BCG-vaccine-induced lupus vulgaris and urticarial vasculitis.
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Biomedical subjects
Publications and source records attributed to P Combemale.
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The authors report a case of association between lupus vulgaris and urticarial vasculitis after BCG vaccination. Lupus vulgaris is a rare classical complication of BCG vaccination. Anaphylactoid reactions after BCG are very rare. This is the first report of association between the two diseases after BCG and the third report of urticaria after BCG. The pathogenesis of this is discussed.
Pityriasis rotunda (PR) is a well-known skin disease in Japan, but it is seldom observed in Europe. Because it is often associated with an underlying, sometimes malignant disease, it is regarded by some authors as a clinical form of acquired ichthyosis. We report the case of a 20-year old male Congolese presenting with PR and ichthyosis vulgaris. This rarely described association raises the problem, discussed here, of whether PR belongs to ichthyosis vulgaris.
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The authors report a case of extensive verrucous epidermal naevus of the face in a 15 year old Senegalese boy. This is the second reported case in Western Africa following the case presented to the French Language African Medical Society in 1984 (B. Ndiaye). The skin lesion in the form of a naevus of variable dimensions is an essential manifestation of the epidermal naevus syndrome described by Solomon, Fretzin and Dewald in 1968. This syndrome consists of a variable but inconstant association of dysembryoplastic abnormalities affecting the central nervous system (epilepsy, mental retardation, hydrocephalus, localized central deficits), the eye (fibrous conjunctival tumours, corneal opacities, colobomas) and the bones (spine, clavicle, pelvis, limb bones). The bones may be affected by malformations or hypoplasia. The epidermal naevus generally has a linear verrucous appearance, but it is not exceptional to find Jadassohn's sebaceous naevus or even localized erythroderma ichthyosiformis. Mucosal lesions, especially oral, well described in 1960 by Brown and Gorlin, correspond to a particular localization of epidermal naevus and must be differentiated histologically from white sponge naevus, which has a fairly similar clinical appearance. This non-hereditary disease must be systematically investigated looking for visceral abnormalities which are very common. Lastly, in terms of therapy, surgery may be justified when the facial lesions are unsightly, extensive or disabling. Various techniques may be applied depending on the extent and the site of these naevi.
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We studied 11 consecutive patients with classical cicatricial pemphigoid (CP) using direct immunoelectron microscopy (IEM) and Western immunoblotting analysis. Direct IEM performed in the skin or gingival mucosa revealed in all 11 CP patients that immunoglobulins and complement deposits were usually thick and discontinuous along the dermoepidermal junction, mostly localized on the lamina densa and occasionally in the lamina lucida. By direct IEM, the ultrastructural aspect in CP differs from the pattern observed in bullous pemphigoid (BP) and from that of chronic epidermolysis bullosa acquisita (EBA). Nine CP patients were studied by Western immunoblotting and, of these nine, only two had detectable anti-basement membrane zone (BMZ) antibodies by indirect immunofluorescence on salt-split skin. By immunoblotting performed on protein extracts of heat-separated epidermis, eight out of the nine CP sera specifically reacted with two protein bands of approximately 230-240 kD and 180 kD, similar to those recognized by BP sera in co-migration experiments. By immunoblotting on skin BMZ extracts, none of these nine CP sera recognized the 290-kD major polypeptide of EBA antigen. Taken together, these results suggest that, in CP, the target-antigen, as identified on immunoblots, is similar to BP antigen, but with an abnormal expression within the dermoepidermal junction of patients, which may in part explain the scarring course of the disease.
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We report the case of a 20-year old male patient without significant familial or personal history who presented with a disseminated papular eruption on the abdomen, flanks and buttocks. The eruption was continual and proceeded by outbreaks, but it was not influenced by seasons. Physical examination of the skin, mucosae and skin appendages was otherwise normal. Histological examination showed all the criteria of perforating folliculitis as described by Mehregan and Coskey. On the basis of our case we would put perforating folliculitis back among the perforating dermatoses. The concept of this curious phenomenon is briefly reviewed; perforating folliculitis is part of the third group of the so-called primary forms. The clinical features are suggestive of the disease, with its small pigmented papules centred around a keratotic plug, forming a permanent disseminated eruption. Histology shows a granuloma facing a lateral perforation of the hair follicle, a pseudo-epitheliomatous epithelial hyperplasia and the presence of hair and keratin debris in the perforation. Various keratolytic treatments have been applied without success; retinoids have not been tried. The main diagnostic and nosological problem is Kyrle's disease. In view of clinical and histological data, many authors regard Kyrle's disease as a major form of perforating folliculitis. Pruritus, ascribed to an underlying illness in Kyrle's disease, is thought to increase the importance of the lesions. The specificity of perforating folliculitis is discussed, but it seems that side by side with secondary forms occurring in recognized diseases, there may be primary forms of perforating folliculitis. Dyskeratosis might be a cause of the perforation.
Homogeneous linear IgA deposits at the dermo-epidermal junction (DEJ) shown by direct immunofluorescence are characteristic of what is termed linear IgA bullous dermatosis. However, it is not yet certain that this disease constitutes an entity distinct from other subepidermal blistering diseases, especially when IgG deposits are also present. Sixty-one cases of subepidermal blistering disease in adults were therefore investigated by immunoelectron microscopy (IEM), and the 16 patients observed to have homogeneous linear IgA deposits were compared with the 45 who had no IgA but had IgG and/or C3. In 11 of the 16 patients with IgA (four of whom also had IgG), the deposits were linear and formed a mirror image pattern on each side of the lamina densa from which they were separated by a clear space. In contrast to this monomorphic IEM pattern, clinical and other laboratory findings were very heterogeneous, making exact clinical diagnosis difficult. Of the remaining five patients in this group of sixteen, three (all with both IgA and IgG) had bullous pemphigoid, epidermolysis bullosa acquisita, and cicatricial pemphigoid, respectively, on IEM and clinical investigation. In the remaining two patients (one with both IgA and IgG, and one with IgA only) the deposits were located in the lamina lucida, making precise classification impossible. None of the 45 patients with isolated IgG and/or C3 deposition displayed the mirror image pattern. We conclude that this IEM pattern may constitute a specific diagnostic criterion of linear IgA dermatosis.
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The authors review 6 cases of severe cutaneous gangrene complicating infectious diseases in more or less debilitated infants. The evoked diagnoses were 1 case of septicemic gangrene, 4 cases of extensive cutaneous necrosis of the infant, and 1 severe case of ecthyma gangrenosum. However, the authors believe this classic nosological background while being precise, is restricting and poses the problem of knowing how to classify necrotizing fasciitis.
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A patient aged 65 years was observed to have successive adenomegalic syndromes over a period of 4 years, with variable histological appearances. A right cervical adenopathy had the histological appearance of a simple antigenic-stimulation lesion. Three years later, a right inguinal adenomegaly presented all the histological criteria of angioimmunoblastic adenopathies, without, however, any humoral immunity disturbance. Two months later, a biopsy of a palatine tumour confirmed the diagnosis of an immunoblastic sarcoma. The condition then became generalized as a lymphoblastic type of diffuse lymphosarcoma, and autopsy one year later showed the presence of a pleomorphic lymphosarcoma in several lymphoid formations. This case serves as a basis for discussing the concept of angio-immunoblastic adenopathy; the result of various antigenic aggressions or the early stage of a haematosarcoma. It also clearly demonstrates the sometimes contingent characteristics and difficulties in classification of haematosarcomas.
INTRODUCTION: Impetigo herpetiformis is a rare dermatitis that occurs during pregnancy and may be life threatening for both mother and child. In this case report, we present an Ondine curse involving the baby, and the good response to isotretinoine. CASE REPORT: A first pregnancy, 26 year-old woman developed at 8 months a widespread skin lesion involving the medial side of the thighs, abdomen and intertriginous areas, with a severe systemic toxic condition and fever. Diagnosis of impetigo herpetiformis was made and corticosteroids, methotrexate and cyclosporine were unsuccessful. Isotretinoine rapidly improved the patient with good control of the disease. The full term baby had an Ondine curse. DISCUSSION: Our case is typical of impetigo herpetiformis. Maternal and infant complication may be life threatening and we report a real Ondine curse the etiology of which remains unknown. Moreover, this observation is unusual because the lesions did not clear despite delivery and good treatment. In our opinion, the great improvement with isotretinoine would suggest it could be used as first line treatment.