[Teledermatology--importance of telemedicine and dermatology]. / La télédermatologie--intérêt de la télémédecine en dermatologie.

Due to a constantly improving technology the transfer of clinical images associated with histological images represents the actual answer to the expectation of Dermatologists. The authors give their personal experience regarding the use of telemedicine in Dermatology: discussion of clinical cases, histopathological presentation, diagnostic assistance, follow-up of patients, teaching, and research. Perspectives and development offered by telemedicine as well as the advantages of patient care, and of presenting research topics are mentioned. Also aspects which have to be clarified before telemedicine takes up are presented: as far as that goes for practicing Medicine and Dermatology, technical applicability, credibility, acceptance, confidence and responsibility.

Persistent acantholytic dermatosis: sex-related differences in clinical presentation?

We report the case of a 58-year-old man with a chronic papular eruption of 10 years' duration. Histopathology revealed focal acantholytic dyskeratosis. This condition is thought to represent a distinct entity which has been reported under several names and frequently referred to as persistent acantholytic dermatosis. The relationship between this condition and transient acantholytic dermatosis (Grover's disease), emphasizing sex-related presentations, is discussed.

Pili torti with congenital deafness (Bjornstad's syndrome)--report of three cases in one family, suggesting autosomal dominant transmission.

Pili torti is a rare hair shaft abnormality in which the hair is flattened and intervals twisted at irregular through 180 degrees about its axis. Pili torti may occur as a congenital defect or as an acquired disorder (secondary to patchy alopecia from a variety of causes). When it is congenital, it may be isolated and determined by an autosomal dominant gene or associated with various rare syndromes, including ectodermal dysplasias, neurological defects and metabolic disturbances. The association of neurosensory hearing loss and pili torti has been recognized as Bjornstad's syndrome since 1965. As far as we know, only 15 cases of this syndrome have been reported. We describe here three cases of Bjornstad's syndrome in one family.

Anti-lamin-B autoantibodies in a patient with cold urticaria.

Anti-lamin-B autoantibodies at a significant level had been found on two occasions in the serum of a 56-year-old woman who was suffering from an apparently idiopathic chronic cold urticaria. Anti-lamin autoantibodies can be detected in various autoimmune disorders including hepatitis, vasculitis and peripheral blood cytopenia. In our patient, there was no other clinical or biological abnormality. A chance association cannot be ruled out.

Cutaneous type of adult T cell leukemia/lymphoma in a French West Indian woman. Clonal rearrangement of T-cell receptor beta and gamma genes and monoclonal integration of HTLV-I proviral DNA in the skin infiltrate.

A 45-year-old woman, a native of the French West Indies who had lived in France since 1973, developed multiple cutaneous plaques and nodules in 1987. Histopathologic studies revealed dermal infiltration with mature activated T cells (CD4+, CD25+, DR+) with nuclear convolutions and epidermatotropisim. High titers of specific human T lymphotropic virus (HTLV)-I antibodies were detected in the serum. Molecular analysis of DNA extracted from the skin tumor biopsy specimen showed a clonal integration of an HTLV-I provirus and a T-cell clonal population as demonstrated by T-cell receptor beta and gamma gene rearrangement studies. Neither HTLV-I provirus nor T-cell receptor rearrangements were detected in peripheral blood mononuclear cells DNA despite the presence of rare adult T cell leukemia cells (less than 1%) and a small excess of DR-expressing cells, and detection of HTLV-I Pol and Px sequences by in vitro gene amplification. In this case only gene analysis of the skin lesions made possible an early diagnosis of a cutaneous adult T cell leukemia. This illustrates the need for such molecular studies to differentiate, in HTLV-I seropositive patients from endemic areas, a HTLV-I-induced T cell lymphoma from HTLV-I-nonrelated cutaneous T cell lymphomas.

Familial occurrence of KID (keratitis, ichthyosis, deafness) syndrome. Case reports of a mother and daughter.

Keratitis, ichthyosis, and deafness (KID) syndrome in a mother and her daughter is reported. Both patients showed the typical cutaneous abnormality of papillomatous hyperkeratosis, which gave the skin a grainy, rugose, spiculated appearance, together with keratitis with corneal neovascularization and a neurosensory hearing defect. Treatment with etretinate did not benefit the daughter. The mother had marked keratitis with progressive corneal opacification that required repeated bilateral corneal grafts, which were unsuccessful. This is the second report of hereditary transmission of this disorder. An autosomal dominant mode of inheritance is probable.

[Epithelio-lymphohistiocytic tumor. Apropos of 3 cases]. / Tumeur épithélio-lympho-histiocytaire. A propos de 3 cas.

We report three cases of epithelio-lympho-histiocytic tumour with very original histological features. The patients were young adults presenting with a nodular lesion on the face which had been present for several years. No recurrence was observed after surgical excision. Histologically, the dermal lesion consisted of epithelial pouches edged with a few layers of basal-like cells and filled with lymphocytes and large clear cells. Relations were found between these lobules and hair follicles, and rudiments of piliary differentiation were noted. The stroma was dense and infiltrated by small lymphocytes and large clear cells. An immunohistochemical study showed marking of the large clear cells by the S 100 protein, which suggested that they were Langerhans cells. The lymphocytes were recognized by the common panleucocyte antibody. The KL 1 antibody marked a few isolated cells within the lobules, but not the basal-like cells. These cases seemed to be similar to the 7 cases reported in the literature by Santa-Cruz and Barr who used the term lymphoepithelial tumour of the skin. We felt justified in putting the stress on the histiocytic component of this tumour and calling it epithelio-lympho-histiocytic tumour. We agree with these authors that this is a tumour of the appendages of the skin and in particular the hair.