[Paraneoplastic pemphigus: Retrospective study of a case series]. / Étude rétrospective d'une série de pemphigus paranéoplasiques.

CONTEXT: Paraneoplastic pemphigus (PNP) is a rare condition associated with poor prognosis. It associates polymorphic mucocutaneous manifestations with neoplasia. Diagnosis is difficult because of the various clinical and histological features involved and the lack of specificity of immunological examinations. METHODS: We retrospectively analyzed the records of patients presenting with PNP in the Poitou-Charentes region between 2000 and 2015. RESULTS: Seven patients were included. They presented 9 neoplasias (1 lymphoma, 1 melanoma, and 7 carcinomas) diagnosed from 4 months before to 25 months after the occurrence of cutaneous (6/7) and/or mucosal (6/7) polymorphic lesions. Histological examination revealed epidermal acantholysis (7/7), keratinocytic necrosis (4/7), and interface lichenoid dermatitis (5/7). Intercellular deposits of IgG and C3 or along the dermo-epidermal junction were detected with direct immunofluorescence (IF) (7/7). Four of 6 patients tested had positive indirect IF on rat bladder epithelium. Follow-up ranged from 1-132 months with a one-year survival of 85.7%. DISCUSSION: The clinical and histopathological presentations observed in our patients were polymorphic, with overlap between the clinical and histological features of PNP and classical pemphigus. Prognosis and survival appear better in our series than in the literature. It is possible that in some cases, the association of pemphigus with neoplasia was fortuitous, which might account for the better prognosis. A new consensus on the diagnostic criteria for PNP is needed to help practitioners to consensually diagnose it for prognostic or therapeutic trials.

[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.

[Epidermolysa bullosa acquisita and graft-versus-host disease]. / Épidermolyse bulleuse acquise et réaction du greffon contre l'hôte.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare acquired blistering disorder caused by production of auto-antibody directed against type-VII collagen. Autoimmune disorders can occur after allogenic bone marrow transplantation as manifestations associated with chronic graft-versus-host disease (GVHD). To date, there have been 10 cases reported in the literature concerning autoimmune blistering diseases following allogenic stem-cell transplants. Herein we describe a new case involving EBA. OBSERVATION: A 46-year-old woman developed EBA 4 years after allogenic cord blood transplantation for non-Hodgkin T-cell lymphoma complicated by acute digestive and cutaneous GVHD. At physical examination, she had some cutaneous blisters on the abdomen, arms and face, as well as numerous erosions in the buccal cavity. Direct immunofluorescence microscopy revealed linear IgG and C3 deposits along the dermal-epidermal basement membrane zone. Indirect immunofluorescence showed weak IgG G4 anti-basement membrane zone antibodies, which reacted with the dermal side of 1M NaCl-split skin; the autoantibodies were directed against type-VII collagen. This second case of EBA was evocative of a GVHD blistering disease. After the therapeutic failure of dapsone and of combined mycophenolate-prednisone, treatment with rituximab proved effective. DISCUSSION: EBA may form part of the autoimmune signs associated with chronic GVH. The destruction of basement membrane and of epidermal basal cells that occurs in GVH could give rise to autoimmune bullous disease. However, in our patient, in whom manifestation of chronic GVH was restricted to the lungs, it is difficult to rule out the fortuitous onset of EBA, which presented at a sizeable interval after acute GVH.

[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.

Cutaneous melanoma in patients treated with tumour necrosis factor inhibitors: a retrospective series of 15 patients.

BACKGROUND: Several case reports suggested that tumour necrosis factor-α (TNF) inhibitors might increase the incidence and/or alter the natural course of melanoma towards a more aggressive behaviour. OBJECTIVE: Our objective was to point if history of melanoma in patients exposed to TNF inhibitors could present with a particular pattern at diagnosis or during follow-up. METHODS: We performed a retrospective multicentre study settled in the West part of France to collect and analyse all cases of patients with melanoma who received anti-TNF therapy. RESULTS: Fifteen cases were included. First, 10 patients (mean age: 55.6 years; sex ratio: 1) had a melanoma diagnosed after TNF inhibitors initiation. The mean duration between initiation of treatment and melanoma was 48.7 months. Two patients died of metastatic disease. Second, four patients had a past history of melanoma before anti-TNF therapy (mean duration of treatment: 10.8 months). None experienced a progression of melanoma disease. Last, one woman had a past history of melanoma before and then developed a second melanoma when exposed to biotherapy. CONCLUSION: Our case series does not reveal a distinct profile of melanoma in the patients exposed to TNF inhibitors. Additional prospective trials including larger number of patient are needed to demonstrate the possible link between biological therapy with TNF inhibitors and development of melanoma.

[Rose necrosis: Necrotizing granulomatous reaction with infected node at red pigment of a tattoo]. / La nécrose de la rose : réaction granulomateuse et nécrotique avec adénite au pigment rouge d'un tatouage.

INTRODUCTION: Nowadays, necrotizing cutaneous reaction after a tattoo is rare especially with the sterile tattoo equipment and antisepsis rules. We report the rare case of a necrotizing reaction secondary to a granulomatous reaction after a red tattoo, with a satellite node. CASE REPORT: A 40-year-old patient suffering from a granulomatous reaction to red dye of a large pectoral tattoo, with cutaneous and sub-cutaneous necrosis, and an infected axillary node. This pectoral tattoo also triggered a necrotizing granulomatous reaction on red-pigmented areas of other older tattoos. Local treatments (dressings, antibiotics, repeated excisions of necrotizing tissues) did not stop the allergic reaction, and an infectious origin was eliminated. The patient asked for a complete excision of the pectoral tattoo. Black intramacrophagic pigment was found in the black lymph node analysed. We did not experience any complications and the patient is satisfied with the results. DISCUSSION: Very few examples of cutaneous necrotizing secondary to a tattoo have been found in the literature. The hypothesis of a primitive infection that had secondarily led to necrosis is refuted by the lack of infective structures found in the analysed node, and most of all by the same reaction on other older tattoos on red-pigmented areas. This rare complication must be known by plastic surgeons, who will probably be called upon to take care of more and more tattooed patients. CONCLUSION: Even if it's rare, necrosis with a granulomatous reaction to red pigment after a tattoo must be known. This case illustrates a very violent immune reaction where infection was not proved.

[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.

[Gorlin syndrome: photodynamic therapy, as a useful adjunct to surgery]. / Syndrome de Gorlin : la photothérapie dynamique, technique complémentaire du traitement chirurgical.

Gorlin syndrome, also called nevoid basal cell carcinoma syndrome, is well known by dermatologists. Since its onset, 10 years ago, photodynamic therapy has found new applications and is now currently used to cure single or multiple basal cell carcinomas, with good results and without residual scars. We recall some of the basic principles of this technique, as well as its indications in Gorlin syndrome, which we illustrate with one case. Plastic surgeons must consider this relatively new technique, developed by dermatologists, as a useful adjunct to surgery in the management of Gorlin syndrome.

[Functional impairment and radiologic fasciitis under erlotinib therapy]. / Incapacité motrice et fasciite radiologique sous erlotinib.

BACKGROUND: Targeted molecules are recent and valuable weapons in the management of certain cancers. Among them, erlotinib is an inhibitor of epidermal growth factor receptor approved in non-small lung cancer and pancreatic cancer after failure of first line treatment. Erlotinib is responsible for many cutaneous side effects. We report a case of acute symptomatic fasciitis that has occurred during erlotinib therapy. To our knowledge it is the first case described. CASE REPORT: A 56-year-old man was treated with erlotinib for a metastatic non-small lung adenocarcinoma. Shortly after the treatment by erlotinib was introduced, he had a severe acneiform rash resistant to doxycycline treatment. After a year of treatment, he presented intense pain in the legs with functional impairment. Medical imaging confirmed fasciitis. It regressed along with the rash after using strong topical corticosteroids during ten days. DISCUSSION: Besides bacterial fasciitis, inflammatory and oedematous fasciitis have varied aetiologies. The occurrence of a documented fasciitis during anti EGFR-therapy is original and raises the question of underlying mechanism. We suggest three pathophysiological mechanisms: spreading by contiguity; paraneoplastic fasciitis, or specific lesion of the fascia due to anti-EGFR.

[Severe skin reaction with mucous membrane inflammation during MINE chemotherapy]. / Réaction cutanée sévère avec mucite au cours d'une chimiothérapie de type MINE.

BACKGROUND: MINE chemotherapy is used to treat refractory Hodgkin's disease. Cutaneous adverse effects of MINE regimen are uncommon and chiefly consist of erythema and oedema of the extremities. More recently, a number of cases of panniculitis and subcutaneous inflammatory oedema have been described. OBSERVATION: We report the case of a 17-year-old girl developing acute and painful oedema of the limbs with panniculitis of the trunk. This incident was associated with inflammatory lesions of mucous membrane, in particularly in the genital area and on the tongue. These signs occurred 7 days after initiation of MINE chemotherapy, with no other drugs being introduced. A drug-induced reaction was suspected due to the absence of any other aetiology, particularly infectious disease. The condition gradually improved with symptomatic pain therapy. The patient's chemotherapy was subsequently modified. DISCUSSION: The chronology of the symptoms, spontaneous improvement after the end of treatment, and the absence of other potential causative factors resulted in a hypothesis of a cutaneous adverse reaction to the MINE regimen. The signs could be due to capillary leak syndrome resulting from the toxicity of vinorelbine on endothelial cells. Dermatologists should be aware of these cutaneous adverse effects and of the inflammatory lesions of mucous membrane newly described herein.

[Autochthonous filaria in south-west France caused by Dirofilaria repens]. / Dirofilariose autochtone à Dirofilaria repens dans le Sud-Ouest de la France.

BACKGROUND: Dirofilariasis is an autochthonous disease caused by Dirofilaria (Nochtiella) repens, a subcutaneous parasite of dogs and other carnivorous animals. Human infection leads to a self-limiting disease that generally presents clinically as a subcutaneous nodule. We describe a case occurring outside the classical endemic Mediterranean region. PATIENTS AND METHODS: A 38-year-old woman presented with a scalp nodule present for six months. Surgery revealed a thread-like parasite identified as a female Dirofilaria repens without microfilariae. DISCUSSION: This case points to the spread of this disease to temperate areas beyond the regions classically affected.

[Primary cutaneous aspergillosis in an immunocompetent farmworker]. / Aspergillose cutanée primitive chez un agriculteur immunocompétent.

INTRODUCTION: Aspergillosis is an uncommon fungal infection in which primary cutaneous sites are very rare. Most cases occur in immunodepressed patients and are disseminated in the blood. We report a case of primary cutaneous aspergillosis in a 37-year-old immunocompetent farmworker presenting as a kerion-like lesion. CASE REPORT: A 37-year-old farmworker presented erythematous and nodular lesions of the face. These lesions were not associated with any general symptoms and failed to respond to antibiotic treatment. Histological examination of a skin biopsy sample showed a granulomatous reaction in the dermis associated with an extensive neutrophilic infiltrate. PAS staining revealed the presence of right-angled branched hyphae with conidia. Aspergillus fumigatus was isolated and identified in cultures. Clinical and biological examinations did not reveal any systemic localisation of aspergillosis, ruling out the hypothesis of blood dissemination. This primary cutaneous infection occurred in an immunocompetent patient, in whom laboratory tests ruled out any underlying immunosuppression. Systemic antifungal treatment with voriconazole led to complete resolution. DISCUSSION: Reports in the literature of primary cutaneous aspergillosis in immunocompetent patients are extremely rare. Clinical lesions may comprise often painful weeping or necrotic macules or papules or subcutaneous nodules that can progress towards abscess or necrosis. The semiological similarity between these lesions and kerions can lead to misdiagnosed cases and delayed treatment.

[Iatrogenic cutaneous emphysema]. / Emphysème cutané iatrogène.

BACKGROUND: Tracheal perforation following orotracheal intubation is a rare but classic complication of general anaesthesia, and marked cutaneous emphysema can occur. We report a rare case of facial cutaneous emphysema revealed by swelling with pseudovesicles on the eyelids. CASE REPORT: A 99-year-old woman developed extensive facial swelling with pseudo-vesicles on the upper and lower eyelids following right hip replacement surgery. Swelling of the upper members and thoracic area was also seen. Chest X-rays showed marked cutaneous emphysema of the thoracic wall. Bronchial endoscopy revealed perforation of the posterior aspect of the trachea. The patient was presenting cutaneous emphysema as a result of post-intubation tracheal perforation. DISCUSSION: We report a rare case of cutaneous emphysema in a 99-year-old woman after tracheal perforation following orotracheal intubation. The significant feature of our case report is the actual manifestation of the condition. Our patient developed swelling of the eyelid with pseudovesicles. When confronted with sudden onset of pseudovesicles of the eyelids after surgery, dermatologists must not misdiagnose cutaneous emphysema and must examine their patient carefully for other clinical signs. Bronchial endoscopy should be undertaken immediately to investigate for tracheal perforation.

[Infiltrative basal cell carcinoma presenting as chronic leg ulcer]. / Carcinome basocellulaire infiltrant se présentant comme un ulcère de jambe chronique.

BACKGROUND: Basal cell carcinoma is the most common carcinoma of the skin and is usually found on the head and neck. We report an unusual case of basal cell carcinoma presenting as a chronic leg ulcer, with underlying bone involvement. CASE REPORT: A 70-year-old woman presented with a 15-year history of leg ulcer refractory to treatment. Because of the exuberant granulation tissue on the base and the indurated edges of this circumferential leg ulcer, several biopsies were taken from the edge and the base of the ulcer. Histological examination revealed infiltrative basal cell carcinoma. Treatment consisted of surgical excision of 80% of the primary lesion and coverage with a split-thickness skin graft. Examination of the surgical piece revealed invasion of bone by the carcinoma. The remainder of the lesion not accessible to surgery was irradiated. DISCUSSION: There is a need for awareness among all doctors of the clinical signs evocative of malignant transformation of a leg ulcer so that a skin biopsy may be performed for suspicious ulcers. Our case is distinguished by the underlying invasion of bone by basal cell carcinoma, as attested by imaging and histology.

[Recurring pyoderma gangrenosum in pregnancy]. / Pyoderma gangrenosum récidivant lors des grossesses.

BACKGROUND: We report the case of a 25-year-old patient developing pyoderma gangrenosum (PG) during the third trimester of her first and second pregnancies. CASE REPORT: A 25-year-old woman developed PG on her left calf during week 32 of her second pregnancy; previously, during week 36 of her first pregnancy, the patient had presented abdominal pain with inflammatory syndrome. She underwent caesarean delivery at week 37 of pregnancy. Surgery was complicated by aseptic abdominal and parietal abscesses as well as aseptic endometritis with neutrophil infiltrate. She underwent two further surgical interventions that were followed by skin manifestations typical of PG. The two newborns were otherwise healthy. After five years of follow-up, we found no disorders usually associated with neutrophilic dermatosis. DISCUSSION: Pyoderma gangrenosum is extremely rare in pregnancy, with only 16 cases being reported in the international literature: nine during pregnancy and seven post-partum. The question is thus raised of a possible relationship between this neutrophilic dermatosis and pregnancy. The association could be due to the pathergy phenomenon or to the increase in G-CSF levels occurring in pregnant women.