ABSTRACT
Data concerning 9 cases of Netherton's syndrome (NS) have been collected from 6 French dermatology units (table I). Analysis of these data has confirmed the information previously published, notably the prevalence of congenital ichthyosis erythroderma (CIE) as cutaneous manifestation in the neonatal period (77 p. 100), hair shaft dysplasia being rarely found at that stage (11 p. 100). In the majority of cases (5/9 in our series), CIE evolves in adults as ichthyosis linearis circumflexia (ILC), with trichorrhexis invaginata (TI) as the predominant hair shaft dysplasia. These data are in agreement with the diagnostic elements laid down by Dupré and Traupe. Some points may have been underestimated in the past. They include: hypernatraemic dehydration in the neonate; short stature and low weight (unrelated to endocrine disorders; mental and neurological retardation possibly associated with seizures. Various manifestations of hypersensitivity have been noted in 26 p. 100 of the published cases and in 6 of our 9 patients. The aggravating role played by hypersensitivity may be considered in some cases. NS must be regarded as a broad-spectrum disease the margins of which could be isolated skin manifestations in ILC and ichthyosis erythroderma with various associated disorders in cases with severe illness.
Subject(s)
Hair Diseases/complications , Ichthyosis/complications , Adult , Child , Child, Preschool , Consanguinity , Female , Growth Disorders/complications , Humans , Hypernatremia/complications , Hypersensitivity/complications , Ichthyosis/diagnosis , Infant , Intellectual Disability/complications , Male , Middle Aged , Syndrome , Water-Electrolyte Imbalance/complicationsABSTRACT
We describe 3 cases of keratosis follicularis decalvans (Siemens' disease): a 15 year old boy and a 7 year old boy and his father. They represent 2 different patterns of the disease with different clinical courses and genetic background: an autosomal dominant type of good prognosis with elevated argininemia and absence of follicular atrophy in both father and son, a sporadic type, clinically severe, with follicular atrophy. This raises the question of the nosology of the so-called Siemens' disease, since it includes actually different diseases of variable prognosis.
Subject(s)
Alopecia/congenital , Darier Disease/classification , Adolescent , Adult , Child , Darier Disease/genetics , Humans , Male , Time FactorsABSTRACT
Vaccination against smallpox should be discontinued in all countries except for individuals with a high risk of exposure (WHO, 1980). Since this vaccination is performed less and less often, one must expect complications to occur, the etiology of which may not be recognized. This course of events leads the authors to point out the difficulties in diagnosis and therapy of localized accidental vaccinia encountered in six patients hospitalized in Brest (1971-1979). Diagnosis is considered if the patient himself, or a person he came in contact with, was recently vaccinated. Diagnosis should always be established by virology. Such accidents can be avoided by a faultless vaccination technique and by giving sufficient information to the inoculated subject or to his relatives.
Subject(s)
Smallpox Vaccine/adverse effects , Vaccinia/etiology , Adolescent , Adult , Child , Child, Preschool , Female , France , Humans , MaleABSTRACT
The study of six cases of MacLeod-Donovan chancre, 2 in Paris, 4 in Brest, in young men coming from the West Indies, showed after 15 days incubation, a balano-preputial lesion consisting of an oval-shaped granuloma, 1 to 4 cm diameter, raised, indurated, ulcerated, reddish-yellow, spontaneously painful, bleeding easily, accompanied in 50 p. 100 of cases by inguinal adenitis, due to secondary infection. Swabs, which should be repeated, showed Donovan bodies within numerous histiocytes. The inactivity of penicillin may be contrasted with the rapidly favourable effect of streptomycin, chloramphenicol or aureomycin.
