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Resume Introduction L’hypokeratose palmaire ou plantaire circonscrite est une entite nouvelle, caracterisee en 2002. Il s’agit d’une lesion arrondie resultant d’une diminution majeure d’epaisseur de la couche cornee, d’evolution chronique et posant un probleme de diagnostic differentiel avec la maladie de Bowen ou la porokeratose. Nous presentons deux cas de cette affection, l’un plantaire et l’autre dans une localisation inedite au dos d’un doigt, accompagnes d’une etude microscopique et immunohistochimique. Observations Cas 1 . Un homme de 65 ans presentait une lesion erythemateuse arrondie de 1,5 cm de diametre sur le bord interne du pied gauche, entouree d’un bourrelet keratosique. Cette lesion avait deja ete biopsiee 15 ans auparavant et interpretee alors comme une keratolyse ponctuee ; elle etait restee inchangee depuis. La relecture montrait en fait une diminution brusque d’epaisseur de la couche cornee, avec au centre une acanthose moderee et une dilatation des vaisseaux papillaires. Un immunomarquage des papillomavirus humains (PVH) etait negatif. Cas 2 . Une femme de 75 ans avait une lesion chronique arrondie du dos de l’index droit, inchangee depuis plusieurs mois. Apres une premiere biopsie montrant des cellules claires, elle etait traitee sans succes par cryotherapie, 5-fluoro-uracile et imiquimod. Une deuxieme biopsie montrait une brusque diminution d’epaisseur de la couche cornee, surmontant un epiderme acanthosique contenant des cellules claires. Le marquage PVH etait negatif et le taux de proliferation des cellules basales plutot inferieur a la peau saine, apres immunomarquage utilisant l’anticorps Ki67. Discussion Notre premier cas constitue un exemple caracteristique de l’hypokeratose circonscrite : longue duree d’evolution, semeiologie typique, anomalie tres limitee de la couche cornee. Au contraire, le cas n o 2 illustre une forme originale, non palmaire ni plantaire, plaidant en faveur de l’appellation, plus exacte d’hypokeratose acrale circonscrite. Nous n’avons trouve aucun argument en faveur d’une cause virale ou d’une proliferation anormale des keratinocytes. Comme souvent dans les cas publies, les traitements locaux se sont reveles inefficaces. La chronicite de cette hypokeratose, avec evolution de plusieurs dizaines d’annee, demontre sa benignite, la physiopathologie de cette anomalie epidermique restant toutefois mysterieuse.

Palmar or plantar circumscribed hypokeratosis is a remarkable clinicopathologic entity described in 2002. It consists in a well demarcated decrease in thickness of the stratum corneum, that can be clinically mistaken for Bowen's disease or porokeratosis. We present a classical plantar localisation and a more original case on the dorsum of the finger, together with a microscopic and immunohistochemical study.Case 1. A 65-year-old man was seen 15 years after a first consultation for a well demarcated 1.5cm erythematous lesion localised on the border of his left foot. The biopsy, then misinterepreted as keratosis sulcata, was reviewed. It showed a sudden and well demarcated decrease in thickness of the stratum corneum, overlying a slightly acanthotic epidermis, associated with dilated capillaries in the papillary dermis. HPV immunostaining was negative. Case 2. A 75-year-old woman had a well demarcaated erythematous lesion of the dorsum of her right index finger, lasting for months without significant evolution. A first biopsy showed pale and haloed keratinocytes that could be interpreted as koilocytes. She was therefore treated by cryotherapy, 5-fluro-uracile and imiquimod, that proved unsuccessful. A second biopsy showed a sudden and major decrease in thickness of the stratum corneum, overlying an area containing a few pale keratinocytes with perinuclear halo. HPV immunostaing was negative and Ki67 positive cells were slightly decreased in number when compared to lateral normal skin.Our first case is typical of plantar hypokeratosis characterised by its long evolution, typical semiology and well demarcated anomaly of the stratum corneum. Our second case is original as it shows that the disease can also affect the dorsum of the fingers. Acral circumscribed hypokeratosis is therefore a better name for this condition. We did not find any arguments in favor of a viral cause or an increased proliferation of keratinocytes. As often described in other cases, the lesion can remain unchanged for decades, which confirm its benign evolution. Topical treatments are generally ineffective. The pathogenesis of this localised hypokeratosis remains mysterious.

SummaryBackground The long-term prognosis of patients treated for erythema migrans has only rarely been assessed. Objectives To evaluate the clinical characteristics and long-term prognosis of patients treated for erythema migrans in the region of Alsace, France. Methods In a prospective study, 56 consecutive patients presenting with erythema migrans at the Strasbourg University Hospital between 1995 and 1999 were examined and a Borrelia burgdorferi enzyme immunoassay was performed. Patients were treated with tetracyclines or amoxycillin. Patients were re-examined 6 weeks later and a telephone interview was performed in summer 2000 to evaluate the long-term outcome. Results There were 25 women and 31 men of mean age 49 years presenting with single (n = 54) or multiple (n = 2) erythema migrans lesions. At the time of diagnosis, 30% of the patients had systemic signs, myalgias or arthralgias and only 36% of 50 patients were seroreactive against B. burgdorferi. None of the 51 patients evaluated at 6 weeks and none of the 37 patients interviewed after a median delay of 3 years had developed complications attributable to Lyme borreliosis. Conclusions The prognosis of patients treated for Lyme borreliosis in this part of France is excellent. Therefore, a complete clinical examination is sufficient as an initial evaluation and long-term follow-up is not necessary.

In Europe, Borrelia burgdorferi sensu stricto, Borrelia afzelii and Borrelia garinii are known as pathogens among the Borrelia burgdorferi sensu lato group. Since it is not yet known which Borrelia are responsible for Lyme borreliosis in France, the objective of this study was to identify the species of Borrelia responsible for erythema migrans in the region of Alsace, France.Eighteen patients with erythema migrans (EM) of more than 5 cm of diameter were included in this prospective study. All patients were investigated at the Strasbourg University Hospital. Patients were biopsied on the active border of their lesion. Cutaneous biopsies of the active border of the lesion were cultivated in BSK-H medium (Sigma) and analysed in vitro by PCR after 8 weeks of culture, using flagellin consensus sequences which are present in all species of Borrelia burgdorferi sensu lato group as primers. Species-specific oligotyping was used for species identification.Among the 18 patients biopsied, 7 had evidence of borrelia infection revealed by culture and/or PCR. Borrelia afzelii was detected in 4 patients and Borrelia garinii in three.These preliminary results appear to confirm that Borrelia afzelii and Borrelia garinii are the predominant borrelial species in EM lesions in our geographic area, as in other European countries.

Hereditary progressive mucinous histiocytosis is an uncommon condition which to date has only been observed in women. The disease is a non-Langerhans histiocytosis with a stereotypic clinical presentation limited to the skin. The clinical, histological and genetic features of this disease differ considerably from other types of histiocytoses and overload diseases. We report the first case observed in France.A 49-year-old woman consulted for asymptomatic papules on the dorsal aspect of the hands known since childhood. The lesions had progressively spread to other regions of the body. The woman's mother and two sisters had the same condition but her two sons and her brother and nephews did not. Standard semi-thin slices and electron microscope histology demonstrated dense cellular infiltration of the reticular dermis with interstitial mucinous overload, voluminous histiocytes with characteristic toluidine blue positive granulations, and a cytoplasm rich in myelin and Zebra bodies respectively. The observed clinical, histological and genetic features were characteristic of progressive hereditary progressive mucinous histiocytosis.The pedigree in our case and in those reported in the literature suggest a dominant hereditary condition. The fact that only female cases have been reported to date would suggest X-linked transmission with early death of male fetuses. An autosomal dominant transmission limited to woman as a result of mitochondrial heredity or hormone-related phenomena cannot be ruled out. The progressive extension with age without a tendency for spontaneous regression and the electron microscopy aspect suggest a phospholipid deposit disorder similar to that observed in lysosomial diseases. However, primary macrophage proliferation in response to an unknown stimulus cannot be excluded.