises the problem of sensitivity of serotyping.

[Graves' dermopathy on the big toe]. / Dermopathie basedowienne localisée aux hallux.

BACKGROUND: Localized myxoedema is a rare dermopathy in patients with Graves' disease. The pretibial area is the most commonly affected region but herein we present a case of myxoedema of the big toe. PATIENTS AND METHODS: A 44-year-old male with Graves' disease ongoing for seven years presented bilateral ophthalmopathy and myxoedema of the big toes. The myxoedema was treated successfully with intralesional steroids. DISCUSSION: The physiopathology of myxoedema involves fibroblast activation and glycosaminoglycan production. This activation could result from stimulation of TSH receptors at their surface by TSH receptor antibodies (TRAK) or from an inflammatory process. The pretibial topography may be related to the high frequency in this area of microtrauma, with modulation of the cytokine microenvironment. CONCLUSION: The atypical localization seems to correlate with a Koebner phenomenon. Treatment of Graves' disease is generally insufficient to resolve the cutaneous problems. Topical corticosteroid therapy generally results in rapid improvement of recent lesions.