[A syndrome of hyper-IgE and recurrent infections. Developmental variants? A familial study]. / Syndrome hyper-IgE et infections récidivantes. Variantes évolutives? Etude familiale.

The first patient suffered from a very severe atopic dermatitis with intense pruritus and thickened skin. He had also recurrent infections, particularly related to Staphylococcus coagulase +, and axillary and inguinal lymphodermopathy. The use of tetracosactide given intramuscularly allowed controlling the evolution of his atopic dermatitis. After several months of treatment, the skin became less infiltrated, lymphodermopathy disappeared and no severe infection had happened. The second patient had a less severe atopic dermatitis and recurrent infections without any particular severity. Topical corticosteroids allowed to control the atopic dermatitis. These two patients had high levels of circulating IgE and an important deficiency of polymorphonuclear chemotaxis which was evaluated by migration through boyden room. Study of the family showed atopic manifestations in several members, but with lower levels of IgE. The most characteristic abnormality of this syndrome is the according to considerable increase of IgE. The deficit in polymorphonuclear chemotaxis may vary according to time and even become normal. The prognosis over long periods remains to be determined.

Schistosomiasis mekongi diagnosed by rectal biopsy.

Schistosoma mekongi, a parasite of the dog which is endemic in small foci along the Mikong river, is rarely seen in Europe. A case of schistosomiasis caused by S mekongi was recently seen in France, in a native of Laos who had been living in Thailand. He was admitted for treatment of previous diagnosed tuberculoid leprosy but was found to have hepatosplenomegaly, with a high eosinophil count and high bilirubin concentrations. Serological examination suggested schistosomiasis, but there were no parasites in the urine and the rectal mucosa looked normal on endoscopy. Nevertheless, rectal biopsy showed schistosomal proctitis and many ova of S mekongi. Two courses of praziquantel cleared the ova from his rectal mucosa. Although serological examination using S mansoni antigen suggested schistosomiasis, only biopsy of normal looking rectal mucosa allowed the diagnosis to established.

[Acquired leukomelanoderma caused by topical depigmenting agents]. / Leucomélanodermies accidentelles provoquées par les topiques dépigmentants.

Three patients applied a cream containing monobenzyl ether (MBEH) (2 cases) or monomethyl ether of hydroquinone (1 case) on acquired hyperpigmentary disorders. We observed depigmentation locally and at a distance from the treated areas. The depigmenting ability of MBEH was founding in 1939 by Oliver, at the time of an occupational accident. This happended among black workers wearing rubber gloves containing MBEH as an anti-oxydant. The depigmenting ability has been used as a treatment for acquired hyperpigmentary disorders, but adverse effects were observed--irritation and local sensitization--depigmentation locally and at a distance from the treated areas--melanoleukodermia among asiatics--no repigmentation: therefore treatment with MBEH has been used by Mosher in extensive vitiligo on the unaffected areas. As regards to the mechanism of these pigmentary disorders, knowledge is incomplete, particulary concerning depigmentation at a distance: hypothesis was: destruction by degrees of the melanocytes, as in Lerner's theory concerning vitiligo, autoimmune mechanism? Genetic predisposition? Electron microscopy shows cellular disturbances in melanosomes and degeneration of intracytoplasmic membrane thus killing cell. The physiopathology was the work of Riley: he has shown that MBEH is selectively incorporated into cultured melanocytes. Evidence of free radical formation has been obtained in guinea-pig skin treated with this agent. Cultured melanocytes are irreversibly damaged by exposure to low concentrations. Riley postulates that these compounds diffuse into melanosomes of the pigment cell where they are oxidized by tyrosinase to produce free radicals; the latter bring about lipid peroxidation of cell membrane lipids, thus killing the cell.