Search

Acral pustular disease within the pustular psoriasis/psoriasis-like spectrum mainly includes palmoplantar pustulosis (PPP) and acrodermatitis continua of Hallopeau (ACH). Scarce data argue for a distinction between these two entities, but no study has compared the clinical and epidemiologic characteristics of ACH and PPP.We aimed to perform a comparative description of the epidemiological and clinical characteristics of PPP and ACH in a multicentre retrospective cohort.In this multicentre national retrospective cohort study, we compared the epidemiological characteristics, comorbidities and psoriasis characteristics of patients with PPP and ACH.A total of 234 patients were included: 203 (87%) with PPP, 18 (8%) with ACH and 13 (6%) with both, according to 2017 ERASPEN criteria. As compared with ACH, PPP was associated with female sex, smoking activity and higher median BMI (P = 0.01, P = 0.02 and P = 0.05 respectively). A family background of psoriasis was more frequent in PPP than ACH. Age of onset of palmoplantar disease was similar between PPP and ACH patients, median age 44 and 48 years respectively. Peripheral joint inflammatory involvement was the only rheumatic disease associated with ACH. The association with another psoriasis type was similar in PPP and ACH (57.6% and 61.1% respectively).Our study confirms in a large PPP cohort the predominance of females and a high prevalence of smoking and elevated body mass index but also shows an association of these features in PPP as compared with ACH. In addition, it highlights peripheral arthritis as the only arthritis endotype associated with ACH. Increased knowledge of the immunogenetic backgrounds underlying these two entities is warranted to better stratify pustular psoriasis or psoriasis-like entities for precision medicine.

European guidelines propose a 0·5 mg kgIn a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0·5 mg kgIn total, 198 patients were included between 2015 and 2017. The final analysis comprised 190 patients with a mean age of 80·9 (SD 9·1) years. Control of disease activity was achieved at day 21 in 119 patients [62·6%, 95% confidence interval (CI) 55·3-69.5]; 18 of 24 patients (75%, 95% CI 53·3-90·2), 75 of 110 patients (68·8%, 95% CI 59·2-77·3) and 26 of 56 patients (46.4%, 95% CI 33·0-60·3) had mild, moderate and severe BP, respectively (P = 0·0218). A total of 30 patients died during the study. The overall Kaplan-Meier 1-year survival was 82·6% (95% CI 76·3-87·4) corresponding to 90·9%, 83·0% and 80·0% rates in patients with mild, moderate and severe BP, respectively (P = 0·5). Thresholds of 49 points for BPDAI score and 70 points for Karnofsky score yielded maximal Youden index values with respect to disease control at day 21 and 1-year survival, respectively.A 0·5 mg kg

BACKGROUND Psychiatric disorders have been extensively documented in patients with systemic lupus erythematosus (SLE). However, the prevalence of psychiatric disorders in patients with skin-restricted lupus (SRL) remains unknown, although SRL is more common than SLE. OBJECTIVES To assess current and lifetime prevalence of Axis I psychiatric disorders among outpatients with SRL and to examine the factors associated with psychiatric disorders among such patients. METHODS A multicentre case-control study involving outpatients with SRL and controls matched for sex, age and education level. The Mini International Neuropsychiatric Interview was used for psychiatric evaluation. RESULTS We evaluated 75 patients and 150 controls. Of these, 49% of patients vs. 13% of controls fulfilled the criteria for at least one current psychiatric disorder (P < 0·001). The following disorders were significantly more frequent among patients than controls: current and lifetime major depressive disorder (9% vs. 0%, P < 0·001 and 44% vs. 26%, P = 0·01), generalized anxiety disorder (23% vs. 3%, P < 0·001 and 35% vs. 19%, P = 0·03), panic disorder (7% vs. 0%, P = 0·004 and 21% vs. 3%, P < 0·001), current suicide risk (24% vs. 7%, P = 0·003), alcohol dependence (7% vs. 0%, P = 0·004) and lifetime agoraphobia (20% vs. 9%, P = 0·01). Lupus duration and lupus past treatment by thalidomide were significantly higher among patients with current psychiatric disorders. CONCLUSIONS This study demonstrates a high prevalence of several psychiatric disorders (anxiety, depression, suicide risk, alcohol dependence) in patients with SRL.

Background There are few population-based studies assessing the prevalence of skin diseases. Objectives To estimate the prevalence of five chronic skin inflammatory diseases, i.e. atopic dermatitis (AD), psoriasis, alopecia areata (AA), vitiligo and hidradenitis suppurativa (HS) in France, using validated self-diagnostic questionnaires. Methods Population-based study using a representative sample of the French general population aged more than 15 years and sampling with replacement design. All participants were asked (ii) to fill in a specific questionnaire including socio-demographic characteristics, (ii) to declare if they have been diagnosed with one or more skin problem or skin diseases during their life, and (iii) to fill in five validated self-reported questionnaires for AD, psoriasis, AA, vitiligo and HS. Results A total of 20.012 adult participants responded to the questionnaire of whom 9760 were men (48.8%) and 10.252 (51.2%) were women. We identified a prevalence of 4.65% for AD (931 individuals), 4.42% for psoriasis (885 individuals), 1.04% for AA (210 individuals), 0.46% for vitiligo (93 individuals) and 0.15% for HS (29 individuals), respectively. Limitations Questionnaire-based study and possible disease misclassifications. Conclusion This is the largest population-based study aiming to estimate the prevalence of five chronic skin inflammatory diseases.

Human T-lymphotropic virus type I (HTLV-I) is endemic in the Caribbean region, south-western Japan and Africa, and is associated with tropical spastic paraparesis and adult T-cell leukaemia/lymphoma (ATLL). Cutaneous forms of ATLL are sometimes indistinguishable from other cutaneous T-cell lymphomas (CTCL). We report a woman living in a non-endemic area for HTLV-I, with no risk factors for viral infection, who developed mycosis fungoides-like ATLL. The findings underline the usefulness of molecular biological techniques in distinguishing between mycosis fungoides and ATLL. We emphasize the need to establish the HTLV-I status of patients with CTCL, even in HTLV-I non-endemic areas, not only to establish a preventive policy in these countries, but also to further our knowledge of the lymphoproliferation spectrum associated with human retroviruses.

Dermal and subcutaneous inflammation following direct trauma is initially evocative of soft-tissue infection. However, two differential diagnoses must be considered: Morel-Lavallée syndrome and post-traumatic nodular fat necrosis.Case 1: a 51-year-old woman fell off her motorbike and had dermabrasions on her right and left tibial ridges that rapidly developed into dermo-hypodermitis of the entire limb. There was no improvement after 3 weeks of antibiotics. The patient was apyretic. She had a soft, non-inflammatory tumefaction on the inner aspect of her left knee. Ultrasound revealed subcutaneous collection in both legs. The surgeons confirmed a diagnosis of Morel-Lavallée syndrome and drained the two collections. Progress was good and the patient healed without major consequences. Case 2: following a fall on her stairs, a 40-year-old woman presented dermabrasions and haematomas on her left leg. Antibiotic therapy failed to prevent the progression of dermo-hypodermitis. The patient remained apyretic and there was no inflammatory syndrome. A CT scan showed thickening of a subcutaneous fat and fluid collection, resulting in diagnosis of post-traumatic nodular fat necrosis. Management was surgical and the outcome was good.These two cases show two post-traumatic cutaneous complications: Morel-Lavallée syndrome and post-traumatic nodular fat necrosis. Morel-Lavallée syndrome occurs after tangential trauma next to richly vascularized tissue. Post-traumatic nodular fat necrosis is defined as necrosis of adipocytes. In both cases, diagnosis is confirmed by imagery (Ultrasonography, tomography).Our two case reports show that inflammatory presentation of both Morel-Lavallée syndrome and post-traumatic nodular fat necrosis can lead to diagnostic and therapeutic errors while a surgical procedure is necessary since tissue necrosis can occur.

Annexin A1 (ANXA1) is a Ca2+-regulated phospholipid-binding protein involved in various cell processes. ANXA1 was initially widely studied in inflammation resolution, but its overexpression was later reported in a large number of cancers. Further in-depth investigations have revealed that this protein could have many roles in cancer progression and act at different levels (from cancer initiation to metastasis). This is partly due to the location of ANXA1 in different cell compartments. ANXA1 can be nuclear, cytoplasmic and/or membrane associated. This last location allows ANXA1 to be proteolytically cleaved and/or to become accessible to its cognate partners, the formyl-peptide receptors. Indeed, in some cancers, ANXA1 is found at the cell surface, where it stimulates formyl-peptide receptors to trigger oncogenic pathways. In the present review, we look at the different locations of ANXA1 and their association with the deregulated pathways often observed in cancers. We have specifically detailed the non-classic pathways of ANXA1 externalization, the significance of its cleavage and the role of the ANXA1–formyl-peptide receptor complex in cancer progression. * AA, : arachidonic acid; ABC, : ATP-binding cassette; Akt, : protein kinase B; ANXA1, : annexin A1; BLBC, : basal-like breast cancer cell; DFS, : disease-free survival; EGFR, : epidermal growth factor receptor; EMT, : epithelial–mesenchymal transition; ER, : endoplasmic reticulum; ERK, : extracellular signal-related kinase; FPR, : formyl-peptide receptor; GC, : gastric cancer; HIF, : hypoxia-inducible transcription factor; Iβ1BP1, : integrin β1-binding protein 1; MAPK, : mitogen-activated protein kinase; MMS, : methyl methanesulfonate; MP, : microparticle; MVE, : multivesicular endosome; NES, : nuclear export signal; PAF-AH, : platelet activating factor–acyl hydrolase; PKC, : protein kinase C; PLA2, : phospholipase A2; PM, : plasma membrane; PMA, : phorbol 12-myristate 13-acetate; PMN, : polymorphonucleocyte; TLS, : translesion synthesis; VEGF, : vascular endothelial growth factor; ZA, : zoledronic acid

To assess the efficiency of melanoma screening and prevention campaigns in the Auvergne region (France), cases of melanoma have been recorded since 1st June 1998. The epidemiological follow-up of melanoma was carried out using two sentinel networks; one involving the pathologists, and the other, the dermatologists of the region. Incidence was calculated using the capture-recapture method, by cross-matching the data supplied by both dermatologists and pathologists. Between June 1st 1998 and December 31st 2000, 363 cases of melanoma were recorded. The crude incidence rate of melanoma per 100,000 person-years was 17.1 for all melanomas and 14.6 for invasive melanomas. These rates of incidence were higher than the estimated national rate of France, and were close to incidences found in countries of Northern Europe. This might be explained by an increase in screening for melanoma, by more precise estimation of the incidence due to the capture-recapture method, or by geographic factors (mountainous area). An answer may be provided by following the variation in time of incidence and thickness of melanomas; the increase in the number of thin (low Breslow index) melanomas corresponding with increased screening.

Portal cavernoma follows a chronic occlusion of the portal vein. The long-term consequences of portal cavernoma are not well known. The objective of this study was to report the aetiology of the portal cavernoma and its natural course after excluding liver diseases causes.A single centre retrospective study based on the data collected from the radiology department of the Clermont-Ferrand hospital was conducted from 2000 to 2011. All the patients for whom an imagery found a portal cavernoma have been looked for excluding the patients having a liver disease whatever the aetiology and the Budd-Chiari syndrome.Thirty-two cases (18 women and 14 men) were selected. The mean age at diagnosis was 54.2 years and the mean follow-up period was 5.4 years. The discovery of a portal cavernoma was incidental for 8 cases. An aetiology was found for 24 cases: it was an haematological aetiology in 15 cases (10 myeloproliferative syndromes, 2 antiphospholid syndromes, 1 thalassemia major, 1 hyperhomocysteinemia, 1 prothrombin gene mutation), a general aetiology in 2 cases (1 coeliac disease, 1 pancreatic neoplasia), and a local inflammation in 7 cases. A dysmorphic aspect of the liver was noticed on medical imaging for 11 out of the 32 cases. A liver biopsy was performed in 4 patients and was normal for all of them. Sixteen patients developed oesophageal varices, 4 patients developed ascites, 3 developed asymptomatic biliary compression by the portal cavernoma, and the patient who had been followed for the longest time (15 years) developed an encephalopathy.In addition to its underlying etiology, the prognosis of portal is mainly related to the occurrence of oesophageal varices that may develop during the follow-up of the patients.

Our group has developed a new radiopharmaceutical, (123)I - N-(2-diethylaminoethyl)-2-iodobenzamide ((123)I-BZA2), a benzamide derivative able to bind to melanin pigment in melanoma cells. In a prospective and multicentric phase III clinical study, the value of (18)F-FDG PET/CT and (123)I-BZA2 scintigraphy was compared for melanoma staging.Patients with a past history of cutaneous or ocular melanoma were included from 8 hospitals. (18)F-FDG imaging was performed according to a standard PET protocol. Whole-body, static planar, and SPECT/CT (if available) images were acquired 4 h after injection of a 2 MBq/kg dose of (123)I-BZA2. (18)F-FDG and (123)I-BZA2 sensitivity and specificity for the diagnosis of melanoma metastasis were calculated and compared on both a lesion basis and a patient basis. True-positive and true-negative lesion status was determined after 6 mo of clinical follow-up or according to lesion biopsies (if available). Melanin content in biopsies was evaluated with the standard Fontana-Masson silver method and was correlated with (123)I-BZA2 uptake. Based on statistical analysis, the number of inclusions was estimated at 186.In all, 87 patients were enrolled from 2008 to 2010. Of these, 45 (52%) had metastases. A total of 338 imaging abnormalities were analyzed; 86 lesions were considered metastases, and 20 of 25 lesion biopsies found melanoma metastases. In a patient-based analysis, the sensitivity of (18)F-FDG for diagnosis of melanoma metastases was higher than that of (123)I-BZA2, at 87% and 39%, respectively (P0.05). For specificity, (18)F-FDG and (123)I-BZA2 were not statistically different, at 78% and 94%, respectively. In a lesion-based analysis, the sensitivity of (18)F-FDG was statistically higher than that of (123)I-BZA2 (80% vs. 23%, P0.05). The specificity of (18)F-FDG was lower than that of (123)I-BZA2 (54% vs. 86%, P0.05). According to biopsy analysis, only 9 of 20 metastatic lesions (45%) were pigmented with high melanin content. (123)I-BZA2 imaging was positive for 6 of 8 melanin-positive lesions, fairly positive for 3 of 10 melanin-negative lesions, and negative for 7 of 10 melanin-negative lesions. The sensitivity and specificity of (123)I-BZA2 for the diagnosis of melanin-positive lesions were 75% and 70%, respectively. Because of a low (123)I-BZA2 sensitivity, this clinical trial was prematurely closed after 87 patients had been included.This study confirms the value of (18)F-FDG PET/CT for melanoma staging and strengthens the high accuracy of (123)I-BZA2 for diagnosis of melanin-positive metastatic melanoma. Moreover, benzamide derivatives radiolabeled with therapeutic radionuclide may offer a new strategy for the treatment of metastatic melanoma patients harboring melanin-positive metastases.

TIBOLA (tick-borne lymphadenopathy) is a rickettsiosis caused chiefly by R. slovaca, transmitted by a Dermacentor tick. We report five cases.Three patients were diagnosed at the initial inflammatory stage (facial oedema, necrotic eschar, lymphadenopathy, fever) and two at the stage of sequelae (alopecia and fatigue). Microbiological evidence was present in only one case.TIBOLA is a form of rickettsiosis that is currently spreading in Europe. Clinical diagnosis is often made late because of the mild symptoms and the lack of knowledge among clinicians concerning the condition. Microbiological tests (serology, PCR, culture of eschar or serum samples) are negative in one third of cases. The reference treatment consists of antibiotics effective against intracellular bacteria, cyclines and macrolides.

Lymphomatoid contact dermatitis is a delayed hypersensitivity reaction predominantly featuring T-cell infiltration. We report a case mainly involving B-cell infiltration associated with eczema and resulting from an indirect proxy contact with an allergen in a conjugal setting.A 32-year-old man had an infiltrated cutaneous lesion on the interior aspect of the left arm with eczematous lesions of the waist and the anterior aspect of the left arm which were present for 6 months. All of these lesions were unresponsive to strong local steroids. Biopsy of the infiltrated lesion showed a dense lymphoid dermal infiltration chiefly comprising B cells. Histological examination of a waist lesion revealed chronic eczema. Patch testing was performed with the ECDRG test battery. A PPD (paraphenylenediamine) patch test was the only examination yielding a positive result. Detailed questioning revealed use of a hair dye by the patient's spouse and withdrawal of the allergen resulted in complete remission of the two types of lesion.This case highlights the ability of a single allergen to induce different lymphoid phenotypes. It also underscores the value of detailed questioning in allergology.

scleromyxoedema is characterized by dermal mucin deposition associated with a monoclonal gammopathy. This is a rare disease, mostly reported as isolated cases. There is limited data regarding the course and prognosis of the disease. The aim of this study was to determine the clinical characteristics and course of scleromyxoedema.this was a retrospective study in patients from five French university hospitals between 1987 and 2007. Data were collected using a standardized questionnaire. The inclusion criteria were based on the disease diagnosis criteria proposed by Rongioletti and Rebora: (1) generalized, papular and sclerodermiform skin eruption, (2) mucin deposition in the dermis, fibroblastic proliferation and skin fibrosis, (3) presence of a monoclonal gammopathy, (4) absence of thyroid disease.eight patients were included. The mean age at disease onset was 51.5 years (range: 35-67). The mean time from primary symptoms and diagnosis was 41.6 months (range: 4-120). Seven patients had extra-cutaneous involvement: four with peripheral neuropathy and three with interstitial pneumonia. The mean follow-up time was 9 years. Four patients improved: two experienced partial remission and two complete remission. Complete remission was obtained under treatment with dexamethasone (one patient) and thalidomide (one patient). One patient presented a myeloma and one patient presented encephalopathy leading to death.our study shows the frequency of extra-cutaneous involvement and shows that complete remission occurs in some patients.

The management of immune diseases in children remains challenging, although significant advances have been made. In addition to pharmacological approaches, extracorporeal photochemotherapy (ECP) is distinctive in its ability to provide immunomodulation without immune suppression or toxicity. However, in practice, this therapy is not widely used because of logistical issues and the lack of robust clinical pediatric studies. Here, we discuss the potential clinical applications of ECP in children and emphasize the need for a rigorous and specifically pediatric clinical evaluation of ECP.

Classification of diagnostic methods, of initial staging and of the treatment of primary cutaneous T-cell lymphomas, particularly the most common epidermotropic forms, constitutes an essential step in rationalising therapeutic practice and in evaluating the results of treatment.We carried out an analysis of the literature and of existing recommendations in order to create recommendations regarding the diagnosis, initial staging and treatment of primary T-cell lymphomas.We present the key elements of diagnosis and initial staging. The selected therapeutic strategy, which necessarily changes over time, must avoid both unnecessarily aggressive early treatment as well as an overly timid therapeutic approach that could allow lesions to rapidly progress towards more advanced stages. Regular reassessment of the benefit/risk ratio is necessary and involves the use of first- and second-line measures, in which it is difficult to establish any hierarchy, with the current tendency favouring in particular combined therapy as second-line treatment in order to limit the toxicity of each individual constituent drug within the combination. The creation of a national SPC marks significant progress in difficult cases.As a result of the offer, limited level of proof in existing studies, which are generally unsatisfactory in terms of methodology, the new recommendations described herein are timely and should be updated regularly in accordance with advances in knowledge. The organisation of clinical trials and validation of the scoring systems currently being developed should be encouraged.

To provide recommendations for the treatment of cutaneous B-cell lymphomas (CBCL).Literature review and expert opinions from the French Cutaneous Lymphoma Study Group.Diagnosis of marginal zone BCL (MZ BCL), centrofollicular BCL (CF BCL) or cutaneous large B-cell lymphoma, leg type (CLBCL, LT) is based on combination of clinical signs and histopathological features, together with B-cell clonality analyses whenever possible. Staging relies on straightforward laboratory examinations and imaging, completed in selected cases with bone marrow biopsy. Treatment may be topical, including excision, curative radiotherapy (30Gray) or adjunctive/low dose (4Gray) radiotherapy, topical corticosteroids, interferon or intralesional rituximab; or systemic, using chemotherapy and/or intravenous rituximab. For indolent forms of the disease (MZ CBCL and CF CBCL), curative (30Gray) may be given as first-line treatment in patients with localized lesions or few scattered skin lesions. For more numerous slow-growing lesions with a low tumour burden, simple monitoring with adjunctive ad hoc local treatment of individual lesions is acceptable. For multiple growing lesions, systemic rituximab or chlorambucil may be proposed. Polychemotherapy should only be used for progressive forms unresponsive to previous therapies. CLBCL LT forms are more aggressive and occur in older subjects. These lymphomas are best treated with age-adapted combinations of polychemotherapies and rituximab.Appropriate clinical trials are still needed to strengthen the levels of evidence of current recommendations.

The LRINEC score was developed in a retrospective study in order to distinguish necrotizing fasciitis from severe soft tissue infections using laboratory data.To evaluate the prognostic value of the LRINEC score in infectious cellulitis.A prospective study was performed at the departments of infectious diseases and dermatology of the Clermont-Ferrand University Hospital. The three evaluation criteria were: time from initiation of antibiotics to regression of erythema, duration of fever and occurrence of complications (abscess, surgery, septic shock, necrotizing fasciitis, death, transfer to intensive care). Potential predictive variables were: LRINEC score6 at admission, comorbidities, local appearance, clinical presentation and soft tissue ultrasound results.Fifty patients were included. The rate of complications was higher for patients with a LRINEC score6 (54%) than for patients with a score6 (12%, P=0.008). However, a LRINEC score6 on admission was not significantly associated with increased duration of erythema or of fever. Prior lymphoedema was associated with a better prognosis.The LRINEC score may be a useful tool for the detection of complicated forms of soft tissue infections. Patients with a LRINEC score6 on admission should be carefully evaluated (hospitalization, surgical assessment, close monitoring).

Dermatological treatments for the follicular occlusion triad have only partial and transient efficacy.A 20-year-old patient presented folliculitis of the scalp, acne and hidradenitis suppurativa, associated with spondyloarthritis. Treatment with infliximab for rheumatologic symptoms induced complete and lasting dermatological and rheumatological remission.The efficacy of anti-TNF-alpha in follicular occlusion triad provided confirmation that infection is not at the heart of the aetiological process. However, efficacy data is still sparse and additional studies are required.

Nephrogenic fibrosing dermopathy is a cutaneous and systemic sclerosis affecting patients with renal failure.A 68-year-old man with renal insufficiency and on dialysis developed hardening of the skin and severe joint contractions. He had previously undergone angiography with gadolinium-containing contrast agents. A skin biopsy confirmed nephrogenic fibrosing dermopathy. The patient was treated by oral steroids followed by extracorporeal photopheresis. An improvement was seen after 12 cycles.Treatment of nephrogenic systemic fibrosis is not codified and is normally based on the methods used for other forms of systemic sclerosis. Six cases of patients showing improvement under extracorporeal photopheresis have been published. The physiopathology of the disease is unknown. Gadolinium could act as a triggering agent by attracting circulating fibrocytes in the dermis of patients. Medical authorities recommend avoidance of gadolinium in patients with advanced kidney failure unless strictly necessary.

Chronic actinic dermatitis is usually controlled under systemic immunosuppressive drugs. We report herein two patients successfully treated with topical tacrolimus.Two men aged 60 and 70 years were treated for chronic actinic dermatitis for two years using systemic immunosuppressive drugs. Due to drug intolerance and treatment resistance, systemic treatment was substituted by topical tacrolimus. Cutaneous lesions improved dramatically within two months but withdrawal of daily application was immediately followed by recurrence of the lesions.Our cases, together with the 10 others reported in the literature, confirm the efficacy of topical tacrolimus in the treatment of chronic actinic dermatitis. Since tacrolimus chiefly targets activated T lymphocytes, it has been successfully deployed in various inflammatory dermatoses and its use is logical in the treatment of chronic actinic dermatitis. While dramatic improvement is achieved within a few weeks in this indication with twice-daily applications of tacrolimus ointment 0.1%, symptoms recur rapidly on dosage reduction, and extremely long-term, or even lifelong, treatment is thus probably needed. Topical tacrolimus application has been shown to be safe for periods of three years. However, the peculiar mechanism of chronic actinic dermatitis with a pronounced imbalance in T-cell subsets raises the question of theoretical risk of carcinogenicity of tacrolimus applications, and this, together with the prolonged duration of treatment, calls for long-term follow-up of chronic actinic dermatitis patients.

The predictive value of regression in melanoma is debated.A retrospective single-centre study to evaluate the correlation between regression in primary skin tumor and the presence of micrometastases in sentinel lymph nodes.Histological signs of regression in 84 melanomas (1 mm) with corresponding sentinel lymph nodes were studied by two independent pathologists.Regression was seen in 40 skin melanoma tumors while micrometastasis was seen in 24. Of the tumors with micrometastasis, only 10 were regressive (RR: 0.47, p=0.49). Breslow value2 mm and male sex were predictive for node micrometastasis (RR: 4.6, p=0.03 and RR: 7.6, p=0.006, respectively). On multivariate analysis, these two factors were independent.These data suggest that regression in primary cutaneous melanoma is not predictive for lymph node metastasis.

The immunomodulatory effect of extracorporeal photochemotherapy (photopheresis) coupled with its efficacy in lymphocytic skin diseases provides a rationale for its use for erosive lichen planus. We report two cases of chronic oral erosive and corticoresistant lichen planus successfully treated with photopheresis.Case 1. A 61-years-old man had erosive oral lichen planus for four years. Oral steroids were contra-indicated due to iatrogenic pancreatitis. After nine photopheresis sessions, subjective improvement occurred and the oral lesions were stabilized. Case 2. A 17-years-old woman presented corticodependent oral and genital erosive lichen planus and cutaneous lesions. After seven sessions of photopheresis, the patient was able to eat again and the pain decreased. After 20 sessions, the cutaneous lichen planus disappeared and complete remission of the mucous lesions was obtained without corticotherapy.These two cases, together with 23 other cases reported elsewhere, strongly suggested the value of photopheresis in the treatment of erosive lichen planus. However, relapses after treatment withdrawal appear extremely frequent.

Erosive pustular dermatosis of the legs is a rare and recently described condition seen in elderly subjects. It is characterised by symmetric erosions of the legs resulting from a confluence of sterile pustules, usually following minor trauma. Treatment with dermal corticosteroids is rapidly effective but is not codified and relapse is common.Three patients aged respectively 74, 84 and 92 years presented ulcers of the legs and ankles associated with peripheral pustules following minor injury. These pustules were sterile and exhibited a spongiform appearance on histological examination. None of these patients had a prior history of psoriasis. However, all presented zinc deficiency. Treatment with dermal corticosteroids combined with oral zinc gluconate resulted in complete resolution in two of the patients after several days of therapy and proved effective during relapse in the third patient.Erosive pustular dermatosis of the legs is a benign dermatosis that is probably under-reported that should be included in the category of inflammatory neutrophilic dermatoses. The cases we present suggest a triggering or enhancing role of zinc deficiency.

Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years.A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset ofcorticosteroid dependency, iatrogenic Cushing's syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient.Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.

The clinical and histological features of cuniculatum carcinoma (CC) are often misleading.We report a case of CC of the foot, which was misdiagnosed as osteomyelitis for a two year period and which relapsed 2 months after complete resection.The CC has to be evoked in patients with chronic osteomyelitis and torpid wound. The anatomopathologist needs to be aware of the suspected diagnosis.

Clinical features and extent of bullous pemphigoid lesions differed widely among patients. The pathogenic role of anti-BPAG2 antibodies has been recently demonstrated. The aim of this study was to analyze the relationship between clinical features of bullous pemphigoid patients and the antigens recognized by their serum.One hundred and twelve bullous pemphigoid patients were included in this prospective multicenter study. Inclusion criteria were the following: 1) diagnosis of bullous pemphigoid established on the presence of 3 of the 4 clinical features of bullous pemphigoid, histological picture of bullous pemphigoid and positive direct immunofluorescence; 2) serum available for immunoblotting studies. The clinical and biological findings were prospectively recorded on standard forms. Sera were collected and analyzed using indirect immunofluorescence and immunoblotting on human epidermal extracts.Analysis of patient's clinical features depending on the antigens recognized by their serum showed that patients whose serum contained anti-BPAG1 antibodies had more frequently pruritus, blisters on the lower limbs and a positive indirect immunofluorescence. Patients whose serum contained anti-BPAG2 antibodies had blisters more frequently localized on the head, and a more frequently negative indirect immunofluorescence. Patients whose serum was negative by immunoblotting had less frequently urticarial and/or eczematous lesions, bullae less frequently localized on the lower part of the trunk, abdomen and lower limbs, lower eosinophilia and a more frequently negative indirect immunofluorescence.Patients with circulating anti-BPAG1 antibodies exhibited the most typical, clinical and biological features of bullous pemphigoid.

Iodobenzamides are reported to possess an affinity for melanoma. A first selected compound, BZA, was studied in a phase 2 clinical trial on 159 patients as an imaging agent for the detection of primary melanoma and metastases with good results. We report the results of a second phase 2 clinical trial on 40 patients with a new radiopharmaceutical BZA2 (an orthoiodinated BZA analog), which was expected to provide quality images sooner after injection and with better imaging contrast.Performance was evaluated in 40 patients classified with primary ocular lesions (12), suspicion of metastases of ocular or cutaneous origin (15), or with no known secondary lesion (13), and results were compared with conventional investigation techniques (ophthalmoscopy, ultrasonography, and angiography for ocular melanoma, whole-body CT scan and ultrasonography for metastases).No adverse events were recorded. The overall results on a per patient basis showed a sensitivity of 78% and a specificity of 95%. The four false negatives observed were ocular lesions (three with a thickness3mm and one achromic), but all the proven secondary lesions were imaged. Moreover, negative BZA2 scintigraphy in cases of suspicious lesions led to the correction of two diagnoses: the prostatic origin of bone metastases and the endocrine tumor origin (APUD system) of an ocular lesion.BZA2 scintigraphy is an easy test with good tolerance. In the diagnosis of ocular primary melanoma, the sensitivity of the test is 64%, although limited by the thickness (3mm) and the pigmentation of the lesion. However, the BZA2 scintigraphy is a very useful test for the detection of melanoma metastases, with a sensitivity of 100% and a specificity of 95%.BZA2 scintigraphy showed good tolerance in patients and it appears promising for differential diagnosis, staging, and restaging of melanoma.

Background: We previously proposed a set of 4 clinical criteria for the diagnosis of bullous pemphigoid (BP) that consisted of age greater than 70 years, absence of atrophic scars, absence of mucosal involvement and absence of predominant bullous lesions on the neck and head. These results have been challenged because direct immunoelectron microscopy (IEM), which was used as the standard diagnostic criterion in our initial study, does not identify the different antigens of the basement membrane zone. Objective: To reassess the validity of these clinical criteria for the diagnosis of BP using immunoblot analysis of patient sera as the main diagnostic criterion, in order to precisely identify the antigens recognized by patient sera. Methods: One hundred and eighty-nine sera from patients with various subepidermal autoimmune blistering diseases (AIBDs) were tested by immunoblotting using dermal and epidermal extracts. IEM was used as a complementary diagnostic procedure in a few patients whose serum recognized BPAG2 exclusively or was negative in immunoblotting. Results: 142 patients (75%) had at least 3 of the 4 clinical diagnostic criteria. Sera from patients who lacked the set of BP clinical criteria were more frequently immunoblot negative (34%) than sera from patients who had the criteria (18%; p = 0.025). BPAG1 was more frequently recognized by sera from patients with the set of BP clinical criteria (78%) than by sera from patients without the criteria (45%; p = 5·10–4). In contrast, BPAG2 was recognized by a great number of sera from patients who lacked the criteria of BP (71%), which was in accordance with the presence of numerous patients with cicatricial pemphigoid in this group. Among patients with various subepidermal AIBDs, the diagnosis of BP could be made with a sensitivity of 86%, a specificity of 90% and an excellent prognostic positive value over 95%, if 3 of these clinical criteria were present. Conclusion: These results confirm the interest of this set of clinical criteria for the rapid diagnosis of BP.

Intraoperative fluoroscopy imaging during coronaroplasty or transjugular intrahepatic portosystemic shunt may induce chronic radiodermatitis. Temporary hair loss is a peculiar form of radiodermatitis following endovascular surgery of the cerebral arteries.Six patients (2 women, 4 men, age range: 27-47 years old) were seen for a solitary plaque of alopecia. In all of the cases, the plaque had appeared two weeks after a neuroradiologically guided embolization procedure. No other skin lesions were seen. Alopecia spontaneously and completely regressed within three to four months. However, it reappeared after a subsequent embolization (one case) but not after arteriographies (five cases).Five similar cases have been reported in the literature. Transient alopecia often occurs after neurologically guided endovascular surgery of the cerebral arteries. This side-effect is well known by neurosurgeons and thus, these patients are rarely referred to a dermatologist. Two differential diagnoses must be evoqued: alopecia aerata and anticonvulsant - induced alopecia. The role played as cofactor by carbamazepine which is a photosensitivant drug, is discussed.

Henoch-Schonlein purpura is a rare eventuality in adulthood. Abdominal involvement can worsen the short-term prognosis and justify medical treatment to avoid surgery.Abdominopelvic computed tomography (CT) was performed in six adult patients (four men and two women; age range, 19-74 years) with Henoch-Schonlein purpura before any treatment. Abdominal symptoms were concomitant with the purpuric rash in two cases and followed it in the other cases.The small bowel was always involved with a parietal thickening, and the target sign was observed in three patients. The lesions were located in the duodenum in two patients, the jejunum in two and the ileum in five. Two segments were involved in one patient and three segments in another patient. A peritoneal effusion was always present. No colonic lesion was discovered on CT. Once the results were known, all patients received steroids with initially intravenous methylprednisolone in five patients at doses ranging from 80 to 1,000 mg/day. On follow-up, none of the patients underwent surgery.Abdominopelvic CT scan demonstrated signs suggestive of intestinal vasculitis. An underlying neoplasia or an eventual other cause of abdominal pain were explored and a steroid therapy was then began with a good outcome. Abdominal CT can be helpful in the diagnosis of abdominal involvement in Henoch-Schonlein purpura.

Scleromyxedema is the consequence of a dermal infiltration by mucine, commonly associated with a monoclonal gammapathy of unknown significance.A 46 year-old woman was hospitalized for a scleromyxedema with a bilateral macular edema and a restricted pulmonary syndrome. A quite complete cutaneous response and a complete ocular and pulmonary response were obtained after 12 extracorporeal photopheresis courses and 4 flashes of prednisolone (17 months follow-up).There is no consensus on guidelines for the treatment of scleromyxedema. Steroids and melphalan are usually indicated. However these drugs induce severe side-effects. In the absence of controlled studies concerning the efficiency of the different drugs used in scleromyxedema, the main advantage of photopheresis lies in the safety of this procedure.

Drug hypersensitivity syndrome and drug-induced cutaneous pseudolymphoma were for a long time considered as one and the same. Today, however, two clinical entities have been distinguished; the former is responsible for a mortality rate estimated at 10% whereas the prognosis of the latter is always excellent. Acute generalized exanthematous pustulosis is a rare drug eruption individualised in 1980. Numerous drugs can induce acneiform eruptions or trigger or even worsen an authentic acne. The number of drugs responsible for buccal mucous membrane involvement is on the increase; gingival hypertrophy remains a rare drug reaction, a certain number of drugs such as nicorandil can cause buccal aphtae and even giant ones.

Angioneurotic edema (AE) is a rare but severe disease. Hereditary AE is the more well-known form. The acquired form is exceptional: the symptoms are the same but there are some biologic and treatment differences. We investigated the clinical and biochemical features in nine patients with acquired angioneurotic edema (AAE).Four of the patients with type I AAE presented an accelerated metabolism of C1Inh, associated with a hematology disease. Their C4, C1q and C1Inh plasma levels were low. Four patients had type II AAE associated with an autoantibody to C1Inh. Their C1Inh plasma levels were normal or low but the functional levels were low in all four. One patient had AAE induced by oral contraceptives. The C1Inh plasma level was normal but the functional level was very low; there were no autoantibodies. Symptoms resolved when oral contraceptives were withdrawn and the C1Inh level returned to normal.Treatment of AAE is a difficult matter. For type I AAE, it consists in treating the associated disease. For type II AAE, the treatment goal is to lower the autoantibody level. Management of these diseases requires close collaboration between clinicians and biologists.

Graft versus host reaction is a life-threatening complication of allogenic bone marrow transplantation. Extracorporeal photopheresis has been used for some years in the treatment of graft versus host reaction. We report on three children treated with extracorporeal photopheresis for a graft versus host reaction resistant to immunosuppresive drugs.Three children with a graft versus host reaction were submitted to 18, 30 and 46 extracorporeal photopheresis courses respectively. In the same time, the other immunosuppressive treatments were tapered or definitively stopped (ciclosporin).A dramatic improvement of cutaneous status and biological data was observed after the first courses. However, the extracorporeal photopheresis treatment did not improve the mucous lesions. No serious adverse effect was encountered.As published elsewhere, extracorporeal photopheresis was effective on the graft versus host reaction lichenoid cutaneous lesions and in case of visceral involvement. In all of our cases, the immunosuppressive drug could have been tapered. No adverse event was observed. Thus, extracorporeal photopheresis should be indicated in case of resistance to immunosuppressive drugs.

Drug-induced cutaneous pseudolymphomas are poorly reported. They clinically and pathologically mimic malignant lymphomas but their evolution is benign after drug withdrawal.We herein reported a case of carbamazepine++-induced pseudolymphoma in a 30 year-old woman of particular interest because of the paucity of clinical symptoms (only six papules of less than one centimetre each).Phenytoins, carbamazepine++, barbiturates and ACE inhibitors are the main drugs inducing cutaneous pseudolymphomas. These usually mimic T-cell lymphomas. The clinical spectrum of cutaneous pseudolymphomas is broad including severe aspects, such as erythroderma or tumoral eruption and benign appearing one as in our case. Drug investigation is mandatory for each patient with lymphoma appearing cutaneous infiltrates because dramatic and definitive healing of the lesion is always expected in case of pseudolymphoma.

Transient bullous dermolysis of the newborn is a bullous eruption limited to friction zones. It appears at birth and disappears during the first months of life.Immediately after delivery, an infant girl presented cutaneous bullae on areas of trauma which spontaneously regressed after a few weeks. The histology examination confirmed subepidermal involvement (the roof of the bullae took up the anticollagen IV antibody) and ultrastructure anomalies in the baseline membrane: intracellular vacuoles in the keratinocytes containing fibrillary material, disorganization of the anchoring fibers.Transient bullous dermolysis of the newborn is a rare (less than 15 cases reported in the literature) benign disease which regresses spontaneously, possibly an explanation of the small number of cases reported. The anomalies in the ultrastructure observed in the baseline membrane strongly suggest transient impairment in collagen VII maturation and excretion. These anomalies are not pathognomonic and can be observed in dystrophic bullous epidermolysis. Currently there is no specific genetic marker to established transient bullous dermolysis as unique entity.

Cutaneous lymphomas occurring in HIV infection are a rare disease. Most of them are high grade lymphomas with fulminant course and poor prognosis.Evaluate clinical and histological aspects as well as immunophenotype and evolution of these lymphomas.Eight patients with HIV infection were studied between 1992 and 1994. The clinical and histological features were reviewed by the members of the French Study Group for Cutaneous Lymphomas. Staging procedures for lymphomas were performed in 7/8 patients.Seven non epidermotropic lymphomas and one mycosis fungoides were reviewed. Patients were male (6 cases) and female (2 cases); their mean age was 45 years (27-63). The mean level of CD4 T cells/mm3 was 141 (20-380). Only one patient presented with extracutaneous lesions. These lesions were similar to seronegative patients, but unusual features were observed in two cases. Histological classification showed high grade lymphomas in 6/8 cases. The immunophenotype was: T-cell lymphoma in 4 cases, B-cell lymphoma in 3 cases; it could not be determinated in one case. Six patients died. The median of survival is 8 months in this series.Our series confirms the predominance of high grade lymphomas presenting in the skin. The T-cell phenotype is more frequent. The onset of a cutaneous lymphoma has a poor prognosis in HIV infection. Most of our patients had localised disease at presentation. Therapeutic management of these lymphomas must be codified.

Pachydermoperiostosis (PDP) is a rare genetically determined disease belonging to the group of hypertrophic osteoarthropathies. Its aetiopathogenesis remains unclear. Most hypotheses favour an exogenous stimulation of fibroblasts.A clinically typical patient with PDP was studied by electron microscopy with particular reference to the dermis and its cellular constituents. Fibroblasts from involved skin were cultured and studied in comparison with control cells.Remarkable modifications of the structure of the dermis were observed, encompassing irregular caliber of collagen fibres, extracellular deposits of microfibrils and of amorphous granular substance corresponding to the Alcian blue positive deposits seen by conventional histochemistry. The in vitro growth of fibroblasts was normal.Authors reviewed aetiopathogenic hypotheses. Our data suggest a genetically determined alteration of extracellular matrix production by fibroblasts as a possible explanation for the development of PDP.

The changes in the immune system during the ageing process have been described in numerous publications, which are sometimes contradictory. The study of cell mediated immunity and of hormonal immunity provide only a partial reflection of the smooth working of the cutaneous immune system, which is dependent on fragile cell interactions between T lymphocytes, cells of Langerhans and keratinocytes, which involves numerous soluble mediators. Three possible mechanisms leading to or contributing to the decline in immune function with age should be considered: a modification of the distribution of lymphocyte subpopulations (the T helper lymphocytes and T-suppressing lymphocytes are affected by age in particular), changes in the mechanisms of suppression and changes in the maturation of B and T lymphocytes.

Genomic DNA digests of skin biopsies from 20 patients with cutaneous T-cell lymphomas and pseudolymphomas were studied by hybridization, using probes for the constant region of the T-cell receptor beta chain and the joining region of the immunoglobin heavy chain gene. Skin biopsies from all 20 patients contained a monoclonal T-cell population. In addition, DNA from 5 patients contained an immunoglobulin gene rearrangement. These results demonstrate that cutaneous T-cell lymphomas are clonal T-cell malignancies that frequently express a dual genotype, which may sometimes reflect the clonotypic heterogeneity of these disorders.