[Self-inflicted lesions in the context of hidradenitis suppurativa: Pathomimicry]. / Lésions auto-provoquées dans un contexte d'hidradénite suppurée : un cas de pathomimicrie.

BACKGROUND: Factitious disorders constitute a complex pathology for the dermatologist. Although a diagnosis is often indicated, it is difficult to confirm and treatment is complicated. Dermatitis artefacta is the somatic expression of an often serious psychiatric disorder consciously created by patients on their own cutaneous-mucosal surfaces but the motivation is unconscious and no secondary benefits are sought (in contrast to simulation). Pathomimicry represent a specific entity: the provocation of outbreaks of a known disease, triggered by voluntary exposure to a causative agent. Herein we report on a case of pathomimicry in a context of hidradenitis suppurativa. PATIENTS AND METHODS: A teenage girl whose main previous medical history consisted of grade-2 obesity and an episode of pubic abscess was seen at our clinic for axillary lesions. She presented in a state of negligence, was suspicious and aggressive, and refused to undress. After gaining her trust, clinical examination revealed prominent ulcerations (each with a granulated base) at a distance from the folds in the axillary areas, as well as typical hidradenitis lesions of Hurley Grade 2 with purulent openings and rope-like scars from the inguinal folds. Hospitalization was recommended and a positive outcome was achieved under antibiotic therapy with doxycycline, topical alginate and hydrocellular dressings. A psychiatric evaluation concluded that the patient was presenting dysmorphophobic narcissistic weakness, probably in reaction to recurrent harassment at school since childhood. Once she developed trust with us, which was difficult to establish, the patient admitted to having caused the lesions herself. Given the history and clinical data, as well as the negative laboratory tests, a diagnosis of pathomimicry was made. DISCUSSION: Several cases of dermatological pathomimicry (sustainment by the patient of an ulcer with a known cause, contact with an allergen found in eczema, or renewed use of a medication implicated in toxiderma) or systemic disease (insulin injection in a diabetic patient) have been reported. To the best of our knowledge, this is the first description of pathomimicry associated with hidradenitis suppurativa. Regarding therapy, aftercare should be multidisciplinary. Confessions should not be forced and confrontations, which risk serious psychiatric collapse, should be avoided. A reassuring attitude enables psychiatry to be applied once trust has been sustainably established, hence the crucial role of the dermatologist.

[Cutaneous metastases on the extremities]. / Métastases cutanées des extrémités.

BACKGROUND: Cutaneous metastases (CM) on the extremities are rare complication of cancer with poor prognosis. In general, lesions simulate an infection. Herein, we report two new cases with atypical presentation. PATIENTS AND METHODS: Case no 1: a 71-year-old man consulted for suspicion of left hand pyogenic granuloma present for 3 months. His history revealed two treated squamous-cell carcinomas (tongue and lung). On physical examination, he presented three budding and foul-smelling lesions on his left hand. Histopathology showed metastasis of squamous-cell carcinoma. Radiographic examination revealed spread of pulmonary nodules with suspicion of metastasis. Case no 2: a 68-year-old man was hospitalized for indurated edema of the right leg present for several months. Six months earlier, he had undergone surgery for left pulmonary adenocarcinoma without metastasis. Physical examination revealed an indurated edema on the right foot. Histopathology showed metastasis from adenocarcinoma. A scan revealed several osteolytic lesions in the right foot as well as lymphadenopathy. DISCUSSION: Herein, we report two original cases of CM of the extremities diagnosed as tumor progression. This is a rare complication of variable clinical presentation and impacts both cancer management and prognosis. It is important to consider the diagnosis when distal cutaneous lesions persist, particularly where there is a history of cancer.

[Characteristics of chronic wounds in substance abuse: A retrospective study of 58 patients]. / Caractéristiques des plaies chroniques chez les toxicomanes : étude rétrospective de 58 patients.

BACKGROUND: Drug addiction causes chronic wounds (CW) responsible for severe complications. Very few studies are available on this topic. The aim of our study was to describe the demographic, clinical and etiological characteristics as well as the course of CW in drug addicts. PATIENTS AND METHODS: This was a retrospective and prospective multicenter study including all drug addicts with CW. RESULTS: We included 58 patients (17 prospectively), 84.5% of whom were male, of median age 43 years, presenting multiple CW as a result of intravenous (78.2%), inhaled (41.1%) and/or snorted (20%) drug abuse. Addiction to opioids (68.4%), cocaine (47.4%) and/or cannabis (40.4%) was ended and/or treated through substitution in 79.3% of patients. CW were fibrinous and necrotic (42.9 to 53.6%), recurrent (54.2%), and in some cases had been present for more than 1 year (61.5%). Intravenous drug addiction was associated with large, fibrinous, ulcers in a setting of venous and lymphatic insufficiency (74%). Only 23% of these wounds involved the upper limbs. Necrotic ulcers associated with clinical arteriopathy were described mainly with inhaled addiction. Abscesses (50%) and erysipelas (29.3%) were the most common cutaneous complications. After 3 months, 50% of CW were improved and 29.2% of patients were lost to follow-up. DISCUSSION: Drug abuse-related CW occurred preferentially in young men with history of intravenous abuse. For the most part, CW were seen on the legs and were associated with venous and lymphatic insufficiency, and the resulting major risk for cutaneous infection increased morbidity and mortality in this population in whom medical follow-up is inherently complicated.

[Rare presentations of infantile hemangiomas: 4 cases]. / Présentations rares d'hémangiomes infantiles : 4 cas.

BACKGROUND: Infantile hemangioma (IH) is a common benign vascular tumor in children. In most cases, diagnosis is based entirely on clinical examination. When the diagnosis is uncertain, the first-line complementary examination is Doppler ultrasound. We report 4 cases of atypical infantile hemangiomas with delayed diagnosis and non-contributory imaging. PATIENTS AND METHODS: One child had congenital purple papules and nodules on the back of the foot, the second had inaugural ulceration of the buttocks, and the last two presented telangiectasia, either isolated or on an erythematous macula. In two cases, ultrasound showed no vascular lesions, and in the other two cases, the absence of hyperemia did not allow a diagnosis of IH to be made. For one patient, diagnosis was made on the basis of cutaneous biopsy, and for the other three, on the basis of clinical course. DISCUSSION: We report 4 rare forms of infantile hemangioma resulting in initial diagnostic error. The atypical nature of some IHs may direct the clinician and the radiologist toward other diagnoses that in some cases have no vascular contingent. It is important for the dermatologist to be aware of these rare forms of IH in order to reduce the time to diagnosis and allow early initiation of appropriate management.

[Pseudocyst of the scalp: two cases]. / Pseudo-kystes du cuir chevelu : deux cas.

BACKGROUND: Pseudocysts of the scalp are a poorly known entity. Herein we present two new cases. CASE REPORT: Two male patients aged respectively 42 and 33 years had been presenting a small number of pseudocystic lesions on the scalp, as well as alopecia limited to the same region, for a number of years. Inflammatory episodes had occurred in certain lesions. In one case, biopsy showed an aspect typical of pseudocyst of the scalp and the lesions appeared to stabilize on treatment with doxycycline. DISCUSSION: Pseudocysts of the scalp are also known as aseptic nodules of the scalp, a term that appears to us to be less appropriate. They form lesions involving alopecia and they may be inflammatory to various degrees. Following incision of these lesions, a deep yellow fluid liquid is released that may be shown by microbiological analysis to be sterile. Histological analysis reveals a pseudocystic wall associated with perilesional inflammatory remodelling. The cysts are frequently confused with dissecting cellulitis of the scalp, but the prognosis is more encouraging. Treatment involves surgical excision, and intralesional injections of corticosteroids or doxycycline.

[Stevens-Johnson syndrome and Hodgkin's disease: A fortuitous association or paraneoplastic syndrome?]. / Syndrome de Stevens-Johnson et maladie de Hodgkin : association fortuite ou syndrome paranéoplasique ?

BACKGROUND: Hodgkin's disease has been associated with a variety of cutaneous symptoms. We report two cases of Stevens-Johnson syndrome (SJS) associated with Hodgkin's disease. PATIENTS AND METHODS: Case 1: a 22-year-old man was hospitalized for a second erythematous vesicular eruption with intense mucosal involvement. Histopathological examination confirmed the diagnosis of Stevens-Johnson syndrome. He also developed enlarged cervical lymph nodes that revealed Hodgkin's disease. The latter diagnosis was followed by two recurrent rashes. Treatment consisted of systemic chemotherapy. Complete remission was obtained with no signs of cutaneous recurrence after 24 months of regular follow-up. Case 2: a 29-year-old man was admitted for a generalized erythematous and bullous rash with intense mucosal involvement. Histopathological examination confirmed the diagnosis of Stevens-Johnson syndrome. He then developed muco-cutaneous icterus that was secondary to Hodgkin's disease. Under specific hematologic treatment, no cutaneous relapse was noticed. DISCUSSION: These cases illustrate the rare association of SSJ revealing Hodgkin's disease. In these cases, no evidence was found of infectious disease or drug-induced cutaneous effects. Only one case of toxic epidermal necrolysis associated with Hodgkin's disease had previously been reported. The link between both diseases may be immunosuppression induced by Hodgkin's disease, which could favor infection inducing SJS or secretion by tumor cells granulysin, a mediator responsible for damage to keratinocytes.

[Changes in the European baseline series from 1981 to 2011 in a French dermatology-allergology centre]. / Évolution de la batterie standard européenne de 1981 à 2011 dans un centre de dermato-allergologie français.

BACKGROUND: Periodically updated, the European baseline series (EBS), first introduced in France in 1980, is an indispensable tool for the exploration of contact allergy. The aim of our study was to describe the prevalence of contact sensitization in a French centre between 1981 and 2011 to determine whether certain allergens may be deleted from the current BSE. PATIENTS, MATERIALS AND METHODS: A retrospective study was conducted in a department of dermatology-allergology to analyse the results of all EBS tests performed every 10 years, from 1981 to 2001, and annually from 2007 to 2011. Some added allergens, introduced in 2010, were also studied. Changes in allergen positivity were analysed using a Cochran-Armitage test. RESULTS: Among the 4551 patients included for the 8-year period studied, the prevalence of positivity was 42.91%. The most common allergens in the general population were nickel sulphate (17.25%), with a constantly increasing prevalence, Myroxylon pereirae (10.68%), fragrance mix I (8.11%), cobalt chloride (6.99%) and chromium (6.33%). The least frequent sensitizations, with a decreasing prevalence, were found with clioquinol (0.25%), primin (0.54%) and benzocaine (0.55%). CONCLUSION: Due to modifications in exposure to allergens, the incidence of contact sensitization can change, but nickel sulphate sensitization is increasing despite recent European directives. Allergens with less than 1% of positive results could be withdrawn from the EBS, with benzalkonium chloride, methylisothiazolinone and lavender absolute being added.

[Recurrent toxin-mediated perineal erythema in an 11-year-old child]. / Érythème périnéal récidivant toxinique chez un enfant de 11 ans.

INTRODUCTION: Recurrent perineal erythema is a rare toxin-mediated disease. We report the case of recurrent toxin-mediated perineal erythema in a child. CASE REPORT: An 11-year-old boy was hospitalized for erythematous pustular eruption involving the perineum and the axillary area. This erythema started a few days after the onset of pharyngotonsillitis and the patient's personal history involved another episode of pharyngotonsillitis which was followed by an identical cutaneous eruption. Laboratory analysis confirmed the diagnosis of recurrent toxin-mediated perineal erythema. The skin disorder quickly improved and antistreptococcal antibiotic treatment was initiated to eradicate bacteria. DISCUSSION: Recurrent toxin-mediated perineal erythema is a cutaneous disease mediated by superantigens which are toxins produced by staphylococci and streptococci. It is characterized by recurrent macular erythema involving the perineum. Streptococcus pyogenes is the most common cause of recurrent toxin-mediated perineal erythema, with Staphylococcus aureus being isolated most rarely. This observation emphasizes the possibility of atypical clinical presentation with pustular lesions, and dermatologists must be mindful of this aetiology in order to isolate bacterial toxins and to initiate appropriate antibiotics.

[Cutaneous phaeohyphomycosis due to Cladophialophora bantiana]. / Phaeohyphomycose cutanée à Cladophialophora bantiana.

BACKGROUND: Among dematiaceous fungi responsible for phaeohyphomycosis, Cladophialophora bantiana is an opportunistic pathogen that causes central nervous system infections, chiefly in immunocompromised patients. Only a few reports on skin involvements have been reported in the recent dermatological literature. Herein we report the case of an immunocompetent patient with cutaneous phaeohyphomycosis. CASE REPORT: A 48-year-old male presented a nodular, painless and non-suppurative lesion with a diameter of 1cm on the right buttock that had developed since his return from a trip to Vietnam. A diagnosis of phaeohyphomycosis due to C. bantiana was made based on the histopathology and mycology examinations, which allowed the identification of C. bantiana, a dematiaceous (black) fungus from hyphomycete species. DISCUSSION: C. bantiana is a neurotropic fungus that causes central nervous system infections in particular. Extracerebral involvement is rare and only a few cases of cutaneous phaeohyphomycosis have been reported. Furthermore, since immunocompromised hosts are more vulnerable, this mycosis is more commonly seen in immunocompromised patients. However in this particular case, an intramuscular injection of corticosteroids could have caused local immunosuppression. The prognosis depends on both localization and site. There are no guidelines for optimal treatment.

[Bullous paraneoplastic acrokeratosis]. / Acrokératose paranéoplasique bulleuse.

BACKGROUND: Bullous lesions of acral distribution are an uncommon finding in Bazex's syndrome (acrokeratosis paraneoplastica). We report here one of these rare cases. PATIENTS AND METHODS: A 65 year-old-man, an alcoholic and a smoker, presented with characteristic lesions of Bazex's acrokeratosis paraneoplastica associated with a right cervical lymph node mass. An epidermoid carcinoma of sinus piriformis was then discovered. The acrokeratosis lesions worsened and spread to the knees, elbows, trunk and genitalia, with an erosive aspect, pain in the extremities, and tender, hemorrhagic bullous lesions on the toes and sides of the feet. Histological examination of a biopsy sample showed a subepidermal blister with numerous eosinophilic leucocytes. Direct immuno-fluorescence showed C3 deposits on dermal capillaries and IgA and IgM on colloid bodies in the papillary dermis. Blood eosinophilia and high levels of IgE were noted. Indirect immunofluorescence was negative for anti-epidermal and anti-basement membrane antibodies. Radiation and chemotherapy for the neoplasia resulted in healing of the bullous lesions and almost complete disappearance of keratotic squamous lesions with residual hyperpigmentation. DISCUSSION: Although bullous lesions are rare in Bazex's syndrome (acrokeratosis paraneoplastica), they were noted as early as 1968 by Degos et al. These acral bullae mimic autoimmune bullous disease. An immunological reaction is thought to occur with antigens of the dermal-epidermal junction and eosinophils appear to be involved.

Recent advances in understanding structure-function relationships in the type II topoisomerase mechanism.

DNA topos (topoisomerases) are complex, multisubunit enzymes that remodel DNA topology. Members of the type II topo family function by passing one segment of duplex DNA through a transient break in another, a process that consumes two molecules of ATP and requires the co-ordinated action of multiple domains. Recent structural data on type II topo ATPase regions, which activate and enforce the directionality of DNA strand passage, have highlighted how ATP physically controls the catalytic cycle of the enzyme. Structural and biochemical studies of specialized DNA-binding domains in two paralogous bacterial type IIA topos (DNA gyrase and topo IV) show how these enzymes selectively negatively supercoil or decatenate DNA. Taken together, these findings expand our understanding of how disparate functional elements work together to co-ordinate the type II topo mechanism.

Vulvar cicatricial pemphigoid of childhood.

We report a 9-year-old girl with a vulvar autoimmune bullous dermatosis. A diagnosis of localized bullous pemphigoid or cicatricial pemphigoid was made on the basis of immunohistologic data. Since the lesions were unresponsive to topical corticosteroids but healed completely on dapsone at a dosage of 1.5 mg/kg/day, we favor the diagnosis of vulvar cicatricial pemphigoid. Only two such cases have been reported thus far. The diagnostic criteria and therapeutic modalities are discussed.