Sondes Makni

Lichen planus pemphigoides: four new cases and a review of the literature

Lichen planus pemphigoides (LPP) is a rare autoimmune blistering disease. It appears to be combination of lichen planus and bullous pemphigoid. We describe four new cases of LPP and discuss the epidemiological, clinical, pathological, and therapeutic features of this singular association through a review of the 74 published cases within the English literature. We report four cases of LPP (three women aged respectively 47, 51, and 53 years old, and a 53‐year‐old man). All patients presented with bullae on lichenoid and normal skin, predominately on the extremities. The diagnosis was confirmed by immunohistological findings. Our patients were treated with oral corticosteroids with a good response. Our review of the literature of 78 cases of LPP (65 adults and 13 children) showed that it involved adults (mean age: 54 years), with a slight female preponderance. A mean lag time between LP and the development of LPP was 8.3 months. LPP is characterized by developing blisters on lichenoid lesions and on uninvolved skin with more acral distribution of bullous lesions. Involvement of palms and soles was more frequent in children. The diagnosis is based on pathological and immunological confrontation. LPP is usually idiopathic, but some cases were reported in association with various drugs. There have also been reports of association with internal malignancy. Most cases of LPP are successfully treated with systemic corticosteroids. In most cases, the prognosis was good.

Immunogenetics of pemphigus: An update

Pemphigus are rare but informative models of organ-specific autoimmune diseases, resulting from the interplay of environmental, genetic and stochastic factors. There are many arguments to consider that pemphigus have a genetic basis involving, as many other autoimmune diseases, several different genes with additive or synergistic effects. So far, the unique strategy used to identify the contributive loci has been direct analysis of candidate genes through conventional case-control association studies. The major histocompatibility complex in particular the class II locus was demonstrated to be associated with pemphigus with a high rate of replicability. The progresses in the understanding of pemphigus physiopathology and the development of new molecular tools offer new perspectives to unveiled the genetic basis of this group of autoimmune blistering diseases, as shown by recent studies of candidate genes expressed at different levels of the autoimmune process.

IgG/IgA pemphigus with IgG and IgA antidesmoglein 3 antibodies and IgA antidesmoglein 1 antibodies detected by enzyme-linked immunosorbent assay: a case report and review of the literature

IgA pemphigus represents a group of autoimmune blistering skin diseases characterized by intraepidermal neutrophilic pustules, intercellular IgA antibodies, or circulating IgA antibodies to keratinocyte surfaces. Currently, it is seen as a distinct subset of pemphigus with particular clinical and immunopathologic pictures. As it is a newly defined disorder, its frequency is unknown but surely rare. Even less reported is the pemphigus with deposits of both IgG and IgA on keratinocyte cell surfaces; a new entity described in the recent literature as IgG and IgA pemphigus. In this study, we report the first Tunisian case with IgG ⁄ IgA pemphigus and try to discuss the clinical and immunopathological features of this new type of pemphigus.

High Prevalence of Antineuronal Antibodies in Tunisian Psychiatric Inpatients

The authors aimed to determine the prevalence of antineuronal antibodies in 103 psychiatric inpatients and 41 control subjects with no history of malignancies or neurological disorders. All sera were tested by indirect immunofluorescence and positive sera by immunoblot. Using immunofluorescence, antineuronal nuclear autoantibodies were detected in 20 patients and none of the control subjects, and antibodies reacted with the cytoplasm of Purkinje cells in six patients and two control subjects. The immunoblot confirmed well-characterized antineuronal antibodies only in five patients: two had anti-Ri and three had anti-Yo antibodies. After a follow-up of 5 years, none of these patients developed neurological disorder or malignancy.

Human herpesvirus-8 and hepatitis B and C virus infections in pemphigus.

we obtained clinical resolution. No recurrence was observed over the 11-month period. Pseudochromhidrosis is characterized by the secretion of colorless sweat, which becomes colored when it reaches the skin surface because of contact with dyes, exo-genous pigments, or products derived from chromogenic microorganisms (corynebacteria, Bacillus spp., Piedraia, Malassezia furfur). The differential diagnosis includes chromhidrosis, where it produces colored sweat from eccrine or apocrine sweat glands. The apocrine form is caused by the increased concentration or a change in the oxidation state of lipofuscin, the pigment normally present in the apocrine sweat. In the eccrine form, less commonly, sweat glands release sweat in the water-soluble pigments derived from the ingestion of drugs or dyes. Pseudochromhidrosis, eccrine chromhidrosis, and apocrine chromhidrosis differ not only in pathogenesis but also in terms of prognosis and treatment: in the first two forms, we can achieve complete healing by removing the cause of the pigmentation of the sweat; in the last one, we can act only with symptomatic drugs, such as pilocarpine, and reduce or inhibit sweating. Apocrine chromhidrosis is a chronic disease that occurs in puberty; when under hormonal stimulation the apocrine secretion activates and tends to resolve spontaneously in adulthood, when physiologically apocrine secretion is reduced. In our case, videodermoscopy was of great help, which allowed us to evaluate the distribution of pigment on the skin surface.

High B-cell-activating factor levels in endemic Tunisian pemphigus

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