Childhood epidermal necrolysis and erythema multiforme major: a multicentre French cohort study of 62 patients.

INTRODUCTION: The distinction between epidermal necrolysis [EN; including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) and overlap syndrome] and erythema multiforme major (EMM) in children is confusing. We aimed to better describe and compare these entities. MATERIALS AND METHODS: This French retrospective multicentre study included children ≤18 years old referred for EN or EMM between 1 January 2008 and 1 March 2019. According to pictures, children were reclassified into TEN/overlap, SJS or EMM/unclassified (SJS/EMM) groups and compared for epidemiological and clinical data, triggers, histology and follow-up. RESULTS: We included 62 children [43 boys, median age 10 years (range 3-18)]: 16 with TEN/overlap, 11 SJS and 35 EMM. The main aetiologies were drugs in EN and infections (especially Mycoplasma pneumoniae) in EMM (P < 0.001), but 35% of cases remained idiopathic (TEN/overlap, 47%; SJS, 24%; EMM, 34%). The typical target lesions predominated in EMM (P < 0.001), the trunk was more often affected in EN (P < 0.001), and the body surface area involved was more extensive in EN (P < 0.001). Mucosal involvement did not differ between the groups. Two patients with idiopathic TEN died. Histology of EMM and EN showed similar features. The recurrence rate was 42% with EMM, 7% with TEN/overlap and 0 with SJS (P < 0.001). Sequelae occurred in 75% of EN but involved 55% of EMM. CONCLUSION: Clinical features of EN and EMM appeared well demarcated, with few overlapping cases. Idiopathic forms were frequent, especially for EN, meaning that a wide and thorough infectious screening, repeated if needed, is indicated for all paediatric cases of EN/EMM without any trigger drug. We propose a comprehensive panel of investigations which could be a standard work-up in such situation. Sequelae affected both EN and EMM.

[Diffused ulcerated cutaneous sarcoidosis occurring on top of scars]. / Sarcoïdose cutanée ulcérée et diffuse survenant sur cicatrices.

INTRODUCTION: Sarcoidosis in patients of African or Afro-Caribbean origin presents semiological characteristics, together with polymorphic skin lesions, more extensive forms and a poorer prognosis. Ulcerated and particularly diffuse skin forms are very rare. Herein we report a case of diffuse ulcerated skin sarcoidosis appearing on top of old scars in a female patient of Afro-Caribbean origin. PATIENTS AND METHODS: A 16-year-old Caribbean girl consulted for multiple painful papules that appeared on old burns scars. The skin biopsy was consistent with sarcoidosis. At the same time, the patient developed sarcoid uveitis, and sarcoid dactylitis was suspected. Shortly thereafter, painful and diffuse ulcerations appeared on pre-existing skin lesions. Systemic corticosteroid therapy resulted in clinical improvement, but corticosteroid-dependency warranted subsequent initiation of methotrexate. DISCUSSION: Ulcerated cutaneous forms of sarcoidosis are rare and are often associated with severe forms, and they should prompt screening for systemic damage. There do not appear to be any previous reports of the presentation of ulcerated and extensive skin sarcoidosis presenting on scars, as described in the present study.

Distinctive cutaneous and systemic features associated with specific antimyositis antibodies in adults with dermatomyositis: a prospective multicentric study of 117 patients.

BACKGROUND: Identification of myositis-specific autoantibodies (MSAs) for dermatomyositis (DM) could allow the characterization of an antibody-associated clinical phenotype. OBJECTIVE: We sought to define the clinical phenotype of DM and the risk of cancer, interstitial lung disease (ILD) and calcinosis based on MSA. METHODS: A 3.5-year multicentre prospective study of adult DM patients was conducted to determine the clinical phenotype associated with MSAs and the presence of cancer, ILD and calcinosis. RESULTS: MSAs were detected in 47.1% of 117 included patients. Patients with antimelanoma differentiation-associated protein-5 antibodies (13.7%) had significantly more palmar violaceous macules/papules [odds ratio (OR) 9.9], mechanic's hands (OR 8), cutaneous necrosis (OR 3.2), articular involvement (OR 15.2) and a higher risk of ILD (OR 25.3). Patients with antitranscriptional intermediary factor-1 antibodies (11.1%), antinuclear matrix protein-2 antibodies (6.8%) and antiaminoacyl-transfer RNA synthetase (5.1%) had, respectively, significantly more poikiloderma (OR 5.9), calcinosis (OR 9.8) and articular involvement (OR 15.2). Cutaneous necrosis was the only clinical manifestation significantly associated with cancer (OR 3.1). CONCLUSION: Recognition of the adult DM phenotype associated with MSAs would allow more accurate appraisal of the risk of cancer, ILD and calcinosis.

Squamous cell carcinoma in the Afro-Caribbean community: an 11-year retrospective study.

BACKGROUND: Squamous cell carcinoma (SCC) is considered the most frequent skin cancer in Black people. Its incidence is not known in the Afro-Caribbean population. OBJECTIVE: To assess the incidence of SCC in Guadeloupe, the largest island of the Lesser Antilles (405 000 inhabitants, mostly Black people of African and European descent). The second objective was to characterize clinical and histological patterns of SCC occurring in the Afro-Caribbean community. METHODS: This retrospective study was conducted over an 11-year period (2000-2010). Data regarding 723 histological confirmed cases of SCC identified using the three Guadeloupean pathology laboratories' computerized databases were retrieved from the records of 551 patients. Private practice dermatologists and general practitioners were contacted to obtain any missing data. RESULTS: The annual age-adjusted incidence of SCC was 15 per 100.000 residents in Guadeloupe. In the Afro-Caribbean community, SCC had a greater size (i.e. 2.8 ± 2.8 cm vs. 1.5 ± 1.0 cm, P < 0.001), was more often located on the anogenital area (i.e. 48/79-60.8% vs. 14/320-4.4%, P < 0.001) in association with an underlying dermatosis due to HPV infection (15/71-21.1% vs. 3/366, 0.8%, P < 0.001) and led more frequently to metastasis (13/84-15.5% vs. 10/366-2.7%, P < 0.001) and/or fatal evolution (11/83-13.3% vs. 7/365-1.9%, P < 0.001). CONCLUSIONS: The results of this original study, which first estimated the incidence of SCC in West Indies, suggest that anogenital examination should be routinely performed in skin cancer screening of Afro-Caribbean people to detect the presence of SCC at an early stage. IMPLICATION FOR PRACTICE: Squamous cell carcinoma is the most frequent skin cancer in Black people. Its incidence is not known in the Afro-Caribbean population. In Guadeloupe, the largest island of the Lesser Antilles, the annual age-adjusted incidence of SCC was estimated to be 15.0 per 100 000 residents, 95% CI:[13.8; 16.2]. In the Guadeloupean Afro-Caribbean community, SCC seems to more frequently occur in the anogenital area, due to HPV infection. These results support to include a routine genital urinary examination in the skin cancer screening of people of Afro-Caribbean descent.

[Efficacy of hyperbaric oxygen therapy in the treatment of ischemic toe ulcer in a patient presenting systemic sclerosis]. / Efficacité de l'oxygénothérapie hyperbare dans le traitement d'un ulcère nécrotique d'orteil au cours d'une sclérodermie systémique.

BACKGROUND: The medical treatment of ischemic ulcers in patients with systemic sclerosis remains difficult. Despite the major help provided by vasodilator treatments, the risk of spontaneous or surgical amputation remains high. OBSERVATION: A 48-year-old female patient from Guadeloupe was treated in our department for diffuse systemic sclerosis present for 15 years complicated by lung, joint and digestive involvement, and associated with severe Raynaud's phenomenon. The clinical course was marked by the occurrence of multiple ischemic ulcers, which were resistant to conventional medical treatment and resulted in two surgical amputations (to the 2nd and 3rd interphalangeal joints of the toes of the left foot). Treatment with an endothelin-receptor antagonist and a calcium inhibitor was then introduced for secondary prevention. Two years later, the patient consulted for a further ischemic ulcer of the left 4th toe. She refused the proposed treatment with iloprost. Because of the unfavorable outcome and the absence of therapeutic alternative to amputation, hyperbaric oxygen therapy was initiated. Thirty 90-minutes sessions of pure oxygen at 2.5 ATA were conducted over a 10-week period. Complete healing was obtained after 8 months. DISCUSSION: We report herein a clinical case illustrating the efficacy of hyperbaric oxygen therapy for the treatment of ischemic ulcers of the toes in systemic sclerosis. It could offer an alternative therapeutic option, in particular for patients presenting resistant ischemic ulcers and a contraindication for or intolerance to the conventional medical treatment.

[Incidence of skin and non-skin cancers in Afro-Caribbean renal transplant recipients: Guadeloupe data from 2004 to 2011]. / Incidence des cancers cutanés et non cutanés chez les transplantés rénaux afro-caribéens : données guadeloupéennes de 2004 à 2011.

BACKGROUND: Cancer is the main complication of transplantation surgery. The literature concerning renal transplant recipients among the Afro-Caribbean population is scant. The aim of this study was to determine the incidence of cancer in these patients, with the secondary objective being to identify predisposing factors for cancer. PATIENTS AND METHODS: This was an epidemiological and retrospective study that included all Guadeloupians of phototype V-VI undergoing renal transplantation from 01/01/2004 to 31/12/2011. Skin cancer screening was performed before transplantation and during an annual dermatological consultation following transplantation. Screening for non-cutaneous cancers was guided by clinical symptoms or by the results of the screening examinations recommended in the current guidelines. At the study time-point (31/12/2011), all patients were examined by a dermatologist. RESULTS: One hundred and two patients were included : 42 women and 60 men (mean age: 52.1±11.6 years at transplantation). Eight cancers were diagnosed. The cumulative incidence of cancer was 7.8% at 3 years. Three factors were associated with more rapid onset of cancer: personal history or familial history of cancer, and genital lesion induced by HPV. CONCLUSION: Our results suggest a low incidence of cancer in Afro-Caribbean renal transplant patients. Personal or family history of cancer and HPV-induced genital lesions would appear to accelerate the onset of cancer in this population.

[Prevalence of cancer in the Afro-Caribbean population presenting dermatomyositis and anti-synthetase syndrome: a preliminary study conducted at Pointe-à-Pitre University Hospital, 2000-2012]. / Prévalence des cancers dans la population afro-caribéenne atteinte de dermatomyosite et de syndrome des anti-synthétases : étude préliminaire au CHU de Pointe-à-Pitre, 2000-2012.

BACKGROUND: An association with cancer is described in 17-32% of cases of dermatomyositis (DM) and in 5-16% of cases of anti-synthetase syndrome (ASS). The literature contains very few studies involving Afro-Caribbean patients with DM or ASS. The aim of our retrospective study was to determine the prevalence of cancer in a series of patients with DM or ASS at the University Hospital of Pointe-à-Pitre between 1st January 2000 and 31st December 2012. The secondary objective was to review the clinical and laboratory features as well as the course of DM/ASS in these patients. PATIENTS AND METHODS: The inclusion criteria were as follows: Afro-Caribbean origin; age >15 years; patient living in Guadeloupe; screening for malignancy. RESULTS: Twenty-two patients were included (15 DM, 7 ASS). Only one case of cancer was diagnosed in the entire study population at a mean follow-up of 6 ± 4 years (prevalence: 6.7%, CI95% [1.7-31.9]). Of the 15 patients presenting DM (sex ratio F/M: 4, mean age: 45 ± 14 years), 6 (40%) had associated connective tissue disease. CONCLUSION: Our study suggests a weak association between DM and cancer in Afro-Caribbean patients. These results may be explained by the features of the disease seen in these patients (female gender, young age at onset, associated connective tissue disease) and the low prevalence in the Caribbean region of cancers typically associated with DM.

[Diagnosis of skin cancer by dermatologists in the French West Indies: a prospective study]. / Étude prospective de l'incidence des cancers cutanés dépistés en pratique dermatologique aux Antilles françaises.

BACKGROUND: The incidence of skin cancer is not well established in the French West Indies, aside from old data concerning cutaneous melanoma. METHOD: A prospective study was performed over a 3-month period in late 2007 in the French West Indies. The number of new cases of histologically confirmed skin cancer was determined using a questionnaire. RESULTS: The rate of participation of dermatologists was 82 %. During the study period, 166 skin cancers were diagnosed in 134 patients (66 women and 68 men: mean age=63.3 years, SD=2.5), including 137 basal cell carcinomas, 12 melanomas, seven squamous cell carcinomas, six Bowen's disease, one B lymphoma and one Paget's disease. The raw incidence of skin cancers detected by dermatologists was calculated as 64.6 cases/100 000 inhabitants/year for basal cell carcinoma, 5.7 cases/100 000 inhabitants/year for melanoma, 3.3 cases/100 000 inhabitants/year for squamous cell carcinoma, and 2.8 cases/100 000 inhabitants/year for Bowen's disease. Seven melanomas (almost exclusively of plantar topography) and seven basal cell carcinomas were diagnosed in patients of phototype V or VI. DISCUSSION: Although lower than in metropolitan France, the number of skin cancers diagnosed by dermatologists in the French West Indies is fairly high. In addition, this study indicates the significant incidence of basal cell carcinomas and melanomas in subjects with phototype V or VI, underreported in the literature. These findings highlight the need to begin longer-term studies and to include skin carcinomas in the cancer registry of the French West Indies in order to better identify their characteristics among the Caribbean population.

Management of ocular involvement in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: french national audit of practices, literature review, and consensus agreement.

Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) can lead to severe ophthalmologic sequelae. The main risk factor is the severity of the initial ocular involvement. There are no recommendations for ocular management during acute phase.We conducted a national audit of current practice in the 11 sites of the French reference center for toxic bullous dermatoses and a review of the literature to establish therapeutic consensus guidelines. We sent a questionnaire on ocular management practices in SJS/ TEN during acute phase to ophthalmologists and dermatologists. The survey focused on ophthalmologist opinion, pseudomembrane removal, topical ocular treatment (i.e. corticosteroids, antibiotics, antiseptics, artificial tear eye drops, vitamin A ointment application), amniotic membrane transplantation, symblepharon ring use, and systemic corticosteroid therapy for ophthalmologic indication. Nine of 11 centers responded. All requested prompt ophthalmologist consultation. The majority performed pseudomembrane removal, used artificial tears, and vitamin A ointment (8/9, 90%). Combined antibiotic-corticosteroid or corticosteroid eye drops were used in 6 centers (67%), antibiotics alone and antiseptics in 3 centers (33%). Symblepharon ring was used in 5 centers (55%) if necessary. Amniotic membrane transplantation was never performed systematically and only according to the clinical course. Systemic corticosteroid therapy was occasionally used (3/9, 33%) and discussed on a case-by-case basis.The literature about ocular management practice in SJS/ TEN during acute phase is relatively poor. The role of specific treatments such as local or systemic corticosteroid therapy is not consensual. The use of preservatives, often present in eye drops and deleterious to the ocular surface, is to be restricted. Early amniotic membrane transplantation seems to be promising.

[Large-vessel granulomatous vasculitis during the course of sarcoidosis: Takayasu's arteritis?]. / Angéite granulomateuse au cours d'une sarcoïdose : maladie de Takayasu ?

BACKGROUND: Vascular involvement in sarcoidosis is very rare and is characterized by preferential involvement of large vessels similar to that observed in Takayasu's disease. Distinguishing between these two diseases is often difficult and constitutes a diagnostic pitfall. The association between sarcoidosis and Takayasu's arteritis is not coincidental and a common physiopathological factor may exist; it suggests a possible aetiopathogenetic relationship between sarcoidosis and Takayasu's arteritis and casts doubt on whether this form of vasculitis is a disease in its own right or simply a syndrome caused by other diseases. CASE REPORT: We report the case of a man with a 10-year history of cutaneous and pulmonary sarcoidosis who developed ischaemia of the right upper limb evocative of Takayasu's arteritis. The patient was successfully treated with oral steroids and methotrexate. DISCUSSION: This case prompts discussion about the relationship between Takayasu's disease and sarcoidosis. Physicians should be aware of the possible occurrence of granulomatous arteritis during the course of sarcoidosis which requires a special work-up.

[Off-label use of intravenous immunoglobulin therapy in the treatment of lupus myocarditis: Two case reports and literature review]. / Utilisation hors AMM d'immunoglobulines intraveineuses dans le traitement de la myocardite lupique : à propos de deux cas et revue de la littérature.

INTRODUCTION: Several case reports have reported the benefit of intravenous immunoglobulin therapy in many autoimmune diseases, including systemic lupus erythematosus. CASE REPORTS: Here, we report on two cases of lupus myocarditis treated with high dose of intravenous immunoglobulin. The first patient was a 42-year-old woman who presented with lupus myocarditis that was resistant to corticosteroids and cyclophosphamide, and who was finally successfully treated with a single dose of 2 g/kg of intravenous immunoglobulin. The patient displayed clinical improvement a few days later. The second case - a 43-year-old woman was diagnosed with lupus myocarditis and immunosuppressive drugs were contraindicated because of the context of a recent infective endocarditis. She was treated with repeated dose of 2 g/kg of intravenous immunoglobulin. Clinical improvement was observed and the left ventricular ejection fraction increased from 20 % to 60 % within a few days. We also report 9 similar observations identified from a literature review. CONCLUSION: The use of intravenous immunoglobulin in lupus myocarditis is not officially recognized but could be considered as an alternative when conventional therapies have failed or are contraindicated.

[Hypopigmented mycosis fungoides]. / Mycosis fongoïde hypopigmenté

INTRODUCTION: Hypopigmented mycosis fungoides is a rare clinical form of the disease, first described in 1978. Since then, only a hundred odd cases have been documented. CASE REPORT: A young 19 year-old African woman had presented with hypochromatic macules since the age of 9 and for which the diagnostic enquiry had finally concluded in hypopigmented mycosis fungoides. DISCUSSION: The particularities of this form of mycosis fungoides, grade I according to the TNM classification, are principally its onset in black-skinned persons or of Asian origin, and the age of early onset with a predominance of pediatric cases. Its course is indolent for several years and thus source of delayed diagnosis. The differential diagnoses to be evoked are basically vitiligo, achromate eczematides and parapsoriasisis. The post-inflammatory depigmentation, frequent in black-skinned subjects, is only an eliminating diagnosis. Its treatment is that of classical Grade I mycosis fungoides: topical mechlorethamine, phototherapy and topical corticosteroids.

[Study of the clonality of cutaneous and blood lymphocytes during drug-induced hypersensitivity in 6 patients]. / Etude de la clonalité des lymphocytes cutanés et sanguins au cours du syndrome d'hypersensibilité médicamenteuse chez 6 malades.

INTRODUCTION: The drug-induced hypersensitivity syndrome or DRESS (drug reaction with eosinophilia and systemic symptoms) is a severe toxiderma because it is accompanied by lethal visceral involvement in 6 to 10% cases. Its physiopathology remains unclear. In order to specify the immunological characteristics of this toxiderma we analyzed, prospectively, the rearrangement of the blood and cutaneous T-cell lymphocyte receptor (TCR) genes of patients exhibiting a drug-induced hypersensitivity syndrome between April 1998 and April 2000. PATIENTS AND METHODS: The inclusion criteria were: age over 18 years, occurrence of a drug-induced generalized eruption, existence of associated systemic involvement (lymph node or visceral), and presence of hypereosinophilia greater than 0.5 G/l and/or circulating atypical lymphocytes. Six patients (3 men and 3 women), with a mean age of 54 years were included. The imputable drug was an anti-seizure in 3 cases, allopurinol in 2 and oxazepam in one. Remission occurred within a delay of 10 to 30 days after the acute phase. Two patients presented several flares. RESULTS: No clonal rearrangement in TCR genes was detected in the cutaneous samples. A clonal rearrangement of TCR genes was initially detected in the blood lymphocytes of 3 out of the 6 patients (allopurinol: n=2 and oxazepam: n=1). The latter remained detectable during the evolution, during the second or third flare of the drug-induced hypersensitivity in 2 patients (allopurinol: n=1 and oxazepam: n=1). DISCUSSION: The presence of circulating T-cell clones detectable for several months after the occurrence of a drug-induced hypersensitivity shows the mono or oligoclonal expansion of activated T-cells, induced by the drug imputed. Their persistence over several months corresponds to a remnant activation of the immune system that can explain the prolonged and/or recurrent evolution of the drug-induced hypersensitivity syndrome in some patients.

[Detection of clonal T-cell receptor gamma gene rearrangement with the use of PCR-DGGE for diagnosis of erythroderma]. / Réarrangement des gènes du récepteur des lymphocytes T cutanés par la technique de PCR-DGGE pour le diagnostic étiologique des érythrodermies.

BACKGROUND: It is often difficult to establish the etiological diagnosis of erythroderma because clinical findings and immunohistology cannot always distinguish between lymphomatous erythroderma and inflammatory erythroderma. The purpose of this work was to assess the contribution of PCR-DGGE for detecting clonal T-cell receptor gamma gene rearrangement to the etiological diagnosis of erythroderma. PATIENTS AND METHODS: The following inclusion criteria were used: patient with erythroderma; skin biopsy for histologic study, immunophenotyping and molecular biology; minimal follow-up of 12 months after initial diagnosis. Thirty patients were included from May 1, 1995 to November 30, 1998. Histology slides were reread by one of the authors blinded to other data who classed them in three categories: probable lymphoma, probable inflammatory disease, uncertain diagnosis. Molecular data were also analyzed in the same blinded manner. Immunohistology diagnosis was compared with the molecular data and the final diagnosis retained from clinical, histological and molecular findings as well as the disease course to last follow-up (November 1, 1999) after a mean 12 +/- 18 months follow-up. RESULTS: Eight biopsies were classed as probable lymphomas; a T-cell clonal rearrangement of the TCR genes was detected in 7/8 cases. The one sample with no detectable T clone was a drug-induced Sézary pseudolymphoma. The histologial classification identified 16 cases of probable inflammatory disease; no clonal rearrangement of the TCR genes was found in these cases. One of these patients had fungoid mycosis treated with caryolysin for three months and developed treatment intolerance at the time of the skin biopsy. For six biopsies the histological diagnosis was "uncertain"; a clonal rearrangement of the TCR genes was found in 2/3 of the fungoid mycosis cases and in none of the three cases of toxic dermal reactions. DISCUSSION: This study demonstrated the contribution of genotypic analysis with PCR-DGGE to the diagnosis of erythroderma. Monoclonal TCR gene rearrangement was detected in 9/11 (82 p. 100) of the patients with lymphoma and in 0/19 of the patients with an inflammatory dermatosis. The etiological diagnosis of erythroderma is an excellent indication for molecular stud of skin biopsies with PCR-DGGE.