Varicella in patients infected with the human immunodeficiency virus.

In a retrospective study of 421 patients infected with human immunodeficiency virus, 15 (3.5%) had varicella. Twelve patients had a typical varicella. Complications were as follows: profuse eruption, 6; hemorrhagic eruption, 1; hepatitis, 5; and pulmonary involvement, 1; 1 patient developed an intravascular disseminated coagulation and died of varicella. Three patients with acquired immunodeficiency syndrome, having a history of varicella, presented with an atypical form of varicella with a small number of disseminated cutaneous poxlike lesions; 1 of these patients experienced three relapses of atypical varicella. Assay of serum antibodies to varicella zoster virus showed that, while typical varicella was the primary varicella zoster virus infection, atypical varicella was a reactivation of varicella zoster virus infection. Acyclovir was given to 11 patients and vidarabine to 1 patient. The one patient who died and the one who suffered a relapse had received acyclovir. Thus, varicella in patients infected with human immunodeficiency virus may be complicated and even lethal. Atypical forms of varicella could be, as is the case with herpes zoster, a reactivation of endogenous varicella zoster virus.

[Multinucleated cell angiohistiocytoma. Report of 6 cases]. / Angiohistiocytome à cellules multinucléées. A propos de six cas.

Multinucleate cell angiohistiocytoma is a recently described entity. It is a benign vascular proliferation. Clinically, it is characterized by violaceous red papules, often mimicking Kaposi's sarcoma. Acral sites and face were the commonest sites. The six patient's age was between 41 and 64 years and sex ratio was equal. Microscopic features were an increased number of blood vessels together with mononucleated and multinucleated histiocyte-like cells with scalloped borders. Staining of mononucleated cells with CD68, anti vimentin and anti factor XIIIa antibodies emphasized a fibrohistiocytic origin. Loss of factor XIIIa expression in multinucleate cells gets clue to think that these cells are dedifferenciated.

[Nevus of Ota associated with chronic glaucoma]. / Naevus de Ota associé à un glaucome chronique.

Oculodermal melanocytosis (nevus of Ota) is an abnormally large accumulation of pigment in ocular tissues as well as the skin in the distribution of the trigeminal nerve. Elevated intraocular pressure is a serious complication of this disease, as well as transformation to malignant melanoma. We report a case of nevus of Ota, documented clinically and histopathologically, associated with severe chronic glaucoma.

[Nodular and granulomatous form of periarteritis nodosa caused by the hepatitis B virus]. / Forme cutanée nodulaire et granulomateuse d'une périartérite noueuse liée au virus de l'hépatite B.

Polyarteritis nodosa (PAN) is, in rare cases, associated with subcutaneous nodules and pathology does not usually show the presence of peri and extravascular granulomas. When present in patients with hepatitis B virus (HBV) related PAN these facts demonstrate that classification of PAN is not homogeneous. CASE REPORT. A patient infected by HBV developed a PAN demonstrated by clinical symptoms and pathology. The disease was characterized by the presence of subcutaneous nodules and histologically by peri- and extra-vascular granuloma which surrounded necrosis of medium-sized vessels. Outcome was also unusual in the patient who did not respond to the association of plasma exchanges and antiviral agents and was only slightly improved by steroids and cyclophosphamide. COMMENTS. HBV-related PAN is considered to be an immune complex disorder. In the present case report granuloma were present as observed in Churg Strauss syndrome or Wegener's granulomatosis which are the consequence of other pathogenetic mechanisms as anticytoplasmic neutrophil antibodies (ANCA). This case reports underlines the heterogeneity of the PAN group of vasculitis and the probable role for various pathogenetic mechanisms.

[Ophthalmologic manifestations of leukocytoclastic vasculitis. Apropos of 3 case reports]. / Manifestations ophtalmologiques des vascularites leucocytoclasiques. A propos de 3 observations.

Ophthalmological lesions enter in the definition of Cogan's syndrome, associated with vestibulo-auditory symptoms. They are present in almost one half of patients with Wegener's granulomatosis and in 10 to 20 p. 100 of patients with periarteritis nodosa, where they may be the initial symptom. Yet they are seldom described in leucocytoclastic angiitis of small vessels, 3 cases of which are reported here. The first case concerns a 71-year old woman with cutaneous leucocytoclastic angiitis which regressed within a few days under dapsone. However, the drug had to be withdrawn on account of acute haemolysis. Thereafter, new skin lesions and severe pain in the left eye with fall in visual acuity developed simultaneously. Eye examination showed a large, marginal ulceration of the cornea extending to the adjacent sclera and reflecting deep involvement of the sclerocorneal limbus. Systemic corticosteroid therapy in doses of 1 mg/kg/day was instituted, resulting in gradual disappearance of the skin lesions and stabilization of the ophthalmic lesion. There remains, however, thinning of the cornea and sclera which entails a definite risk of perforation should the angiitis recur. The second patient was a 54-year old man seen for fever, polyarthralgia, purpura of the lower limbs and rapidly extending necrotic ulceration of the scrotum. A few days previously, inflammatory chemosis of the right eye had suddenly developed. Under systemic corticosteroids (1.5 mg/kg/day), the ophthalmic symptoms rapidly regressed, but the patient died of digestive tract haemorrhage. The third case was that of a 36-year old woman with urticaria associated with arthralgias and diffuse myalgia.(ABSTRACT TRUNCATED AT 250 WORDS)

[Intra-epidermal pustulosis in a child. Demonstration of a target antigen similar to foliaceus pemphigus antigen]. / Pustulose intraépidermique à IgA chez un enfant: Mise en évidence d'un antigène cible identique à celui du pemphigus superficiel.

INTRODUCTION: Intraepidermal IgA pustulosis is a vesiculopustular dermatosis defined by pemphigus type intercellular deposit exclusively of IgA. It is a member of the pemphigus group and may be related to neurtrophilic dermatoses. CASE REPORT: A child had vesiculopustular lesions of the limbs since the age of 11 years. Biopsy showed the subcorneal intraepidermal nature of the pustules and exclusive IgA deposit throughout the epiderma. Indirect immunofluorescence and protein immunoelectrophoreses were negative. Immunotransfer to beef tongue extract evidenced a 150-160 kDa band corresponding to IgA and IgG desmoglein. Treatment with general corticosteroids followed by pristinamycin was successful. DISCUSSION: This is the first case report showing evidence of antiepidermal antibodies directed against the superficial pemphigus antigen.

[Cutaneous localizations of disseminated Histoplasma capsulatum histoplasmosis in a case of acquired immunodeficiency]. / Localisations cutanées d'une histoplasmose disséminée à Histoplasma capsulatum au cours d'un cas d'immunodéficience acquise.

A widespread maculo-papular cutaneous rash appeared on a HIV-positive young bisexual Cambodian man. He was treated for Mycobacterium tuberculosis and Pneumocystis carinii infections. He had been residing in France for seven years. Histology showed, within the dermis, abundant extracellular and intramacrophagic yeast-like organisms suggestive of histoplasmosis. Cultured specimens produced a growth of colonies after three weeks on Sabouraud 4 p. 100 dextrose agar at 25 degrees C. Numerous macroconidia were found which made the species diagnosis of Histoplasma capsulatum possible. Despite initiation of therapy with amphotericin B the patient died. Cutaneous involvement with or without specific features is uncommon in disseminated histoplasmosis. The specific cutaneous lesions are protean. They rarely are the presenting sign of initial infection. Disseminated histoplasmosis has a poor prognosis in acquired immunodeficiency syndrome: amphotericin B is not curative. Maintenance suppressive therapy with ketoconazole has been recommended following amphotericin B completion, although break-through has been reported.

Urticarial vasculitis associated with a monoclonal IgM gammopathy: Schnitzler's syndrome.

We describe two cases of Schnitzler's syndrome presenting with urticarial vasculitis, in which there was a monoclonal IgM of kappa light-chain isotype. Skin histology showed a perivascular leucocytic infiltrate and leucocytoclasis. One patient had an abnormal complement profile and positive rheumatoid factor while the other had antibodies to unidentified soluble nuclear antigens, anti-cytoplasmic antibodies and circulating immune complexes. In addition, in the first case deposits of IgM, C3 and, to a lesser degree, of IgG were observed in the walls of small blood vessels.

Cutaneous manifestations of Takayasu arteritis. A retrospective study of 80 cases.

The aim of this retrospective study is to delineate in Europe the frequency and type of cutaneous manifestations associated with Takayasu arteritis (TA). Eighty patients with TA were analyzed. Symptoms suggestive of Raynaud's syndrome were noted in 11 patients (14%) and could be directly related to large vessel involvement. Other skin lesions were observed in 10 patients (12.5%). Five had acute tender erythematous nodules on the legs with a clinical diagnosis of erythema nodosum; 2 had subacute ulcerated nodules of the legs; 1 had pyoderma-gangrenosum-like ulcerations of the four limbs which resulted from the breakdown of subcutaneous nodules; 1 had lupus-like malar flush, and the last one had urticarial lesions with livedo reticularis. Skin samples were obtained from 4 patients. Three of them agreed that reiterated biopsies be done on recurrent lesions. A granulomatous vasculitis was observed in 2 cases involving hypodermal arterioles in one case and veins in the other. The other pathological findings were septal and lobular panniculitis which can be associated with granulomatous vasculitis. Different histological findings on reiterated biopsies were frequently found. The absence of any other etiology and chronological arguments suggested a relationship between these skin lesions and TA. Tuberculosis was probable in 1 case but apparently was not related to the skin lesions.

Subcutaneous eosinophilic necrosis associated with refractory anemia with an excess of myeloblasts.

We followed up over a period of 10 months a Cambodian patient in whom refractory anemia with excess blasts was discovered after the onset of fever and chronic dermatologic involvement. Violaceous, firm, and painful subcutaneous nodules (1-3 cm in diameter) were present on the arms, legs, trunk, scalp, neck, and chin and were associated with violaceous infiltrating plaques on the face and forehead. The microscopic examination of repeated biopsy specimens showed a predominantly lobular panniculitis characterized by an extensive eosinophilic necrosis, leukocytoclasia, and fibrinoid deposits within a few vessels. Such lesions might be the consequence of the immune response against leukemic clones, which have been shown to be present in a steady state in at least some cases involving myelodysplastic syndromes.

Lichen planus pemphigoides: an immuno-electron microscopic study.

We report a case of lichen planus pemphigoides. Immuno-electron localization of G-immunoglobulin and C3 showed that in the peribullous zone these were in the lamina lucida, as in typical bullous pemphigoid, but in the bulla itself they were along the floor, and not along the roof as in bullous pemphigoid. We conclude that bullous pemphigoid antigen and lichen planus pemphigoides antigen have different localizations.