Biomedical subjects
Publications and source records attributed to C Bedane.
A 69-year-old man with a history of relapsing polychondritis developed a widespread cutaneous eruption that was clinically and histologically typical of erythema elevatum diutinum. This eruption was accompanied by a recurrence of arthralgias. The significance of this association between relapsing polychondritis and erythema elevatum diutinum, which has not been previously reported, is discussed.
Subepidermal autoimmune bullous dermatoses are defined by clinical, histological and immunological criteria, notably the presence of anti-basement membrane antibodies detectable in vivo by direct immunofluorescence. We report three cases where anti-basement membrane antibodies were not detectable in vivo by direct immunofluorescence but were detected in high titres by indirect immunofluorescence. This situation is extremely rare in the literature. The first case concerns a 69-year old woman seen for bullous and pruriginous lesions of the lower limbs of 2 months' duration. Histological examination found dermoepidermal bullae with, in their lumen, a serous fluid spotted with numerous polymorphonuclears. A search for anti-basement membrane antibodies was positive in significant titres (1,280; 320; 480) at indirect immunofluorescence on rabbit lip whereas five successive direct immunofluorescence test in perilesional skin and on the thigh medial surface remained negative. The second case is that of a 91-year old woman suffering from generalized pruritus associated with erythematous lesions predominant on the extension surfaces of the forearms and thighs, without any bullous lesion. Pathological examination only showed a superficial dermal lymphocytic infiltrate. Four direct immunofluorescence tests were negative, whereas a search for anti-basement membrane antibodies on rat oesophagus was positive at 1/1,280. The third case resembles the second one. It concerns a 72-year old woman who consulted for generalized pruritus of several months' duration which interfered with sleep and was incompletely relieved by emollients. There was no specific skin lesion. Pathological examination revealed nothing more than a discrete perivascular mononucleate infiltrate. Direct immunofluorescence tests performed on two occasions on the skin of the abdomen and of the medial thigh surface were negative.(ABSTRACT TRUNCATED AT 250 WORDS)
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A 74-year-old woman developed a generalized pustular dermatosis with erythema-multiforme-like and vesiculobullous lesions 10 days after beginning a treatment by nadoxolol. Despite the clinical polymorphism of the eruption, histology constantly revealed the presence of subcorneal pustules, even within the epidermis overlying subepidermal bullae. The eruption spontaneously healed after discontinuation of nadoxolol.
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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.
A case of chronic urticaria-like lesions with macroglobulinaemia (Schnitzler syndrome) is described. This case is unique by its double IgM monocomponents and by the follow-up study which shows immunoglobulins modifications between cutaneous flare-up and remission. As 1 case had a lymphoma, we studied the mononuclear cells of the bone marrow of Schnitzler syndrome to raise a possible malignant evolution of that disorder, and we discovered a hypodiploid population, which we compared with similar studies of myeloma and monoclonal 'benign' gammopathies.
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We prospectively studied 42 adult patients with acute dermis and soft-tissue infections (27 with erysipelas and 15 with acute cellulitis) involving the lower limb in all except one case. Streptococcus organisms (groups A, C, D, and G) were researched in skin biopsy specimens by a direct immunofluorescent (DIF) technique using commercially available antibodies. Our results showed that DIF gives a sensitivity of 0.70 for the in situ detection of streptococci in cases of erysipelas and cellulitis. With the obvious contribution of this DIF technique, streptococcal pathogens could be detected in situ and grouped in 19 of 27 cases of erysipelas (group A, 13; group B, 1; group C, 1; and group G, 4) and in ten of 15 cases of cellulitis (group A, 9; group B, 1). Combined data, including conventional cultures, DIF studies, and serologic findings, established that Streptococcus organisms, especially Streptococcus pyogenes (A), were, in nearly all cases, responsible for both erysipelas (26/27 cases) and acute cellulitis (11/15 cases) involving the lower limb in adults.
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We report two patients in whom angiosarcoma of the aorta (ASA) with distal emboli and skin metastases was initially clinically misdiagnosed as polyarteritis nodosa (PAN). Both presented with features highly suggestive of systemic necrotizing vasculitis, including constitutional symptoms, orchitis (in one), leg pain with sensory neuropathy, livedo, focal areas of skin necrosis, painful cutaneous nodules demonstrating vasculitis or inconspicuous changes on biopsy, raised acute phase reactants, and transient improvement with systemic corticosteroids. Repeated skin biopsies revealed an atypical endovascular cell proliferation with strong reactivity for factor VIII. Magnetic resonance angiography (MRA) demonstrated an intra-luminal aortic tumour at the infra-renal level. In retrospect, the (PAN)-like features appeared to involve exclusively the lower part of the body in these patients.
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a possibly misleading presentation. It is rare in childhood. We report an 18-month-old child with an acquired atrophic plaque of the left ankle. Based on clinical findings, initial proposed diagnoses included localized scleroderma and localized lipoatrophy. At the age of 5 years the plaque had increased in size and become a purplish color. Several firm nodules were seen at its periphery. A diagnosis of DFSP was proposed and confirmed histologically. A large surgical resection was performed. This case illustrates that DFSP may occur in childhood, during which initial diagnosis is generally more difficult than in adulthood.