[Epidermolysis bullosa acquisita with Brunsting-Perry type pemphigoid: Diagnostic and therapeutic difficulties]. / Épidermolyse bulleuse acquise à type de pemphigoïde de Brunsting-Perry : difficultés diagnostiques et thérapeutiques.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare auto-immune blistering disease. We report a case of Brunsting-Perry pemphigoid diagnosed by immunoelectron microscopy (IEM). PATIENTS AND METHODS: A 46-year-old man presented very pruriginous vesicles on the face and neck present for 6 years and which were difficult to diagnose and treat. The appearance of atrophic scars and milium cycts evoked EBA, which was confirmed at IEM. Due to limited involvement of the face and the neck, we conclude on EBA of the Brunsting-Perry pemphigoid variant. Treatment with dapsone produced a favorable outcome. DISCUSSION: Diagnosis of EBA is often difficult. In a case review, Asfour et al. collated 60 cases of Brunsting-Perry pemphigoid. These patients had either anti-collagen VII or anti-BP180 and anti-BP230 antibodies. IEM showed cleavage either under the lamina densa or within the lamina lucida, suggesting that Brunsting-Perry pemphigoid is a subtype of EBA or bullous pemphigoid (BP), depending on the paraclinical elements, and localized to the head and neck. The majority of EBA-like cases required systemic therapy, whereas in the presence of BP antibodies, topical corticosteroids were effective. CONCLUSION: We report a case of EBA of the Brunsting-Perry pemphigoid type, diagnosed by IEM after 6 years of progression. We highlight the diagnostic and nosological difficulties of Brunsting-Perry pemphigoid. Classification of this dermatosis as a subtype of EBA or BP may enable effective adaptation of therapeutic management, which has not as yet been coded.

[The cutaneous porphyrias]. / Porphyries cutanées.

The porphyrias are a group of metabolic disorders resulting from an innate abnormality in haem biosynthesis, and the clinical settings of which vary according to the genetic enzyme abnormality in question. These are genetic disorders with autosomal dominant or recessive inheritance of varying penetrance, and whose clinical expression differs according to the preferential location of haem precursors. Different classifications have been proposed according to genetic inheritance, the enzyme anomaly at issue, and clinical expression. The clinical classification distinguishes between acute porphyria (acute intermittent porphyria, porphyria variegata, hereditary coproporphyria), bullous cutaneous porphyrias (porphyria cutanea tarda, porphyria variegata and hereditary coproporphyria), painful photosensitive acute cutaneous porphyrias (erythropoietic protoporphyria and X-linked dominant protoporphyria), and rare recessive porphyrias (congenital erythropoietic porphyria, Doss porphyria, hepatoerythropoietic porphyria and harderoporphyria). Treatment depends on the clinical expression of the disorder.

[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.

Silver absorption and toxicity evaluation of silver wound dressings in 40 patients with chronic wounds.

BACKGROUND: Silver-containing dressings are considered to be safe even though there have been some reports of complications, including argyria and various organ system dysfunctions. Despite the widespread use of silver dressings, little research has been done regarding the absorption and toxicity of silver. OBJECTIVE: We aimed to study the systemic absorption of silver in patients with chronic inflammatory wounds and to determine associated factors of systemic silver absorption and evaluated its association with silver toxicity. PATIENTS AND METHOD: Prospective, longitudinal, observational, multicentre, open-label pilot study. Patients from the Dermatology Departments of Lorraine (France) with the following inclusion criteria: (i) a chronic wound of more than 6 weeks and (ii) an ulcer needing silver-containing dressing were included. Before and after 28 days of treatment, clinical characteristics of the wound were recorded; hemogram, hepatic and renal functions, albumin sera and serum silver level were measured. RESULTS: Half of the cases displayed raised levels of silver after 1 month of treatment. Predictive factors for systemic silver absorption were wound area, anaemia and malnutrition with anaemia and malnutrition confirmed on multivariate analysis. Wound vascularization may also play a role, as a higher absorption was observed in cases of wound granulation without arterial components. No toxicity was detected. This work has also emphasized the slow elimination of silver from the body. CONCLUSION: Both long-term application and iterative treatments with silver dressings should be discouraged, especially in the elderly, who often suffer from malnutrition and anaemia to avoid potential cumulative toxicity.

[Extra-facial Lever granuloma on a herpes zoster scar: Wolf's isotopic response]. / Granulome de Lever extra-facial sur cicatrice de zona : une réaction isotopique de Wolf.

BACKGROUND: Granuloma faciale (GF), or facial granuloma of Lever, is an uncommon dermatosis occurring classically on the face. We report a case of a GF on zoster scars located on the trunk, which is a highly particular isotopic response. PATIENTS AND METHODS: A 60-year-old man with a sole history of left lumbar zoster presented with a skin lesion present for two months. Clinical examination revealed a 2.5-cm papular erythematous lesion that was both infiltrated and pruritic. This lesion was situated precisely on zoster scars. Lab tests revealed no abnormalities. Histopathologic examination showed an inflammatory process without neovessels but rich in eosinophils with chronic vasculitis and polymorphic infiltrate, on which basis GF was diagnosed. DISCUSSION: GF is a localized form of cutaneous small-vessel vasculitis. Although it usually affects the face, extra-facial locations also exist. The original character of this presentation, aside from the location, is the occurrence of this GF on a zoster scar, which corresponds to Wolf's isotopic response. This phenomenon, described by R. Wolf, comprises the occurrence of a new skin disorder at the site of another unrelated and already healed skin disease, usually herpes. The physiopathology is still unclear. This differs from isomorphic reactions such as Koebner's reaction, which consists of the appearance at an injury site of skin lesions typical of an existing dermatosis but not a new disorder. CONCLUSION: This is a rare case of Wolf's isotopic response with GF, of which, to our knowledge, there are no reports in the literature.

[Livedoid vasculopathy secondary to coeliac disease]. / Vasculopathie livédoïde secondaire à une maladie cœliaque.

BACKGROUND: Livedoid vasculopathy (LV) is a painful ulcerative condition involving white atrophy and livedo; a histopathologic feature seen is occlusive dermal vasculopathy. This may be associated with coagulation disorders such as hyperhomocysteinaemia (HHC). PATIENTS AND METHODS: We report the case of a 52-year-old woman presenting LV in which an abnormal scan image led us to diagnose coeliac disease. This enteropathy had caused vitamin B12 and folic acid deficiency, as well as HHC. Vitamin supplementation and a gluten-free diet resulted in complete healing of the lesions. DISCUSSION: This case underlines the importance of screening for and correction of coagulation disorders in patients with LV. It also suggests that in the event of HHC, coeliac disease should be sought, even in the absence of gastrointestinal symptoms.

Zosteriform configuration of multiple eccrine spiradenomas of the scalp.

BACKGROUND: Eccrine spiradenoma (ES) is a benign adnexal tumor predominantly located in the head and neck regions. Multiple neoplasms located on the scalp have been reported but never with a zosteriform configuration on the first trigeminal area. CASE REPORT: We describe an original case report of a 75-year-old Caucasian man presenting multiple subcutaneous blue and purple nodules disseminated on the first left trigeminal dermatome. All the nodules appeared gradually on a one-year period. Biopsy revealed a nodular adnexal tumor in the dermis without malignant eccrine spiradenoma (MES) transformation. The surgical procedure was performed in a manner to protect the galea aponeurotica in the upper half on the first left trigeminal area. The frontalis muscle was raised with the surgical specimen in the lower half of the first trigeminal area. A split-thickness skin graft was applied on the surgical defect. Histological examination revealed multilobular well-defined tumors located in the dermis. CONCLUSION: The presence of multiple subcutaneous nodules in a trigeminal pattern should suggest a multiple localized zosteriform ES. The diagnosis is focused on clinical findings and the treatment is based on a large surgical excision. The histological examination is essential for not to fail a MES transformation.

Combined theoretical and time-resolved photoluminescence investigations of [Mo6Brⁱ8Br(a)6]²â» metal cluster units: evidence of dual emission.

The combined time-resolved photoluminescence (PL) and theoretical study performed on luminescent [Mo6Br(i)8Br(a)6](2-)-based systems unambiguously shows that their NIR-luminescence is due to at least two emissive states. By quantum chemical studies, we show for the first time that important geometrical relaxations occur at the triplet states either by the outstretching of an apex away from the square plane of the Mo6 octahedron or by the elongation of one Mo-Mo bond. Experimental PL measurements demonstrate that the external environment (counter-ions, crystal packing) of the cluster has a noticeable impact on its relaxation processes. Temperature and excitation wavelength dependence of the two components of the luminescence spectra is representative of multiple competitive de-excitation processes in contradiction with Kasha's rule. Our results also demonstrate that the relaxation processes before and after emission can be tracked via fast time-resolved spectroscopy. They also show that the surroundings of the luminescent cluster unit and the excitation wavelength could be modulated for target applications.

[Linear tuberculoid leprosy along the lines of Blaschko: a very rare presentation]. / Lèpre tuberculoïde de disposition linéaire blaschkoïde: une présentation exceptionnelle.

BACKGROUND: Leprosy continues to be present in certain regions throughout the world, and the dermatologist plays a central role in its diagnosis. Herein we report a case of tuberculoid leprosy that is atypical in terms of its linear presentation which appears to follow the lines of Blaschko. PATIENTS AND METHODS: A patient from Mayotte was referred to the neurological department for suspected tuberculoid leprosy. He was presenting a deficiency of the ulnar nerve together with neuronal hypertrophy and cutaneous involvement. Dermatological examination revealed linear hypo-aesthetic hypopigmented lesions on the arm and forearm. The atypical clinical presentation also suggested to us pigmented mosaicism or post-inflammatory pigmentation. The biopsy showed granulomatous epithelial dermatitis with perinervous involvement. Imaging examinations confirmed the presence of neuronal hypertrophy. A diagnosis of linear tuberculoid leprosy was made. DISCUSSION: This case illustrates the need to bear in mind a diagnosis of leprosy in the event of hypopigmented lesions, even where they are linear and of Blaschkoid appearance. Several potential hypotheses may account for this particular topographical pattern.

[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.

[Acquired haemophilia A: two cases]. / Deux observations d'hémophilie A acquise.

BACKGROUND: Acquired haemophilia A (AH) is an uncommon bleeding disorder that presents as multiple, disseminated spontaneous subcutaneous bleeds. Diagnosis may be made on the basis of prolonged activated partial thromboplastin time (aPTT). The severity of the disease is associated with the low risk of haemoglobin levels and with potential links with other diseases. OBSERVATIONS: Two men were hospitalized for extensive and spontaneous subcutaneous hematoma. In both cases, the International Normalized Ratio (INR) was normal, but aPTT was 3 times higher than normal. Autoantibodies against coagulation factor VIII confirmed the diagnosis of AH. The patients received immunomodulatory treatment. In one patient, diffuse large B-cell lymphoma was discovered one year after successful treatment of AH. DISCUSSION: AH may be revealed by areas of bruising, subutaneous haematomas mimicking erythema nodosum, and muscle pain. APTT results alone can prompt the biologist to screen for factor VIII inhibitors. Aside from the risk of fatal bleeding, in half of all cases, the prognosis is determined by associated disorders such as blood dyscrasias, solid tumours, autoimmune diseases, use of certain medicines and pregnancy. After treatment for bleeding complications, therapy focuses on restoring the coagulation time. The aim of immunomodulatory therapy is to stem production of autoantibodies against coagulation factor VIII. CONCLUSION: AH must be considered rapidly in order to reduce the risk of bleeding emergencies and to screen for potential related diseases.

[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.

[A case of primary cutaneous PEComa]. / Un PECome cutané primitif.

BACKGROUND: PEComas (PEC: Perivascular epithelioid cell) are tumours expressing both melanocytic and myogenic markers. The clinical features are non-specific. PATIENTS AND METHODS: A 55-year-old woman presented with a non-specific tumour on her right arm. Histological examination revealed an intradermal tumour composed of clear epithelial cells. The tumour was positive for HMB45 and protein S100, and was negative for Melan-A and actin. A diagnosis of PEComa was retained despite negative myogenic markers. DISCUSSION: PEComas are neoplasms composed of nests and fascicles of clear to granular epithelioid cells that express both melanocytic markers (HMB45, Melan-A, NK1C3, tyrosinase) and myogenic markers (actin, caldesmin, desmin, calponin), whereas S100 protein and cytokeratins are usually absent. Included in this broad category are angiomyolipoma, clear-cell "sugar" tumours and lymphangioleiomyoma. Some deep PEComas may have a malignant course. Cutaneous primitive forms are exceptional and often benign. Histological differential diagnoses to consider are those of clear-cell tumours: paraganglioma-like dermal melanocytic tumour, melanoma, metastatic clear-cell renal carcinoma and epithelioid sarcoma. In our patient, a diagnosis of cutaneous primitive PEComa was retained despite the absence of myogenic markers and the positivity of the protein S100, based on the cytological and immunohistochemical features, which were not evocative of any other diagnosis. CONCLUSION: Cutaneous PEComa are non-specific tumours. Histological analysis confirms the diagnosis and allows other more aggressive tumours to be ruled out.

[Melanoma of the anal margin]. / Mélanome de la marge anale.

BACKGROUND: Primary anal mucosal melanoma is rare and is associated with a poor prognosis. The observation of a case of anal melanoma at a localized stage in a woman led us to analyze recent data from the literature on therapeutic alternatives. PATIENTS AND METHODS: A 49-year-old woman presented with a pigmented swelling of the anal margin that had begun three months earlier. Complete local excision of the tumour was performed with the conservation of the anal sphincters. Histological examination revealed SSM mucosal melanoma. Abdominoperineal resection was finally performed because of tumoural invasion of the lateral margins. Staging assessment was normal. Half-yearly MRI monitoring of the pelvis was proposed and at nine months no relapse was seen. DISCUSSION: The unusual and misleading symptoms often account for the late diagnosis and poor prognosis of anal melanoma. Treatment is not well defined: local excision with conservation of the anal sphincters is recommended as first-line therapy, but the surgical technique is controversial. Abdominoperineal resection is recommended if the surgical margins are invaded, in the case of local recurrence or if the tumour is inaccessible. The place of adjuvant therapies remains to be defined. More recently, the discovery of mutation in c-KIT mucosal melanoma has allowed the use of biotherapy. Our observation underscores the importance of early detection of anal melanoma by all practitioners concerned in view of its aggressiveness and we report the difficulties of therapeutic management in the absence of established guidelines.

Is diabetes an acquired disorder of reactive glucose metabolites and their intermediates?

AIMS/HYPOTHESIS: We hypothesised that diabetic patients would differ from those without diabetes in regard to the handling of glucose-derived reactive metabolites, evidenced by triosephosphate intermediates (TP(INT)) and methylglyoxal (MG), irrespective of the type of diabetes, plasma glucose level or HbA(1c) value. METHODS: To test this hypothesis, erythrocytes were isolated from patients with type 1 (n = 12) and type 2 (n = 12) diabetes with varying blood glucose and HbA(1c) levels. These were then compared with erythrocytes isolated from individuals without diabetes (n = 10), with respect to MG, as determined by HPLC, and TP(INT), as determined by endpoint enzymatic assays. RESULTS: The concentrations of intracellular TP(INT) and MG were significantly elevated in erythrocytes from diabetic patients. Normalisation of either TP(INT) or MG to intracellular glucose concentration (nmol glucose/mgHb) confirmed that erythrocytes from diabetic patients accumulated more reactive metabolites than did those from healthy controls. CONCLUSIONS/INTERPRETATION: Diabetic patients can be characterised by an increased formation of TP(INT) and MG. The 25-fold increase of MG in type 1 and the 15-fold increase in type 2 diabetes, together with a several-fold increase in TP(INT) and decreased glyceraldehyde-3-phosphate dehydrogenase activity even under normal glucose conditions, imply that normalising glucose level cannot completely prevent late diabetic complications until this acquired error of metabolism has been restored.

[Indolent lymphoma of the ear]. / Lymphome indolent de l'oreille.

INTRODUCTION: In 2007, Petrella et al. identified a new entity: CD8 T-cell indolent lymphoma of the ear. CASE REPORT: A 40-year-old man presented a nodular erythematous and violaceous painless lesion on his right ear that had appeared four months earlier. Excision histology revealed a non-epidermotropic T-cell proliferation infiltrating the entire dermis and subcutis but with sparing of a grenz zone. The monotonous infiltrate was positive for CD8, CD3, CD5 and TIA-1, and negative for CD30, CD4, CD56, ALK and EMA. The Mib1 proliferation index was 20%. Lyme serology and PCR for EBV were negative. Additional examinations showed no extracutaneous involvement. DISCUSSION: CD8+ indolent lymphoma is an entity first described in 2007 and reported in the literature in 15 patients. Lesions are located on the nose or external ear. It comprises a non-epidermotropic proliferation of CD8+ T lymphocytes negative for CD4, CD30, CD56, CD57, granzyme B and perforin. The Mib1 proliferation index is low. This new entity appears neither in the 2005 World Health Organization/European Organization for Research and Treatment of Cancer (WHO/EORTC) classification of cutaneous lymphomas nor in the WHO 2008 Classification of tumours of haematopoietic and lymphoid tissues. Surgical treatment or radiotherapy is sufficient, and unlike aggressive, epidermotropic CD8+ T lymphomas chemotherapy is not required.

Usefulness of the script concordance test in dermatology.

BACKGROUND: The script concordance test (SCT) measures clinical data interpretation in the context of uncertainty. To our knowledge, its feasibility has not yet been demonstrated in dermatology. OBJECTIVES: This study describes the feasibility, reliability and validity of the SCT for use in dermatology teaching to family medicine residents. METHODS: We designed an SCT for dermatology teaching, adapted to the level of family medicine students. The family medicine residents attending a dermatology lecture course and a reference panel of dermatologists underwent the test. Software available on the SCT-dedicated website was used to determine the scores. The Cronbach's α was calculated. The scores were described by means, standard deviation, and minimum and maximum scores. Mann-Whitney tests were used to compare resident and reference panel scores. RESULTS: The test contained 33 vignettes of four questions each. Cronbach's α was 0.80. Mean scores were 75.6 for the reference panel and 65.0 for the residents (P = 0.0009). CONCLUSIONS: This study confirmed the SCT's capacity to distinguish groups on the basis of experience. This study demonstrated the feasibility and reliability of the SCT in the field of dermatology.

[Neutrophilic dermatosis of the hands]. / Dermatose neutrophilique des mains.

BACKGROUND: In 1964, based on eight cases, R. Sweet described a form of "acute febrile neutrophilic dermatosis" that was quickly renamed Sweet's syndrome. Over time, other entities (pyoderma gangrenosum, erythema elevatum diutinum, etc.) came to be included in the same nosological group, giving rise to the concept of neutrophilic dermatosis. In addition to types of passage between these different diseases, neutrophilic dermatoses may have a variety of atypical presentations such as that described as "neutrophilic dermatosis of the hands", of which we present a case herein. PATIENTS AND METHODS: A 60-year-old woman with non-insulin-dependent diabetes and mild psoriasis was consulting for an eruption mainly on the hands. The lesions, of various shapes, comprised pustules and large bullous lesions on purple oedematous skin; these were painful and some were progressing to ulceration. They measured between 0.5 and 3 cm and were filled with an opaque liquid. These lesions were painful and some were progressing towards ulceration. The patient was also presenting inflammatory joint pain. The histopathology study revealed infiltration of the dermis, predominantly by neutrophils. A diagnosis of neutrophilic dermatosis was made without any clearly discernible distinction between Sweet's syndrome, palmoplantar pustulosis or pyoderma gangrenosum. DISCUSSION: Since the initial description of Sweet's syndrome in 1964, many unusual cases have been described in the literature (associated with neoplasias or systemic diseases, concomitant pustular or bullous lesions, etc.). In 1995, Strutton et al. described a new entity, pustular vasculitis of the hands, based on six cases, which is fairly similar to the present case. Understanding of this syndrome developed and Gallaria renamed it neutrophilic dermatosis of the dorsal hands. Walling and Duquia termed this form of dermatosis palmoplantar Sweet syndrome. CONCLUSION: This case highlights the difficulties in clearly distinguishing forms of neutrophilic dermatosis, adding to the notion of a continuum in neutrophilic disease.