Leg ulcers in childhood: A multicenter study in France.

BACKGROUND: Leg ulcers in adults are a major public health concern. Their incidence increases with age and many causes have been identified, predominantly associated with vascular diseases. Leg ulcers in children and teenagers are less frequent. The aim of our study was to identify the causes of leg ulcers in children and teenagers, and to evaluate their management. METHODS: This retrospective multicenter study was conducted by members of the Angio-dermatology Group of the French Society of Dermatology and of the French Society of Pediatric Dermatology. Data from children and teenagers (< 18 years), seen between 2008 and 2020 in 12 French hospitals for chronic leg ulcer (disease course>4 weeks), were included. RESULTS: We included 27 patients, aged from 2.3 to 17.0 years. The most frequent causes of leg ulcer were: general diseases (n=9: pyoderma gangrenosum, dermatomyositis, interferonopathy, sickle cell disease, prolidase deficiency, scleroderma, Ehlers-Danlos syndrome), vasculopathies (n=8: hemangioma, capillary malformation, arteriovenous malformation), trauma (n=4: bedsores, pressure ulcers under plaster cast), infectious diseases (n=4: pyoderma, tuberculosis, Buruli ulcer) and neuropathies (n=2). Comorbidities (59.3%) and chronic treatments (18.5%) identified as risk factors for delayed healing were frequent. The average time to healing was 9.1 months. DISCUSSION: Leg ulcers are less frequent in children and teenagers than in adults and their causes differ from those in adults. Comorbidities associated with delayed healing must be identified and managed. Children and teenagers tend to heal faster than adults, but a multidisciplinary management approach is necessary.

Biological treatments for paediatric psoriasis : a retrospective observational study on biological drug survival in daily practice in childhood psoriasis.

BACKGROUND: Three biotherapies - etanercept, adalimumab and ustekinumab - are licensed in childhood psoriasis. The few data available on their efficacy and tolerance are mainly derived from industry trials. However, biological drug survival impacts long-term performance in real-life settings. OBJECTIVE: The objective of this study was to evaluate the survival rates of biological therapies in children with psoriasis in real-life conditions. Secondary objectives were to evaluate the factors associated with the choice of the biological therapy and to report severe adverse events. MATERIALS AND METHODS: This study was an observational retrospective study. Data were extracted from the clinical records of 134 children. Kaplan-Meier estimates were used to analyse drug survival overall and in subgroups of plaque psoriasis, bio-naïve and non-naïve patients. RESULTS: We analysed 184 treatment courses: 70 with etanercept, 68 with adalimumab and 46 with ustekinumab. Factors associated with the choice of first-line biological agent were age at initiation (younger for adalimumab, P < 0.0001), age at onset of psoriasis (younger for adalimumab and etanercept, P = 0.03) and baseline Psoriasis Assessment Severity Index and Physician global assessment (both higher for adalimumab, P < 0.001). Drug survival rates were higher for ustekinumab than for adalimumab and etanercept (P < 0.0001) for all treatment and all psoriasis types, plaque-type psoriasis (P = 0.0003), patients naïve for biological agents (P = 0.0007) and non-naïve patients (P = 0.007). We reported eight serious adverse events (SAEs): severe infections (n = 3), significant weight gain (n = 2), psoriasis flare (n = 1) and malaise (n = 1). Biological therapy was discontinued in three children (one with psoriasis flare and two with weight gain). Only the two cases of weight gain resulted in an unfavourable outcome. CONCLUSIONS: Our real-life comparative study found that ustekinumab had the best drug survival outcome. The profile of SAEs in children was comparable to that in adults. These results will assist dermatologists in the decision-making process when choosing treatment options for children with psoriasis in daily practice.

Tongue psoriasis: Clinical aspects and analysis of epidemiological associations in 313 children, with a systematic literature review.

BACKGROUND: Little information is available on the prevalence and clinical aspects of tongue involvement in children with psoriasis. The aim was to evaluate the prevalence, clinical aspects and risk factors concerning tongue involvement in children with psoriasis. PATIENTS AND METHODS: This study was carried out in two stages. We performed a multicentre, cross-sectional study in 23 French dermatology centers. All children seen for psoriasis during the one-year study were systematically included. The clinical features of the tongue and of psoriasis were recorded. Association with clinical aspects of psoriasis and comorbidities was evaluated. We then carried out a literature review to evaluate the prevalence of tongue involvement in children with psoriasis and its positive predictive value for psoriasis. A search was conducted in the PUBMED database using the following keywords: "child" and "psoriasis" and ("tongue" or "glossitis" or "migratory glossitis" or "benign migratory glossitis" or "geographic tongue" or "fissured tongue"). RESULTS: 7.7% of the 313 children with psoriasis had tongue involvement. The clinical aspects were geographic tongue (4.2%), fissured tongue (2.8%) and both (0.64%). There was no association between tongue involvement and the clinical characteristics of the children. Two hundred and ninety-five articles were referenced and 3 were analysed. Psoriasis is very rare in cases of tongue abnormalities. CONCLUSION: The prevalence of tongue involvement was 7.7% in children with psoriasis. No clinical or epidemiological association was shown. Tongue involvement does not modify the management of psoriasis. In the literature review it was not possible to evaluate either the prevalence of tongue involvement in psoriasis or the positive predictive value thereof.

[Cutaneous periarteritis nodosa recurring over a period of 30 years in streptococcal infections and progressing toward systemic vasculitis]. / Périartérite noueuse cutanée récidivant depuis 30 ans lors d'infections streptococciques et évoluant vers une vascularite systémique.

INTRODUCTION: Periarteritis nodosa (PAN) is a form of vasculitis affecting the small and medium-sized arteries. Below, we report a case of cutaneous PAN relapsing in streptococcal infections over a period of 30 years and progressing towards systemic vasculitis. CASE REPORT: A 35-year-old man was hospitalised for a retro-pharyngeal access associated with fever, arthralgia, myalgia and inflammatory subcutaneous nodules. Peripheral neurological signs were also seen with deficiency of the elevator muscles in the right foot. Examination of a biopsy from a nodule showed a characteristic image of PAN. Following drainage of the abscess, a favourable outcome was obtained with antibiotics and systemic corticosteroids. History taking showed that the patient had presented similar episodes since the age of 5 years involving arthralgia, myalgia and inflammatory subcutaneous nodules. These episodes appeared to follow a streptococcal infection, of which there was either clinical suspicion or objective elevation of antistreptolysin O (ASLO) titre. Skin biopsy resulted in diagnosis of cutaneous PAN 25 years earlier. In all cases, improvement was achieved by oral corticosteroids combined with treatment of the actual infection. DISCUSSION: In addition to the classic association with hepatitis B, and occasionally hepatitis C, PAN may be associated with streptococcal infections. The cases of post-streptococcal PAN described in the literature are predominantly cutaneous, although it is not rare to find associated arthromyalgia and sensory neurological impairment. We examined three cases of cutaneous PAN with long-term follow-up described in the literature. They began in childhood and the outcome was benign, with no systemic manifestations. Our case differed in terms of the appearance of motor neurological involvement. CONCLUSION: Post-streptococcal PAN of childhood onset generally carries a better prognosis than adult systemic forms. However, our case shows that on rare occasions, there may be very long progression complicated by systemic involvement.

Cutaneous events during treatment of chronic inflammatory joint disorders with anti-tumour necrosis factor alpha: a cross-sectional study.

BACKGROUND: Anti-tumour necrosis factors (anti-TNF) are more and more used, but the rate of skin adverse events is not known. OBJECTIVE: The aim was to assess the number of skin infections and other dermatoses in patients treated with anti-TNFalpha. PATIENTS AND METHODS: One hundred eighty-seven patients suffering from rheumatoid arthritis or ankylosing spondylitis underwent a dermatological exam. Patients with anti-TNF were compared with those without this treatment in a prospective transversal study. RESULTS: Among them, 59 patients were treated with anti-TNFalpha and steroids were prescribed in 100 cases. There was no difference in the prevalence of skin infections or eczema or tumours. Skin drug reactions were observed in six patients. Infections by dermatophytes appear very frequent, approaching 70% in both groups. CONCLUSIONS: This study shows that skin infections (or other skin diseases) are not more frequent in these patients. No differences were observed in infections (bacterial fungal, parasital or viral), tumours, psoriasis or the manifestations of atopic dermatitis. Nonetheless, a long-term survey might be interesting, especially about skin tumours.

[Leukaemia cutis during acute aleukaemic myeloid leukaemia in a two-month-old infant]. / Leucémie cutanée révélatrice d'une leucémie aiguë myéloïde sans blastes circulants chez un nourrisson de deux mois.

BACKGROUND: Congenital cutaneous leukaemia is rare. PATIENTS AND METHODS: A two-month-old girl presented bluish cutaneous macules of the trunk, histological examination of which suggested acute myeloid leukaemia (LAM B 5). The blood picture was negative for circulating tumour cells and the outcome under chemotherapy was favourable at one year of follow-up. DISCUSSION: The prognosis of congenital leukaemia is serious. Aleukaemic congenital leukaemia is seen occasionally but is rare. The existence of multiple cutaneous tumours in newborn infants raises the possibility of TORCH infection and of other malignant tumours such as nephroblastoma or neuroblastoma.

[Neutrophilic dermatosis associated with propylthiouracil-induced p-ANCA (p-antineutrophil cytoplasmic antibodies)]. / Dermatose neutrophilique associée à des anticorps anticytoplasme des polynucléaires neutrophiles (p-ANCA) induits par le propylthiouracil.

INTRODUCTION: We report on a patient who progressively developed polymorphic expressions of neutrophilic dermatosis (Sneddon-Wilkinson subcorneal pustulosis and pyoderma gangrenosum) associated with p-antineutrophil cytoplasmic antibodies (p-ANCA), while receiving propylthiouracil for hyperthyroidism. To our knowledge, such associations have never been published so far. CASE-REPORT: A 40 year-old woman was treated with propylthiouracil for Graves'disease. After 16 months of therapy, she noted flares of pustular lesions surrounded with erythematous halo mainly localized on the trunk. The lesions became chronic, and were not improved by potent topical corticosteroids. When first seen in our department in February 2003, the eruption was typical of Sneddon-Wilkinson subcorneal pustulosis. This diagnosis was confirmed by the histological examination of a skin biopsy of a pustule. One month later, she developed an inflammatory progressively ulcerative lesion on the right ankle, typical of pyoderma gangrenosum. The diagnosis was confirmed by the histological examination of a skin biopsy taken on the evolving border of the lesion and showed polynuclear neutrophilic infiltration without vasculitis. Direct immunofluorescence was negative. The presence of serum anti-myeloperoxydase p-ANCA was known for this patient since October 2002. No IgA monoclonal gammapathy was revealed on extensive biological check-up. Systemic oral corticosteroid therapy (1 mg/kg/day) dramatically improved skin lesions with complete healing within 8 weeks. DISCUSSION: Propylthiouracil is well known to induce the occurrence of ANCA in 20 to 64p. 100 of patients treated for Graves'disease. The mechanisms involved are badly recognized so far. Cutaneous vasculitis, glomerulonephritis and polychondritis may be clinically associated with those antibodies. Rare observations of neutrophilic dermatosis, mostly Sweet's syndrome, have been described in patients with propylthiouracil-induced ANCA. One case-report described a 44 year-old woman who developed pyoderma gangrenosum associated with propylthiouracil-induced p-ANCA. These manifestations usually appear within 2 years, as our patient. The data in the literature, allows us to report the polymorphic expressions of neutrophilic dermatosis in this patient with p-ANCA which could be related to propylthiouracil. Such association of Sneddon-Wilkinson subcorneal pustulosis and pyoderma gangrenosum with p-ANCA has never been described in this endocrinologic context so far. Furthermore we propose that neutrophilic dermatosis should be inscribed in the list of side effects induced by propylthiouracil therapy.

Topical imiquimod treatment of lentigo maligna: clinical and histologic evaluation.

We have treated extensive lentigo maligna in two elderly patients with Imiquimod 5% (Aldara). It was applied 3 times weekly, for 3 to 5 months. Clinical and histological remission was observed over 13 months of follow-up care. Tolerance was good. We suggest that topical imiquimod could be an interesting therapeutic alternative.

[Papulo-erythematous eruption of neonatal lupus]. / Lupus néonatal: éruption papulo-érythémateuse diffuse.

INTRODUCTION: Neonatal lupus is rare and cutaneous lesions are usually suggestive of the diagnosis. We describe the case an infant with atypical clinical and histological aspects. CASE REPORT: A female newborn, 6 weeks of life, presented since 3 weeks a papulo-erythematous eruption involving the trunk and the 4 limbs. Cutaneous biopsy showed a dermal infiltrate of mononuclear cells, which corresponded morphologically to lymphocytes. But myelo-monocytic origin was proved by immunostaining. This result could be seen in hematodermia and macrophagic activation syndrome, but we had no clinical sign for these diagnosis. Later, she presented an erythema of the eyelids and erythematous papules of the face leading to suspicion of neonatal lupus. Questioning the mother revealed that she had Raynaud's syndrome since 1995. Antinuclear factors were positive in the patient and her mother, corresponding to antiSSA and antiSSB antibodies. She exhibited cytolytic hepatitis but no congenital heart block. Direct immunofluorescence was negative. The clinical evolution was good with complete clearing of the eruption at the age of 3 months. At 10 months, clinical and biological examinations were normal, with no arguments for haematological disease. DISCUSSION: This observation is original because of the initial papulo-erythematous eruption which is rare in neonatal lupus. Diagnosis was confirmed when specific secondary cutaneous lesions appeared. The biopsy of the first eruption showed a myelo-monocytic infiltrate which has never been described in neonatal lupus. However cutaneous biopsies are rarely performed in neonatal lupus and immunostaining is lacking in the literature.

[Contribution of anamnesis and clinical examination in the assessment of chronic urticaria]. / Apport de l'interrogatoire et de l'examen clinique dans le bilan d'une urticaire chronique.

Chronic urticaria is defined as an eruption of oedematous and pruriginous papules, localized or widespread, evolving for more than 6 weeks. Cutaneous lesions occurs daily or recurs with remissions of a few days at a few weeks. Diagnosing chronic urticaria is easy, but to find an etiology is much more tiresome. This is why a detailed anamnesis and a clinical examination are significant elements in the assessment of chronic urticaria. The analysis of the literature according to criteria's of the National Agency of Accreditation and Evaluation in Health shows that the anamnesis and the clinical examination are sufficient to carry a diagnosis in the majority of physical urticaria. However, they bring elements to suspect an urticarial vasculitis, a systemic disease or another etiology, allowing orientation of complementary examinations. The anamnesis permits to detect worsening factors, in particular drugs and/or food, their suppression leading to an improvement of symptomatology.