[Solar urticaria: one case treated by intravenous immunoglobulin]. / Urticaire solaire: un cas traité par immunoglobulines polyvalentes.

BACKGROUND: Solar urticaria is a rare photodermatosis which often begins from the third to the fifth decade. Usual treatment consists of photoprotection measures and antihistamines although disease control is sometimes unsatisfactory with both. We report herein a very severe case of solar urticaria we treated with intravenous immunoglobulins. CASE-REPORT: A 55-year-old woman suffered for 3 years from very severe solar urticaria which resisted treatment. Phototests revealed two action spectra: the first in UVA near 380 nm with a minimal urticarian dose of 0.025 J/cm(2), the second near 500 nm in visible light. RESULTS: As last resort treatment, we gave our patient intravenous immunoglobulins. After the third course of treatment, the improvement was impressive as the patient could tolerate visible light and 15 minutes of intense solar exposure. The minimal urticarian dose was raised from 0.025 J/cm(2) to 27 J/cm(2) in UVA. One year after treatment, the solar urticaria has disappeared. CONCLUSIONS: We report herein the first case of solar urticaria treated with success with immunoglobulin. Intravenous immunoglobulin treatment is well for its effectiveness in many autoimmune diseases such as autoimmune thrombocytopenic purpura, and also, as recently proven, in some cases of severe idiopathic chronic urticaria.

Treatment of nine cases of pemphigus vulgaris with cyclosporine.

The effects of cyclosporine were studied in nine patients suffering from pemphigus vulgaris. Of four patients treated with cyclosporine alone, only one cleared. Of four corticosteroid-resistant pemphigus vulgaris patients, all improved after cyclosporine addition to corticosteroids. The last patient treated from the beginning with a combined treatment (cyclosporine-corticosteroids) did not respond. The main advantage of using cyclosporine is to allow a decrease in corticosteroid dosages and to permit treating corticosteroid-resistant pemphigus vulgaris patients. No detectable irreversible side effects were noted. The treatment was discontinued in two patients because of reversible side effects. Cyclosporine alone does not seem to be an adequate treatment of the acute phase of pemphigus vulgaris but could be used in addition to corticosteroids. The most important drawback of cyclosporine treatment is the occurrence of clinically silent renal dysfunction (tubular involvement and interstitial fibrosis), which may occur during long-term treatments. More studies need to be carried out to determine the effects of low doses of cyclosporine on renal function in patients who have normal renal functions.

[Skin manifestations of refractory anemia with excess of blasts (RAEB)]. / Signes cutanés des anémies réfractaires avec excès de blastes (AREB).

The authors describe three cases of refractory anaemia with an excess of myeloblasts in the bone marrow (RAEM), associated with pyoderma gangrenosum (PG) and vasculitis. The first patient was an 85-year old man whose RAEM had begun in 1979. In 1985, he developed pyoderma gangrenosum in the popliteal fossa and on the right heel. Histology confirmed the diagnosis. A direct immunofluorescence test on the biopsy specimen was negative. Apart from the haematological syndrome, there were no laboratory abnormalities. The cutaneous lesions disappeared after 7 weeks of treatment with prednisolone 20 mg per day. The blood disease remained unchanged. The second patient was a 71-year old woman with RAEM since 1982. In 1984, she presented with lesions of cutaneous vasculitis located on the anterior aspect of the upper third of her left leg. There was neither arthralgia nor fever, and no history of drug toxicity or infection. Beside RAEM, polyclonal hyperglobulinaemia was present. Histological examination of the skin showed evidence of vasculitis with fibrinoid necrosis of vascular walls and perivascular lympho-histiocytic infiltrate with granulocytes and slight leucocytoclasia. Direct immunofluorescence testing of the skin demonstrated intravascular complement deposits. There were no circulating immune complexes; measurements of complement and complement fractions gave normal values; no cryoglobulin was found. The cutaneous lesions recurred on two occasions in 6 months, although no drug toxicity or infection could be elicited and the haematological syndrome was unaffected. The third patient was a 67-year old man with RAEM since 1982. In 1983, an ulcero-necrotic lesion spontaneously developed on his right leg.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute generalized exanthematous pustular dermatitis and viral infection.

Three patients with acute generalized exanthematous pustular dermatitis (AGEPD) are presented. This new clinical entity comprises generalized erythema, erythema multiforme, amicrobial pustules, fever and spontaneous healing over a 10-day period. Histologically, there are a mixed neutrophilic and eosinophilic vasculitis and an eosinophilic subcorneal and spongiform pustule. All the patients experienced pharyngitis 1 week before the onset of the rash. Two of them had high-titered IgM-neutralizing antibodies to enteroviruses and 1 had an enterovirus in stool and urine. AGEPD may be related to a viral infection and may occur in patients with a genetic predisposition to psoriasis. The diagnosis of AGEPD should be made early enough so that the patients are not given the often aggressive therapy of pustular psoriasis.

Langerhans' cell and vitiligo: quantitative study of T6 and HLA-DR antigen-expressing cells.

Epidermal Langerhans' cell (LC) densities in vitiliginous skin (VS) and normal-appearing skin (NAS) were studied in 10 patients with common vitiligo. Monoclonal mouse anti human T6 antigen IgG1 and Ia antigen IgG2 (Ortho Pharmaceuticals) were used to characterize LC. Epidermal LC densities were calculated by means of an ocular square grid and expressed per 0.1 mm2. The results showed that LC densities of VS was similar to that of NAS (p: not significant). No differences were noted in terms of age, sex, progressing, stable or repigmenting vitiligo. We concluded that involvement of LC in vitiligo, if any, does not probably occur via a degenerating mechanism, or via variations in regional densities.

Treatment of cutaneous lymphoma with etretinate.

Twelve patients with various types of lymphoma were treated with etretinate. The diagnosis included parapsoriasis en plaque, epidermotropic lymphoma (diffuse chronic erythroderma with atypical mononuclear cells, Sézary syndrome or MF tumours) and non-epidermotropic lymphoma. The patients received etretinate in a dose of 0.8 to 1.0 mg/kg/day for 2 to 14 months. No additional therapy was given. Patients with epidermotropic lymphomas stage I and II had a favourable clinical and histological response whereas those with deeply infiltrating tumours remained unresponsive. Patients with parapsoriasis en plaque and poikiloderma showed little response. Of the four patients who discontinued the treatment, three had recurrences after 3 to 4 months but one remained clear. The results obtained with etretinate may equal those obtained with more aggressive treatments.