Flow-based basophil activation test in immediate drug hypersensitivity. An EAACI task force position paper.

Diagnosing immediate drug hypersensitivity reactions (IDHRs) can pose a significant challenge and there is an urgent need for safe and reliable tests. Evidence has emerged that the basophil activation test (BAT), an in vitro assay that mirrors the in vivo response, can be a complementary test for many drugs. In this position paper, members of Task Force (TF) "Basophil activation test in the evaluation of Drug Hypersensitivity Reactions" from the European Academy of Allergy and Clinical Immunology (EAACI) present the data from a survey about the use and utility of BAT in IDHRs in Europe. The survey results indicate that there is a great interest for using BAT especially for diagnosing IDHRs. However, there are still main needs, mainly in the standardization of the protocols. Subsequently consensus-based recommendations were formulated for: (i) Technical aspects of BAT in IDHRs including type of sample, management of drugs, flow cytometry protocols, interpretation of the results; and (ii) Drug-specific aspects that should be taken into account when performing BAT in relation to betalactams, neuromuscular blocking agents, fluoroquinolones, chlorhexidine, opioids, radio contrast media, chemotherapeutics, biological agents, nonsteroidal anti-inflammatory drugs, COVID vaccine, and excipients. Moreover, aspects in the evaluation of pediatric population have also been considered. All this indicates that BAT offers the clinician and laboratory a complementary tool for a safe diagnostic for IDHRs, although its place in the diagnostic algorithm depends on the drug class and patient population (phenotype, geography, and age). The standardization of BAT is important for generalizing this method beyond the individual laboratory.

Performance accuracy, advantages and limitations of a store-and-forward teledermatology platform developed for general practitioners: A retrospective study of 298 cases.

BACKGROUND: Store-and-forward (SAF) tele-dermatology (TD) platforms could help promote coordination between hospital and general practitioners (GPs). However, very little data exists on the performance accuracy and opinions of GPs participating in this type of project in France. METHODS: We report on the diagnostic and management plan accuracy of an SAF-TD platform developed for neighbouring GPs around our hospital compared with routine face-to-face (FTF) dermatological consultation in our department. We also compared the accuracy of SAF-TD with that of the participating GPs. Lastly, we collected feedback from GPs after their participation in this project. RESULTS: Overall, 298 patients were included by 58 GPs between November 2016 and January 2020, of whom 169 (57%) were female, and with a median age of 44.5 years (range 0-96). The diagnostic accuracy of TD was 62% (n=184/298) for the initial hypothesis and 80% (n=239/298) for aggregated diagnostic accuracy. Management plan accuracy for TD was 81% (n=225/277). At least 43% of consultations (n=127/298) met the criteria for preventable consultation. Diagnostic accuracy for the initial hypothesis was significantly lower for GPs than for TD (Odd Ratio [OR]=0.34; 95% Confidence Interval [95% CI]: 0.20-0.56; p<0.0001), as was management plan accuracy (OR=0.23; 95% CI: 0.10-0.46; p<0.0001). Among the responding GPs, 78% (n=29) reported very high satisfaction and 97% would consider integrating this type of programme in their long-term practice, but they highlighted the time-consuming nature of the platform (46%) and the lack of financial compensation (44%). CONCLUSION: SAF-TD in coordination with GPs seems safe and efficient in the management of outpatients, and enjoys a high satisfaction rate among GPs, despite its time-consuming nature and the lack of financial compensation. Healthcare policy should promote financial participation to help the expansion of TD.

Antiphospholipid-negative Sneddon's syndrome: A comprehensive overview of a rare entity.

The term Sneddon's syndrome (SS) has been used since 1965 to describe a vasculopathy characterized by a combination of cerebrovascular disease with livedo racemosa. SS may be classified as antiphospholipid+ (aPL+) or antiphospholipid- (aPL-). Little is known about aPL- SS; in this review we describe the epidemiology and pathogenesis of aPL- SS, as well as the clinical and histologic features. We discuss recent findings in terms of neurologic and cardiac involvement. Moreover, differential diagnoses of conditions that may present with both livedo racemosa and stroke are discussed. Finally, we discuss real-life practical issues such as the initial investigations to be performed, long-term follow-up, and therapeutic management of aPL- SS patients.

Risks associated with cosmetic ingredients.

The media and social networks often echo fears about the potential toxicity of cosmetics and the dangers they pose to the environment. Dermatologists may be asked about these topics, but despite regulatory labelling constraints and the proliferation of specialized sites and applications, they do not always have access to reliable information. It is for this reason that we are providing the present overview of current knowledge on the subject.

Cutaneous drug-induced lupus erythematosus: Clinical and immunological characteristics and update on new associated drugs.

Cutaneous drug-induced lupus erythematosus (CDILE) is a lupus-like syndrome related to drug exposure which typically resolves after drug discontinuation. It can present as a systemic or a sole cutaneous form and different drugs may be associated with each form. CDILE pharmacoepidemiology is constantly changing. Indeed, older drugs primarily associated with systemic CDILE are no longer prescribed and new drugs associated with either cutaneous or systemic CDILE have emerged. The present study discusses the clinical and laboratory aspects of CDILE and the postulated pathogenesis, and it provides an update on implicated drugs. We performed a literature review to single out the new drugs associated with CDILE in the past decade (January 2010-June 2020). Among 109 drugs reported to induce CDILE in 472 patients, we identified anti-TNFα, proton-pump inhibitors, antineoplastic drugs, and, in particular, checkpoint inhibitors, as emerging drugs in CDILE. Most of the published studies are cases reports or small case series, and further larger studies as well as the development of validated classification criteria are needed to better understand and characterize their implication in CDILE.

Omalizumab in patients with chronic spontaneous urticaria nonresponsive to H1-antihistamine treatment: results of the phase IV open-label SUNRISE study.

BACKGROUND: Omalizumab is approved as an add-on therapy for the treatment of chronic spontaneous urticaria (CSU) in patients with inadequate response to H1-antihistamine treatment. The urticaria control test (UCT) is a reliable, concise tool developed as an alternative to the 7-day urticaria activity score (UAS7) - the standard for CSU disease activity assessment. OBJECTIVES: This prospective, open-label, phase IV study evaluated the efficacy and safety of omalizumab in French adult patients with CSU nonresponsive to H1-antihistamine treatment. MATERIALS AND METHODS: Patients [n = 136; stratified 1 : 2 (with angio-oedema : without angioedema)] received omalizumab 300 mg subcutaneously every 4 weeks for 12 weeks. Study assessments included UCT, UAS7, angio-oedema activity score and d-dimer levels (exploratory objective). RESULTS: At Week 12, 74·6% of the patients achieved disease control [UCT score ≥ 12 (primary endpoint)] and 67·7% of patients showed well-controlled disease (UAS7 ≤ 6). There was a strong negative correlation between UCT score and UAS7 at Week 12 (Spearman's correlation coefficient -0·839). Mean plasma d-dimer concentration was elevated at baseline (1002·1 ng mL-1 ) and decreased notably at Week 8 (455 ng mL-1 ). Among the nine patients with a very high baseline d-dimer concentration (> 3000 ng mL-1 ), eight were responders (UAS7 ≤ 6) at Week 12. CONCLUSIONS: Omalizumab was efficacious in patients with CSU nonresponsive to H1-antihistamines. The UCT was a reliable tool for disease assessment and the scores correlated well with UAS7. This study does not support the usefulness of d-dimer to monitor long-term disease prognosis in adult urticaria; however, it may indicate patients who respond to omalizumab.

Mineral oils and waxes in cosmetics: an overview mainly based on the current European regulations and the safety profile of these compounds.

Mineral oils and waxes are mixtures of predominantly saturated hydrocarbons consisting of straight-chain, branched and ring structures with carbon chain lengths greater than C14. They have been used for many decades in skin and lip care cosmetic products due to their excellent skin tolerance as well as their high protecting and cleansing performance and broad viscosity options. In contrast to vegetable oils, mineral oils are non-allergenic since they are highly stable and not susceptible to oxidation or rancidity. They have a long history of safe use which is confirmed by clinical and epidemiological data. In Europe, mineral oils are only permitted in cosmetics if compliant with purity specifications on polycyclic aromatic hydrocarbons and safety requirements laid down in the European pharmacopoeia and the EU cosmetics regulation EC/1223/2009. The high quality of these mineral oils is assured by robust quality assurance and a refining/purification process designed to exclude substances with carcinogenic potential and to minimize the presence of mineral oil aromatic hydrocarbons. Given their highly lipophilic properties, mineral oils do not penetrate human skin and, thus, are not systemically bioavailable in the body. Moreover, no significant changes in the skin and no effects on any internal organ system have been reported and attributed to the topical application of refined mineral oils. Regarding potential oral exposure from cosmetic lip care products, Cosmetics Europe, the European trade association for the cosmetics and personal care industry, has advised cosmetic manufacturers to only use mineral oil fractions for which recognized food acceptable daily intake (ADI) values apply. The estimated dose of mineral oils ingested via lip care products contributes to <10% of the ADI value and should therefore be considered of no toxicological concern.

[Two cases of unilateral chilblains associated with monoparesis]. / Engelures unilatérales associées à une monoparésie : 2 observations.

BACKGROUND: Chilblains are inflammatory dermal lesions associated with hypersensitivity to cold, and they occur on the extremities bilaterally and symmetrically. Their onset during the course of pro-thermogenic and autoimmune diseases has been widely reported, but the association with predisposing locoregional causes is not well known. PATIENTS AND METHODS: Case 1: a 57-year-old man, who smoked 80 packets per year, presenting a deficit of the levator muscles in his right foot following lumbar sciatica with paralysis of L5, consulted for unilateral necrotic lesions of the toes recurring each winter in the paralysed limb only. Case 2: a 60-year-old man had a previous history of liposarcoma of the right side treated with radiotherapy and surgery, resulting in sequelae of monoparesis and radiation-induced arteritis. Each winter, he presented recurring unilateral purpuric macules of the toes on his right foot, with no necrotic progression. In both cases, clinical examination, disease progression over time, histology and laboratory tests confirmed the diagnosis of idiopathic chilblains. CONCLUSION: The physiopathological hypotheses posited to account for the unilateral appearance of chilblains in the event of paralysis include decreased blood flow to the paralysed limb, imbalance in neuromodulators, dysfunction of the autonomous nervous system, cutaneous atrophy with hypertrophy of underlying soft tissues, and finally, hypoesthesia aggravating the trophic disorders.

[What is neutrophilic urticaria?] / Qu'est-ce que l'urticaire neutrophilique ?

Some debate continues to surround the existence of neutrophilic urticaria (NU) as a nosological entity. Certain authors consider NU as a banal form of urticaria since an infiltrate predominantly made up of polynuclear neutrophils (PNN) is seen in certain cases of chronic and acute urticaria. Moreover, it has been stated that the histological appearance of chronic urticaria varies according to the time between appearance of the plaque and the performance of biopsy: the presence of PNN may occur later. According to the literature, there appear to be no specific clinical characteristics associated with the presence of PNN at histology. Most cases exhibit moderate laboratory inflammatory syndrome. Data concerning therapeutic response are contradictory: some studies have shown no significant difference in terms of therapeutic response in relation to banal urticaria, while only one study has demonstrated superior response to dapsone in the case of histologically demonstrated neutrophilic infiltrate. There does not appear to be any disease more frequently associated in the event of NU. In conclusion, the available data concerning NU are insufficient to confirm the existence of this condition. A prospective study comparing routine acute and chronic urticaria biopsies would be extremely useful to better characterise the relationships between cellular infiltrate and therapeutic response.

[Omalizumab for the treatment of chronic urticaria: Real-life findings]. / Traitement de l'urticaire chronique par omalizumab : données en vie réelle.

OBJECTIVE: Assessment of the efficacy and safety of omalizumab in chronic urticaria refractory to conventional treatment (H1-antihistamines at high dosage and montelukast) in real-life practice. PATIENTS AND METHODS: A retrospective, descriptive, single-centre study was performed of the data for all patients presenting refractory chronic spontaneous urticaria or inducible urticaria and receiving omalizumab (300mg every four weeks) from November 2012 to June 2016. RESULTS: In all, 23 patients were included. Omalizumab led to complete or significant remission in 19 patients (83%) with chronic urticaria, with remission in 9 patients (47%) occurring within 72hours of the first injection. One patient had a partial response and 3 (13%) showed no response. Only 2 patients (9%) in complete remission stopped their treatment at 1 and 3 years. 52% of patients presented non-serious adverse events, which in one case resulted in treatment withdrawal. CONCLUSION: Omalizumab exhibited good real-life efficacy in a small series of chronic urticaria patients in France.

[The value of healthy skin biopsy in the diagnosis of intravascular B-cell lymphoma: A case report and systematic literature review]. / Intérêt de la biopsie cutanée en peau saine dans le diagnostic du lymphome B intravasculaire : cas clinique et revue systématique de la littérature.

BACKGROUND: Intravascular large B-cell lymphoma (ivLBCL) is a rare blood dyscrasia that is difficult to diagnose. Healthy skin biopsies may prove useful in diagnosis of the condition. Herein we report a case of ivLBCL diagnosed using this type of examination, and we provide a literature review to determine the sensitivity of such testing. PATIENTS AND METHODS: A 67-year-old woman was hospitalised for unexplained prolonged fever (UPF) and impaired general well-being. Laboratory tests revealed inflammatory syndrome, elevated LDH>2000IU/L, hepatic cytolysis and decreased prothrombin time at 47 %. Analysis for infection and medical imaging ruled out both an infectious or inflammatory origin and solid tumour. A healthy skin biopsy enabled confirmation of the diagnosis of ivLBCL. DISCUSSION: This clinical case illustrates the value of healthy skin biopsy in establishing a diagnosis of ivLBCL in patients hospitalised for UPF. Following a systematic literature review in PubMed/Medline, we included eight studies involving at least three patients designed to assess the value of healthy skin biopsy in the diagnosis of ivLBCL. The diagnostic sensitivity of this approach ranged from 67% to 100%, with a sensitivity of 100% being seen in four of the eight studies. Details of the biopsy sites were available in three studies and diagnostic sensitivity was similar overall between samples taken from the thigh, abdomen and arms. CONCLUSION: Healthy skin biopsy sampling from at least two sites constitutes a sensitive and relatively non-invasive procedure for early diagnosis of ivLBCL.

Sex- and age-adjusted prevalence estimates of five chronic inflammatory skin diseases in France: results of the « OBJECTIFS PEAU ¼ study.

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.

[Scalp lipedema]. / Lipœdème du cuir chevelu.

BACKGROUND: Lipedematous scalp, with or without alopecia, is a poorly known and rarely reported entity. It was first described in 1935 by Cornbleet. It involves increased thickness of the subcutaneous tissue of the scalp, responsible for an overall thickening of the scalp, which may be associated with alopecia, pruritus or painful sensations. Currently, fewer than 50 cases of lipedematous scalp, both with and without alopecia, have been reported in the literature. PATIENTS AND METHODS: Herein we present the case of a 36-year-old woman from the Ivory Coast, who presented scalp pain associated with infiltration of the entire subcutaneous tissue of the scalp seen clinically and confirmed at MRI. Histology added nothing. DISCUSSION: We diagnosed a new case of lipedematous scalp in an African woman. No cause was found. Therapeutic abstention appears the best management strategy.

[Cancers associated with systemic sclerosis involving anti-RNA polymerase III antibodies]. / Néoplasies associées à une sclérodermie systémique avec anticorps anti-ARN polymérase III.

BACKGROUND: The incidence of cancer is increased in patients with systemic sclerosis (SSc). Further, recent studies have also shown that the presence of anti-RNA polymerase III antibodies is associated with a higher incidence of cancer in this population. PATIENTS AND METHODS: Herein we present the cases of two men aged 56 and 23 years presenting SSc without anti-Scl70 or anti-centromere antibodies but with anti-RNA polymerase III antibodies. Clinical symptoms led us to prescribe more laboratory exams and both patients were diagnosed with cancer of the nasopharyngeal area. DISCUSSION: Anti-RNA polymerase III antibodies are useful for SSc diagnosis in patients without anti-centromere or anti-Scl70 antibodies. Their presence must lead physicians to screen for associated cancer, even in the absence of clinical signs.

[Digital necrosis revealing cold agglutinin disease: Treatment with rituximab]. / Nécroses digitales révélant une maladie des agglutinines froides : traitement par rituximab.

BACKGROUND: Digital necrosis is rarer than lower limb necrosis and constitutes a medical or surgical emergency. Etiological evaluation is required. Cold agglutinin disease is a cause of digital necrosis but diagnosis is difficult. PATIENTS AND METHODS: Herein we report the case of a 57-year-old man presenting recent paroxysmal acrosyndrome of the left hand subsequently complicated by digital necrosis following occupational exposure to cold in his work as a forklift driver. After etiological evaluation, a diagnosis of primary cold agglutinin disease was made. Intravenous rituximab and topical treatment resulted in complete healing. DISCUSSION: Cold agglutinin disease is a rare type of auto-immune hemolytic anemia. Following exposure to cold, paroxysmal cutaneous signs are frequent. The disease may be either primary or secondary with B-cell lymphoproliferative disorder, auto-immune disease or infection. A thorough workup is required. To date, the treatment combining the best positive response rate and good safety is rituximab in weekly perfusions over a 1-month period.

[Prevalence of hydroxychloroquine-induced side-effects in dermatology patients: A retrospective survey of 102 patients]. / Prévalence des effets indésirables de l'hydroxychloroquine (Plaquenil®) dans une population dermatologique : étude rétrospective sur 102 patients.

AIM: Our aim was to assess the prevalence of adverse effects (AEs) pertaining to the use and withdrawal of hydroxychloroquine (HCQ) in dermatological outpatients. PATIENTS AND METHODS: We conducted a retrospective study between January 2013 and June 2014 that included consecutive patients currently or previously receiving HCQ seen in our department. AEs were collated using a standardized questionnaire and validated by clinical and laboratory examination. Drug causality was evaluated using the updated French drug reaction causality assessment method. The main evaluation criterion was the prevalence of AEs in which HCQ had an intrinsic imputability score of I>2. RESULTS: We included 102 patients (93 of whom were women, with a median age of 44.5; range: 22-90years). HCQ was given for cutaneous lupus in most cases (n=70). At least one AE was reported for 55 patients. Among the 91 reported AEs, 59 (65%) had an HCQ intrinsic imputability score I>2. AEs were responsible for permanent HCQ discontinuation in 19 cases. Of these, 8 were unrelated to HCQ based on imputability score. The most common AEs associated with HCQ were gastrointestinal and cutaneous signs. Of the 8 patients diagnosed with retinopathy, only 3 were confirmed after reevaluation. CONCLUSION: AEs associated with HCQ were reported for over 50% of patients and were responsible for permanent HCQ discontinuation in one-third of cases. A more in-depth evaluation of imputability seems necessary, particularly regarding ophthalmological symptoms, since in two thirds of cases the reasons for discontinuation were not related to HCQ.

[Primary cutaneous CD30+ T-cell lymphoproliferation during treatment with fingolimod: Case report and literature review]. / Lymphoprolifération cutanée primitive CD30+ sous fingolimod : un cas et revue systématique de la littérature.

BACKGROUND: Fingolimod is an oral immunomodulator approved for relapsing-remitting multiple sclerosis. We report a case of a primary cutaneous CD30+ T-cell lymphoproliferation occurring 6 months after initiation of fingolimod. Based on a systematic literature review, the characteristics of these fingolimod-induced lymphoproliferative disorders are described. PATIENTS AND METHODS: A 56-year-old woman developed cutaneous indurated and ulcerated nodular lesions 6 months after starting fingolimod for active relapsing-remitting multiple sclerosis. Histological examination of a punch biopsy sample demonstrated a polymorphous dermal infiltrate containing large atypical CD30+ cells, leading to diagnosis of primary cutaneous CD30+ anaplastic large-cell lymphoma. Chest-abdomen-pelvis CT scans were performed to rule out secondary cutaneous anaplastic large-cell lymphoma. Spontaneous clinical regression was observed and after assessing the benefit/risk ratio, it was decided to continue fingolimod under strict surveillance, with no relapse occurring by month 18. DISCUSSION: A systematic review of PUBMED/Medline and Embase identified seven other cases of lymphoproliferative disorders occurring during fingolimod treatment, including two other cases of primitive cutaneous CD30+ lymphoproliferative disorders. CONCLUSION: Even if cutaneous CD30+ lymphoproliferative disorders occur only rarely during fingolimod treatment, dermatologists should nevertheless be aware of this association for which strict dermatological surveillance is required. We would also stress that these CD30+ lymphoproliferative disorders can disappear spontaneously, as in our case, even if treatment by fingolimod is continued.

Clinical approach on challenge and desensitization procedures with aspirin in patients with ischemic heart disease and nonsteroidal anti-inflammatory drug hypersensitivity.

BACKGROUND: Hypersensitivity to acetylsalicylic acid (ASA) constitutes a serious problem for subjects with coronary artery disease. In such subjects, physicians have to choose the more appropriate procedure between challenge and desensitization. As the literature on this issue is sparse, this study aimed to establish in these subjects clinical criteria for eligibility for an ASA challenge and/or desensitization. METHODS: Collection and analysis of data on ASA challenges and desensitizations from 10 allergy centers, as well as consensus among the related physicians and an expert panel. RESULTS: Altogether, 310 subjects were assessed; 217 had histories of urticaria/angioedema, 50 of anaphylaxis, 26 of nonimmediate cutaneous eruptions, and 17 of bronchospasm related to ASA/nonsteroidal anti-inflammatory drugs (NSAID) intake. Specifically, 119 subjects had index reactions to ASA doses lower than 300 mg. Of the 310 subjects, 138 had an acute coronary syndrome (ACS), 101 of whom underwent desensitizations, whereas 172 suffered from a chronic ischemic heart disease (CIHD), 126 of whom underwent challenges. Overall, 163 subjects underwent challenges and 147 subjects underwent desensitizations; 86 of the latter had index reactions to ASA doses of 300 mg or less. Ten subjects reacted to challenges, seven at doses up to 500 mg, three at a cumulative dose of 110 mg. The desensitization failure rate was 1.4%. CONCLUSIONS: In patients with stable CIHD and histories of nonsevere hypersensitivity reactions to ASA/NSAIDs, an ASA challenge is advisable. Patients with an ACS and histories of hypersensitivity reactions to ASA, especially following doses lower than 100 mg, should directly undergo desensitization.

A foot tumour as late cutaneous Lyme borreliosis: a new entity?

Acrodermatitis chronica atrophicans (ACA) is the late cutaneous form of Lyme borreliosis. The early inflammatory phase manifests with a bluish-red discoloration and doughy swelling of the skin. The atrophic phase represents a late-phase process with red discoloration, and a thin and wrinkled appearance of the skin. We present a patient who exhibited a previously undescribed form of late cutaneous Lyme borreliosis (LCLB) with a foot tumour. A 64-year-old woman had a large tumorous lesion on the right sole. The tumour size and deformation of the feet made wearing shoes difficult. On skin histology, a granulomatous lymphohistiocytic infiltrate with plasma cells was noticed. In fact, the patient recalled tick bites 2 or 3 years before. Borrelia burgdorferi (Bb) serology was highly positive and a polymerase chain reaction analysis on the skin biopsy detected Bb sensu lato, genospecies B. afzelii. We diagnosed LCLB and antibiotics were prescribed. On the more recent examination, the tumour had totally disappeared; the skin was atrophic and dry with only few scales. We report an atypical case of European LCLB, suggesting that ACA is not the only possible presentation of LCLB. The diagnosis of ACA is often clinically missed for months or years, and may be mistaken at the inflammation phase for vascular disorders, erysipelas or bursitis/arthritis, and at the atrophic phase for lichen sclerosus atrophicus, morphoea or anetoderma. To our knowledge, no such tumorous LCLB has previously been described.