Y. De Prost

Subcutaneous fat necrosis of the newborn: a systematic evaluation of risk factors, clinical manifestations, complications and outcome of 16 children

Background  Subcutaneous fat necrosis (SFN) of the newborn is a rare acute transient hypodermatitis that develops within the first weeks of life in term infants. It often follows a difficult delivery. Prognosis is generally good except for the development of hypercalcaemia in severe cases. Only several case reports or small patients series have been published.

Serious childhood angiomas: unsuccessful alpha‐2b interferon treatment. A report of four cases

Over a 4‐year period, we managed four children with alarming haemangiomas (two cases of Kasabach‐Merritt syndrome and two life‐threatening haemangiomas). Systemic steroid therapy was ineffective. Other treatments (radiotherapy, anti‐platelet drugs) were also ineffective in the Kasabach‐Merritt patients. On the basis of recent reports on the effects of interferon on endothelial cells, we used alpha‐2 interferon therapy, but obtained no response.

Germline mosaicism in keratitis–ichthyosis–deafness syndrome: pre-natal diagnosis in a familial lethal form

Sbidian E, Feldmann D, Bengoa J, Fraitag S, Abadie V, de Prost Y, Bodemer C, Hadj‐Rabia S. Germline mosaicism in keratitis–ichthyosis–deafness syndrome: pre‐natal diagnosis in a familial lethal form.

Efficacy of tacrolimus 0.03% ointment as second-line treatment for children with moderate-to-severe atopic dermatitis: evidence from a randomized, double-blind non-inferiority trial vs. fluticasone 0.005% ointment.

Doss N, Kamoun M‐R, Dubertret L, Cambazard F, Remitz A, Lahfa M, de Prost Y. Efficacy of tacrolimus 0.03% ointment as second‐line treatment for children with moderate‐to‐severe atopic dermatitis: evidence from a randomized, double‐blind non‐inferiority trial vs. fluticasone 0.005% ointment. 
Pediatr Allergy Immunol 2010: 21: 321–329.
© 2009 John Wiley & Sons A/S

Skin elastic fibers in Williams syndrome

The elastin gene is consistently deleted in Williams syndrome and as this protein represents the major component of the elastic fibers of the dermis, we sought to investigate skin elastic fibers in Williams syndrome as a key to unraveling extracellular matrix disorganization in this condition. Both morphometric parameters analyzed by using automated image analysis and immunofluorescence labeling with monoclonal antibodies against elastin and fibrillin 1 showed a disorganized pre-elastic (oxytalan and elaunin) and mature elastic fibers in the dermis of 10 Williams syndrome patients compared with five healthy children and one patient with isolated supravalvular aortic stenosis. Skin biopsies in Williams syndrome patients provide a simple mean to elucidate extracellular matrix anomalies. Hopefully, this method could give clues to the understanding of the elastic network anomalies in this condition and even to the consequences of these latter on elasticity and resilience of other tissues such as the arterial tree.

Lymphomatoid papulosis in children: a series of 25 cases

Lymphomatoid papulosis (LyP) is an uncommon cutaneous T‐cell lymphoproliferative disorder (CTLPD) rarely encountered in children.

Onychopathie associée au sirolimus chez les transplantés rénaux

Resume Introduction De nombreux medicaments sont incrimines dans la survenue d’alterations ungueales. Le sirolimus est un immunosuppresseur de la famille des rapamycines recemment developpe en prevention du rejet des organes greffes. Ce travail a etudie les alterations associees au sirolimus au sein d’un groupe de malades transplantes renaux. Malades et methodes Les ongles de 80 malades consecutifs, greffes renaux, traites par sirolimus, ont ete examines par un dermatologue de facon systematique en 2003. Il s’agissait majoritairement d’hommes (60 p. 100), d’âge moyen 48 ans et greffes renaux en moyenne depuis 6 ans. Le sirolimus etait donne en moyenne depuis 18 mois et associe a du mycophenolate mofetil et des corticoides chez 86 p. 100 des malades. Resultats Cinquante-sept malades (74 p. 100) avaient des alterations ungueales dominees par une atteinte matricielle (pousse lente, ongles cassants, onychomalacie, onychorrhexie et leuconychies) chez 88 p. 100 d’entre eux. Des atteintes du lit de l’ongle (onycholyse), des phenomenes vasculaires (erytheme, hemorragies en flammeche), et des atteintes peri-ungueales (granulome pyogenique essentiellement) etaient rapportees chez respectivement 42, 42 et 19 p. 100 des cas. Un cas de photo-onycholyse de type 1 a ete observe. Discussion Ce travail met en evidence des alterations ungueales monomorphes dominees par une atteinte matricielle dans un groupe homogene de greffes renaux recevant tous du sirolimus. L’imputabilite de ce traitement est consideree comme importante et l’hypothese physiopathologique la plus seduisante fait intervenir une inhibition de l’EGF (epidermal growth factor) par le sirolimus.

Treatment of severe alopecia areata by topical applications of cyclosporin A: comparative trial versus placebo in 43 patients

Forty-three patients with severe alopecia areata were divided into two groups: group 1 (n = 22) treated with once-daily application of 0.5 ml of an oily solution containing 100 mg/ml of cyclosporin A (CyA) to one half of the scalp during 6 months; group 2 (n = 21): once-daily application of an oily solution without CyA to one half of the scalp. The development of terminal hairs, generally in the form of small tufts 0.5 to 2 cm in diameter was observed in seven patients in group 1 and not in group 2. No cases of complete hair regrowth were observed. Diffuse vellus hair predominant on the treated side was observed in five patients in group 1 and in three patients in group 2. The local tolerance was good. A few episodes of transient folliculitis (four cases in each group) were noted. The laboratory survey (serum electrolytes, creatinine, uric acid) did not reveal any significant variations and cyclosporin blood levels were always below detectable limits. CyA has never been used topically in man prior to this trial. This comparative trial demonstrated a difference between the two groups, as terminal hairs were observed in seven cases in group 1. This regrowth was mild, of delayed onset and never complete. However, the development of terminal hairs in the treated group suggests a local action of CyA and encourages further therapeutic trials with different concentrations and different vehicles.

Blepharophimosis, eczema, and growth and developmental delay in a young adult: late features of Dubowitz syndrome?

The Dubowitz syndrome is a rare autosomal recessive multiple congenital anomaly/mental retardation syndrome. We report here a case of a young adult presenting with several features consistent with this diagnosis. The differential diagnosis is discussed with respect to the absence of microcephaly and intrauterine growth retardation.

New topical immunological treatments for psoriasis

Topical treatments for psoriasis have been used for a very long time. They were designed to reduce epidermal proliferation and hyperkeratosis. Recent pathophysiological data have led to the use of many immunological therapies, initially systemically in severe forms and, in recent years, topically. Calcineurin inhibitors are now a well‐established treatment for atopic dermatitis and have also been evaluated in psoriasis. The first trials of tacrolimus (Protopic®, Astellas) and pimecrolimus (Elidel®, Novartis) failed to demonstrate any positive results on plaque‐type psoriasis, but several studies showed a definite efficacy on facial and intertriginous psoriasis. Calcineurin inhibitors do not induce skin atrophy and are therefore particularly useful in sites in which the skin is thinner (face, intertriginous areas). A third substance, sirolimus (Rapamune®, Wyeth), has recently been tested topically in psoriasis. A recent study demonstrated that topical sirolimus crosses the cutaneous barrier and is effective on psoriasis lesions. These studies therefore show an indisputable efficacy of calcineurin inhibitors and sirolimus in the treatment of localized psoriasis. The major advantage is the absence of skin atrophy. However, large‐scale efficacy and safety studies must be conducted in this indication.