Management of chronic wounds with an innovative absorbent wound dressing.

OBJECTIVE: To evaluate the efficacy and tolerability of an innovative absorbent wound dressing (UrgoClean; Laboratoires Urgo) in the local management of venous leg ulcers and pressure ulcers, during the sloughy stage of the healing process. METHOD: A pilot, prospective, non-controlled open-label clinical trial held in 21 investigating centres. Adult patients, presenting with either a venous leg ulcer (VLU) or a category III/IV pressure ulcer (PU) with more than 50% of the surface area covered with sloughy tissue, a duration of less than 24 months, and no clinical signs of infection were included in the study. Patients were followed over a 6-week period with weekly visits, which included a physical examination, wound-area tracings and photographs by the investigating physician. Evaluations by the nursing staff and by the patients were made at each dressing stage. RESULTS: Fifty patients with either a VLU (n=35) or a PU (n=15) were recruited. At baseline, mean wound surface area was 11.9 ± 11.3 cm(2) and 12.5 ± 10.7 cm(2), with a mean duration of 8.3 ± 6.4 months and 2.9 ± 3.0 months in the VLU and PU groups, respectively. Wounds in both groups were covered with more than 70% sloughy tissue, and the peri-lesional skin was considered to be healthy in 19 patients. By 6 weeks, mean wound surface area reduction in the VLU and PU groups was 23.7% and 29.2%, respectively, with full healing in 6 patients. All treated wounds were considered to be debrided by week 3 (<40% slough for all wounds) and the median relative decrease of the sloughy tissue, at week 6, in the VLU and PU groups was 75% and 89%, respectively. Dressing acceptability was documented as being very good for both patients and nursing staff, particularly conformability and ease of use, with no residue left on the wound bed at dressing removal and the dressing also remained in one piece. Seven local adverse events were deemed to be potentially related to the trial dressing. CONCLUSION: The results suggest that the dressing promoted the healing process of chronic wounds, showing itself to be a credible therapeutic alternative for the sloughy stage of the wound-healing process. It also demonstrated good tolerance and acceptability.

Contribution of PTEN large rearrangements in Cowden disease: a multiplex amplifiable probe hybridisation (MAPH) screening approach.

BACKGROUND: Cowden disease is an autosomal dominant syndrome predisposing to cancer and characterised by the occurrence throughout life of hyperplastic, hamartomatous and tumoural lesions affecting various organs. In 60-80% of patients a germline intragenic point mutation of the PTEN tumour suppressor gene is identified, but at least 20% of patients with a well characterised phenotype remain without any identified mutation. METHODS: To evaluate the impact of large rearrangement involving the PTEN locus in Cowden disease, we analysed by a multiplex amplifiable probe hybridisation (MAPH) technique 80 unrelated patients referred for diagnosed or suspected Cowden disease, and in whom no PTEN point mutation was detected by a denaturing gradient gel electrophoresis (DGGE) screening. RESULTS: Four heterozygous genomic deletions involving the PTEN gene were identified. These deletions ranged from 13.6-662 kb and are restricted to the PTEN locus in two cases. In the two other cases, the deletion encompassed PTEN and either two or three contiguous genes without any obvious phenotypic effect, except a possible consequence of PAPSS2 haploinsufficiency on bone growth. Sequence analysis of the four deleted alleles did not reveal identity or sequence homology at the two breakpoints of a same allele, suggesting that a mechanism such as non-homologous recombination of the breakage and reunion type could lead to the occurrence of these deletions. CONCLUSION: Large rearrangements of the PTEN gene can be involved as causing mutation in Cowden disease and MAPH is an efficient screening methodology to detect such a genetic alteration.

Evaluation of a lipidocolloid wound dressing in the local management of leg ulcers.

OBJECTIVE: To evaluate the efficacy, tolerance and acceptability of Urgotul and DuoDERM E dressings in the local management of venous or mixed-aetiology leg ulcers. METHOD: This was a prospective multicentre randomised phase IV clinical trial conducted open-label in parallel groups. It involved 20 investigating centres, including hospital dermatology and vascular medicine departments, and private practices. Dermatologists and angiologists/phlebologists took part. Subjects were adult, non-immunosuppressed patients presenting with a non-infected, non-malignant leg ulcer of predominantly venous origin (ABPI > 0.8). Ulcers were between 4cm2 and 40cm2 in size, with granulation tissue covering more than 50% of their surface area. Ulcer duration ranged from three to 18 months. Patients were followed-up by the investigating physician for eight weeks on a weekly basis; this included clinical examination, wound area tracings and photographs. Nurses (hospital or visiting) assessed exudate volume and clinical appearance at dressing changes. RESULTS: Ninety-one patients were included: 47 in the Urgotul group and 44 in the DuoDERM E group. Baseline patient demographic data and wound characteristics were comparable in the two groups. After eight weeks of treatment wound surface area had reduced by a mean of 61.3% in the Urgotul group and 52.1% in the DuoDERM E group (NS); dressings were changed more frequently in the DuoDERM E group (2.54 +/- 0.57 times per week versus 2.31 +/- 0.45 in the Urgotul group, p = 0.047). Thirty-three local adverse events were recorded in 27 patients: 10 in the Urgotul group and 23 in the DuoDERM E group (p = 0.039). Nurses reported better acceptability for the Urgotul dressing, based on pain on removal, maceration and odour (p < 0.0001). CONCLUSION: Both dressings showed similar efficacy for the local treatment of venous leg ulcers. Nevertheless, medical and nursing staff reported better tolerance and acceptability for the Urgotul dressing.

Short-term cyclosporin monotherapy for chronic severe plaque-type psoriasis.

Cyclosporin is an effective treatment for psoriasis but its efficacy is only palliative. The aim of this study was to evaluate the percentage of patients in whom a short term therapy may be used without relapse after discontinuation of cyclosporin. In this multicenter, open, non-controlled study fifty-eight patients were included who had severe and extensive chronic plaque-type psoriasis. Treatment duration was 28 weeks. The absence of relapse was defined as the requirement to resume systemic treatment at 16 weeks after discontinuation of Sandimmun . The overall efficacy of Sandimmun at W20 was 72%. No relapse or premature withdrawal occurred in 18 cases out of 39 (47%). In these cases local treatment was sufficient following discontinuation. Thus we show the potential value of a single 5 month course of cyclosporin treatment. In this study tapering of cyclosporin was not useful. In 50% of cases short-term cyclosporin treatment was not followed by resumption of systemic treatment and constitutes an improvement in qualify of life.

[Mycobacterium xenopi osteoarthritis of the ankle in a patient followed for psoriatic rheumatism]. / Ostéo-arthrite de cheville à Mycobacterium xenopi chez une patiente suivie pour rhumatisme psoriasique.

BACKGROUND: Mycobacterium xenopi is an uncommon cause of osteoarticular sepsis. However, a recent series of M. xenopi spondylodiscitis emphasizes the potential risk after invasive procedures. CASE REPORT: A 33-year-old woman was followed for psoriasis rheumatoid arthritis. She had undergone several exploratory procedures including arthroscopy of the left ankle for invalidating joint disease. In 1999, M. xenopi arthritis and osteomyelitis was diagnosis in this joint. DISCUSSION: Sterilization and maintenance of surgical instruments must abide by rigorously controlled strict protocols. The benefit/risk ratio of invasive procedures in debilitated joints should be more precisely evaluated. Specific and repeated sample cultures should be performed to search for mycobacteria in all bone and joint infections, particularly in case of prior invasive procedures.

[Acute pityriasis rubra pilaris in the child. Apropos of 4 cases]. / Le pityriasis rubra pilaire aigu de l'enfant. A propos de 4 observations.

Four cases of Pityriasis Rubra Pilaris of an acute type have been studied in three to six year old children. In three cases the disease occurred after an infection; in one out of these three cases this infection was serologically confirmed as being german measles. In the beginning, only the face is affected or the face and other part of the body. The rash looks like scarlet fever and the folicular papules appear only during the second stage. The dermatosis usually spreads within a month (from twenty days to two months) and covers the whole body without becoming erythrodermic. Then clinical picture is typical of Pityriasis Rubra Pilaris. There are no associated symptoms and no hypo-vitaminemia A is evidenced. The evolution is self limited with a spontaneous regression within three months, and there is no recurrence. These cases are similar to the classical juvenile Pityriasis Rubra Pilaris type III of Griffiths, but they keep their own particularities as far as acute evolution and good prognosis are concerned.

[Parvovirus B19 atypical acrosyndrome]. / Acrosyndrome atypique à parvovirus B19.

INTRODUCTION: Parvovirus B19 infection is a very polymorphic eruption. We describe a new clinical form. CASE REPORT: A 4-year-old child was hospitalized for a painful acrocyanosis localised at the feet and the tip of the nose, which disappeared without any treatment in three weeks. The screening revealed a parvovirus B19 infection. DISCUSSION: This eruption differs from the classic "gloves and socks syndrome" because of the presence of plantar and nasal painful erythrocyanosis and the young age of the child.

[Richner-Hanhart's syndrome or oculo-cutaneous tyrosinosis (about one case) (author's transl)]. / Syndrome de Richner-Hanhart ou tyrosinose oculo-cutanée. A propos d'un cas.

Richner-Hanhart's syndrome correspond to an hypertyrosinemia due to a deficiency of a soluble tyrosine amino-transferase. This recently described tyrosinosis has been called oculo-cutaneous tyrosinosis. This disease transmitted on a recessive way in amenable to a treatment by a low tyrosine diet. In an infant, 18 months old, presenting a bilateral dendritic keratitis, a punctiform keratosis of the extremities, a patchy leucokeratosis of the tongue and a mental ketardation, the hypertyrosinemia reached 52 mg per 100 ml and the urine demonstrated the presence of phenyl-atonic acids. There was no hepato-renal involvement. The deferency of soluble amino-transferase was studied on the hepatocytes and confirmed. The low tyrosine diet made the clinical and biological signs disappear. The improvement was noticeable from the first week on and continued during the 16 months of the follow-up. There was no ill effect of the special diet on the weight and height growth. The oculo-cutaneous tyrosinosis is similar to the experimental form obtained by Schweizer on the rat. The occurrence of intracellular tyrosine crystals probably damages lysosine membrane and the release lysomie proteases induce the cellular lesions.

[Neonatal cutaneous hematopoiesis disclosing congenital rubella]. / Hématopoïèse cutanée néonatale révélant une rubéole congénitale.

INTRODUCTION: Congenital rubella, which should disappear with widespread vaccination and mandatory obstetrical care, can occur as a purpuric eruption in the newborn. We report a case of blueberry muffin baby. CASE REPORT: An infant delivered after an "uneventful" pregnancy presented a generalized "purpuric" eruption and had axial hypotonia. Histology of a biopsy showed evidence of cutaneous erythropoiesis. The complete workup led to the diagnosis of congenital rubella. DISCUSSION: Cutaneous erythropoiesis is a well defined clinical and histological entity. There are several causes including infection and hematology disorders. Metastasis of a neuroblastoma, which must be eliminated by early biopsy, is the main differential diagnosis. CONCLUSION: Blueberry muffin rash is never idiopathic. The prognosis depends on the cause. Physicians should remember that congenital rubella has not yet been completely eradicated in France.

[Toxic staphylococcal cutaneous necrolysis (scalded skin syndrome) (author's transl)]. / Le syndrome toxique staphylococcique cutané dit des enfants ébouillantés (scalded skin syndrome).

The exfoliating exotoxin of serotype II staphylococci is responsable of three diseases with prevailing dermatologic symptoms: staphylococcal scarlet fever, staphylococcal Lyell syndrom and Ritter-Lyell disease. Melish demonstrated the effect of staphylococcal exotoxin on new-born mice less than five days old. This experimental model allowed to study the characteristics of the toxin. Precocious antistaphylococcal antibiotherapy is necessary.

[Dermo-neuro-hypsarrhythmia. Association of a congenital dermatosis and hypsarthymia. 9 cases (author's transl)]. / Dermo-neuro-hypsarythmie. Association d'une dermatose congénitale et d'une hypsarythmie, A propos de 9 cas

These 9 cases confirm that tuberose sclerosis, von Recklinghausen's neurofibromatosis, incontinentia pigmenti, linear warty sebaceous naevus and alopecic naevus resulting in a woolly naevus of the scalp may be complicated by flexion spasms with hypsarhythmia. Early diagnosis of hypsarhythmia makes possible specific treatment with ACTH or hydrocortisone.

[Collodion baby: 32 new case reports]. / Bébé collodion. Trente-deux nouvelles observations.

A critical study of 69 cases of collodion baby observed between 1976 and 1984 is compared with the study conducted in 1976. These 69 cases are divided into 32 examined by the authors personally and 37 published in the literature. The total number of collodion babies is 267. A collodion baby is a high risk newborn who must be nursed in a specialized intensive care unit during the neonatal period. The vital prognosis has been improved by the prevention of metabolic disorders (dehydration with hypernatraemia) and of percutaneous intoxication with topical products, and by the early treatment of infections. The mortality rate, which was 33 p. 100 in 1976, had fallen to 11 p. 100 in 1984. Skin infection with systemic spread remains the major complication (the present situation is summarized in table II). The collodion baby is the neonatal expression of different disorders of keratinization and as such, constitutes a syndrome. Several conclusions can be drawn from a comparison of the keratinization disorders observed in our two series (table VI). Congenital ichthyosiform erythroderma and lamellar ichthyosis are responsible for 60 p. 100 of collodion babies. These two lesions may be the first signs of an ichthyosis transmitted as an autosomal dominant trait in 9.7 p. 100 of the cases. Trichothiodystrophy may be heralded by a collodion baby syndrome. In 9.7 p. 100 of the cases this syndrome disappears without sequelae. Sex-linked ichthyosis never begins with a collodion baby syndrome.

[Vitiligoid achromias and severe atopic dermatitis. Apropos of 4 cases]. / Achromies vitiligoïdes et dermatite atopique grave. A propos de quatre cas.

Four patients with important and disabling atopic dermatitis persisting during adulthood have presented leucodermia in certain areas of eczema. As melanosomes and melanocytes have totally disappeared in these regions, this depigmentation corresponds thus to an achromia. Clinically, we noted macular achromia with hyperpigmented border of special topography since touching pleat regions initially present eczema lesions: anterior face of wrists and footnecks. Slight lichenification is noted in achromic regions. In spite of many analogies with vitiligo, we differentiate this achromia from vitiligo on the basis of absence of familial post-history and absence of new localisations after 5 to 6 years cause. A review of depigmentations described during atopic dermatitis does not show similar facts. Depigmentation induced by local steroid therapy does not give such clinical and ultrastructural aspects. In 3 patients, there was no local application of depigmenting agent. In one case, 8-oxyquinoleine was applied transiently. Vitiliginous achromias of pleat regions initially presenting important atopic dermatitis are probably due to multifactorial factors: possible factors are inflammation and secondary epidermal modification due to local steroid therapy and may be some excipients. Being a rare situation in atopy, we have registered it only 14 times in 860 followed atopic dermatitis. The study of series of atopic dermatitis followed on a long-term basis should allow to evaluate the frequency of such incidents and the respective role of aetiological factors with we suspect.

[Systemic scleroderma in children. Apropos of 5 cases. A review of the literature]. / Sclérodermie systémique de l'enfant. A propos de 5 observations. Revue de la littérature.

Systemic scleroderma is a rare disease in childhood. 62 cases are analyzed. Cutaneous manifestations are identical to those seen in adults. However Raynaud phenomenon is much more frequently missing but follow-up of patients is only of 4 years' duration. We want to draw attention on possible worsening of clinical signs during intercurrent infectious episodes. We report exceptional roentgenological bone anomalies. Gastro-intestinal tract is frequently involved, particularly the oesophagus. We want to draw attention on latent small intestine involvement. A normal thoracic X-ray examination cannot rule out involvement of the lungs; systematic respiratory functional tests are absolutely necessary. All parts of the cardiac wall can be involved: we underline the particular seriousness of this involvement as it was exclusively responsible of death in 10 cases out of 21. Renal involvement is rare. We are reporting 2 cases where a staturo-ponderal retardation remains unexplained; 7 other cases in the literature report on isolated weight retardation. Biological anomalies are similar to those reported in adult. Treatment is not well-codified; we can hope that a better understanding of the disease's physiopathology will lead to the discovery of an efficient therapy.

[Early acroparesthesia in females: a sign disclosing heterozygote Fabry disease]. / Acroparesthésies féminines précoces: signe révélateur d'une maladie de Fabry à l'état hétérozygote.

Heterozygous Fabry's disease has an inconstant expression and very few complications. The theory of X-chromosome inactivation which, according to Lyon, occurs hazardly, is illustrated by the fact that the disease is expressed even in hemizygous women. Ophthalmic manifestations, as detected by the slit lamp method, are almost constant, 80 p. 100 of women with the disease having a verticillate cornea. Angiokeratoma is present in 20 p. 100 of the cases. Episodes of paraesthesia of the hands and feet are less common; in most cases they are attributed to the disease retrospectively, during family investigations. In two girls aged 10 and 11 years respectively and without history of Fabry's disease the only symptom suggestive of the diagnosis was paroxysmal acroparaesthesia. In one of the girls acroparaesthesia was associated with acrocyanosis, livedo and acro-osteolysis, but concordance was the only argument in favour of a link with Fabry's disease. Alterations of the extremities have been reported in this disease, including palmar erythema and a bluish discoloration of the palms due to dilatation of the superficial veins. Only two cases of livedo have been published. Acrosteolysis has never been documented in Fabry's disease, and its presence must be confirmed in further cases. The diagnosis of heterozygous Fabry's disease in these 2 girls was confirmed by the finding of ceramide trihexoside in urine and by leucocyte alpha-galactosidase levels that were 25 to 30 p. 100 of values obtained in controls. A study of the family of one of the girls showed that the father was involved; this hemizygous type of the disease with a 10 p. 100 alphagalactosidase level was totally asymptomatic.(ABSTRACT TRUNCATED AT 250 WORDS)

[Pachydermatoglyphy. A sign of malignancy for acanthosis nigricans (author's transl)]. / Pachydermatoglyphie. Signe en faveur de la malignité au cours de l'acanthosis nigricans.

The pachydermatoglyphy corresponds from the clinical point of view to a diffuse palmo-plantar keratodermia, of a yellowish color, characterised by a rough thickening of the finger prints. The presence of the pachydermatoglyphy has been pointed out in six cases of malignant acanthosis nigricans. This was already found in previous descriptions. Pachydermatoglyphy is specially found in malignant acanthosis nigricans, but we have also observed such cases in erythrokeratodermia with pilar keratosis and deafness.

[Centrifugal annular erythema of Colcott-Fox type (erythema gyratum perstans)]. / Erythéme annulaire centrifuge type colcott-fox (erythema gyratum perstans)

The case of a child with Colcott-Fox type of centrifugal annular chronic erythema is reported. It is a sporadic, non-familial form, associated with a bullous eruption, and an atrophic conjonctivitis with trichiasis. A review is made of 11 previously published cases, allowing a synthesis of their essential features. The action played by the slow reactive substances (S. R. S. A.) in the extension of the erythematous margin is discussed. The clinical diagnosis of centrifugal annular erythema is exposed.

[Systematized papulosis of the lower scapular areas with ultrastructural anhist bodies: specific cutaneous symptoms of Hunter's disease (author's transl)]. / Papulose sous-scapulaire systématisée avec corps anhistes en microscopie électronique: signes cutanés spécifiques de la maladie de hunter.

Papulosis of the scapular areas is a characteristic dermatologic sign of Hunter's disease. It has not been described in the other mucopolysaccharidoses. This sign had been detailed by Hunter in 1917 in his princeps observation. We noted it in two cases of the disease. The ultrastructural study showed an accumulation in the connective interstitial substance of anhist bodies of intra-cellular origin.

[Sarcoidosis: thalidomide treatment in ten patients]. / Sarcoïdose: traitement par thalidomide chez 10 malades.

BACKGROUND: Acute cutaneous sarcoidosis is generally spontaneously regressive but persistent chronic cutaneous lesions are esthetically prejudicial. There have been several case reports on thalidomide efficacy but long-term outcome is unknown. We report results in 10 cases of cutaneous sarcoidosis treated with thalidomide. PATIENTS AND METHODS: Data from ten patients with sarcoidosis treated with thalidomide between January 1998 and March 1999 were collected from delivery authorizations and analyzed. All ten patients had chronic cutaneous sarcoidosis resistant to conventional therapy. Six patients had an associated visceral localization and disease duration of 2 to 18 years (median 6 years). We considered that regression was complete when erythema and infiltration had totally disappeared, that regression was incomplete when cutaneous signs remained, and that treatment had failed when no effect was observed or when the disease worsened. RESULTS: Disease regression was noted in 7 patients for a daily dose of 1.84 mg/kg for 2.8 months. Skin lesions totally regressed in 3 patients, an incompletely in 4. Treatment failed in 3 patients. Patients were treated for 10 months (2 to 21 months). The daily dose of thalidomide was gradually reduced in 5 of 7 patients for whom treatment was effective. Three of these 5 patients relapsed and thalidomide was again given and was effective again at the same dose and after the same delay. We observed improved kidney function in one patient, improvement in nasal infiltration in one other and complete regression in 3 patients who achieved long lasting reduction in angiotensin convertase level. When treatment failed, the daily dose was 1.15 mg/kg and the treatment had to be stopped for 2 patients. Side effects were minor, excepting 2 cases of neuropathy. DISCUSSION: This open study of 10 patients treated with thalidomide showed the efficacy of a 1.84 mg/kg daily dose in 7 out of 10, but complete regression of the lesions was obtained in only 3 patients. Thalidomiide appears to suspend the disease, with relapse when the drug is discontinued and efficacy at re-introduction. This would argue against a placebo effect. The mode of action could involve immunomodulating and antiinflammatory mechanisms.