[Congenital exfoliative erythroderma].
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Biomedical subjects
Publications and source records attributed to R Tomb.
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BACKGROUND: Verrucous carcinoma of the oral cavity is a rare entity that was formerly controversial. Etiopathogenesis remains unclear, notably as for its possible association with lichen planus. We report a case of verrucous carcinoma occurring in lesions of lichen planus of the tongue. CASE REPORT: A 78-year old, non smoking patient, with past history of cutaneous lichen planus presented for lesions of oral lichen planus affecting both the tongue and the palate. A treatment by topical tretinoin improved him in a spectacular way and brought about a remission which lasted 5 years. A recurrence occurred when the treatment was stopped; new whitish, warty cauliflower-like lesions appeared on the tongue. A biopsy confirmed the clinical suspicion of verrucous carcinoma. A laser resection was performed. Three months later, another recurrence was observed. A chemotherapy associating isotretinoin and methotrexate eliminated all lesions. The patient's condition is considered stable, under treatment, one year later. DISCUSSION: Verrucous carcinoma is a rare slow-growing oral tumor that is chiefly exophytic and does not metastasize, but it can invade and destroy oral tissues. Its clinical presentation contrasts with benign histologic features: papillomatosis, acanthosis, dysplasia in variable degrees. The occurrence on lesions of lichen planus, although "classic", is very rarely found in the literature. The treatment is not well codified. An additional chemotherapy seems necessary to prevent recurrences.
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BACKGROUND: Erythromelanosis follicularis of the face and neck, originally described in Japan by Kitamura et al. in 1960, is characterized by a clinical set of three: well-demarcated erythema, hyperpigmentation and follicular papules. It affects the face and the neck generally on both sides. Since the original description, it has seldom been reported in the literature. CASE REPORTS: This paper reports two patients with unilateral presentation. DISCUSSION: Having discussed the various differential diagnoses all published cases were listed and analyzed. The prevalence of this disease appears higher than is shown by the limited number of cases reported in the literature. It deserves more recognition. Its nosologic and aetiologic frames still remain to be clarified.
Hirsutism is a common medical condition that can lead to major psychological and social distress. Patient's complaint is usually cosmetic in nature, but hirsutism can sometimes signal a serious underlying disorder. Detailed medical history and physical examination help identify patients in whom extensive work-up is warranted. Therapy that is directed at suppressing androgen secretion, inhibiting the conversion of testosterone to dihydrotestosterone, or blocking the effects of androgens on their receptors, can be rewarding both for the treating physician and the patient. This article reviews the different agents used against hirsutism and addresses the evolution of their indications based on published clinical studies.
BACKGROUND: Skin ulcerations are rarely reported in dermatomyositis of the adult. We report on a case of perforating ulcers resistant to classical treatments in a woman with dermatomyositis. CASE REPORT: A 34 year-old woman, treated for typical dermatomyositis, developed a few weeks after starting her treatment, multiple perforating skin ulcers on her shoulders, elbows and wrists though general signs had completely disappeared. In spite of the increase in oral steroids, the patient presented again with worsened lesions and a new perforating ulcer on the hand which lead to the breaking of a tendon. She underwent hand surgery and was also treated with a bolus of steroids IV (1 g daily during 3 days), followed by oral steroids, IM methotrexate and antimalarial drugs. The lesions healed completely after one month, with no atrophy. DISCUSSION: Skin ulcers are rarely described in dermatomyosites of the adult and are associated with underlying vasculitis and bad prognosis. Our case is remarkable by the absence of vasculitis, a very slow cure requiring aggressive treatment and excellent prognosis after three years of follow-up.
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Skin irritation induced by tretinoin is commonly observed, whereas allergic contact dermatitis to this drug is very rarely found in the literature. We report the case of a 34-year-old man who developed contact dermatitis in areas where a topical tretinoin preparation was applied. Patch tests were positive to tretinoin in low concentration and to isotretinoin. Oral administration of isotretinoin to this patient would carry a risk of systemic complications.
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For etiologic checking of cutaneous vasculitis, skin testing may be of valuable help: epicutaneous patch-tests with delayed lecture (48 h.) performed with contact haptens (rubber allergens, azo dyes, resins ...) or drugs in pigmented progressive purpuric skin eruptions; prick-tests submitted to delayed (6-8 h.) clinic and histologic lecture, performed with miscellaneous drug or food-derived, microbial or environmental antigens in necrotizing vasculitis. In these conditions skin testing should be approached with great caution, because of the occurrence of non relevant positive reactions and the risk of precipitation of cutaneous or disseminated lesions of vasculitis.
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Safety clothing and individual protective devices may induce contact dermatitis. Typical clinical patterns cannot always be found. Patch testing must be performed with articles actually used and with specific allergens. In some cases, it is necessary to analyze chemically the article itself.
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BACKGROUND: Junctional epidermolysis bullosa (JEB) represents a genetically heterozygous group of bullous disorders characterized by dermo-epidermal separation resulting from mutations affecting the main dermo-epidermal adhesion factor, laminin-5, its cellular receptor, integrin alpha6B4, or collagen XVII. We report the identification of a new mutation of LAMA3, encoding laminin-5 alpha3 subunit in two unrelated Lebanese families. PATIENTS AND METHODS: Two female newborn, descending from 1st degree consanguineous marriages, presented a lethal form of EBJ-Herlitz. Histologic, ultrastructural and immunofluorescence studies were performed in order to ascertain the diagnosis and to direct genetic analysis. Mutation search was conducted through direct DNA sequencing of patients and ascendants. RESULTS: Immunohistology of frozen skin samples revealed an extremely reduced immunoreactivity for the alpha3 laminin-5 subunit. The two patients were homozygous carriers (parents heterozygous) of a new missense mutation of LAMA3 gene (exon 32: 4300 insA) encoding the alpha3 subunit of laminin-5. Resulting messenger RNA, rapidly degraded, induced an extremely reduced synthesis of alpha3-polypeptide, truncated in its Cterminal domain. DISCUSSION: LAMB3 gene recurrent mutations R636X and R42X account for about 50p. 100 of EBJ cases affecting Caucasians while mutation Q1083X, affecting the same gene, is recurrent in Arab populations. The newly identified mutation results in extremely reduced synthesis of alpha3 chain and truncation of its C-terminal domain, which is crucial for the intermolecular interactions of laminin-5. Our data are in accordance with recent reports suggesting geographical specificity of EBJ mutations linked to founder effects which are amplified by consanguineous marriages in genetically isolated populations. Otherwise, the observation of other unexplored cases of bullous dermatoses with early demise originating from the same region of the two families herein reported highlights the need for the implementation of a prenatal and postnatal diagnostic strategy regarding these genodermatoses. These studies should target LAMA3 and other genes involved in JEB too.