Identification of a 168-kDa mucosal antigen in a subset of patients with cicatricial pemphigoid.

This study describes the presence of antibodies in sera from patients with cicatricial pemphigoid specific for a 168-kDa antigen expressed by buccal mucosa. Six cicatricial pemphigoid sera unreactive, with epidermal or dermal proteins in immunoblot assay were tested on mucosal protein extracts. Four of these sera labeled a mucosal 168-kDa antigen (M168) under reducing conditions. An additional cicatricial pemphigoid serum with circulating antibodies to 180-kDa bullous pemphigoid antigen (BPAg2) also labeled M168. None of these cicatricial pemphigoid sera reacted with the alpha, beta, or gamma subunits of laminin-5. Nitrocellulose elution studies showed that the M168 antigen is a basement membrane antigen and labeled the epidermal side of salt-split skin. Immunoaffinity-purified anti-M168 antibodies did not bind to the 230-kDa bullous pemphigoid antigen (BPAg1) or to the 180-kDa BPAg2. None of the control sera from healthy individuals or from bullous pemphigoid, pemphigus vulgaris, or pemphigus foliaceus patients reacted with Ml68. This study demonstrates the specificity of some cicatricial pemphigoid sera against a 168-kDa antigen that is different from the laminin-5 subunits and shares no epitopes with the antigens of bullous pemphigoid (BPAg1, BPAg2) or the epidermolysis bullosa acquisita.

PUVA treatment of alopecia areata.

Twenty-three patients with alopecia areata were treated with photochemotherapy combining oral or topical methoxsalen and UV-A irradiation of the scalp or of the whole body. Eleven of 17 patients with multiple plaques of alopecia areata, alopecia totalis, and alopecia universalis, who were treated with oral methoxsalen and total body irradiation, had complete or more than 90% hair regrowth. Three patients had a relapse. The mean energy required was 505 joules/sq cm. In six cases, topical applications of methoxsalen or oral methoxsalen combined with local irradiation of the scalp were treatment failures. In the patients responding to treatment, the result did not seem to depend on the age of onset or the extent or duration of disease. However, patients with long-lasting alopecia had a higher risk of recurrence notwithstanding a good initial regrowth of hair. Few side effects of psoralens and UV-A (PUVA) treatment were noted. The mean follow-up period was 18.6 months after the completion of treatment. We discuss the possible mechanisms of action of PUVA in the treatment of alopecia areata.

Diagnostic value of indirect immunofluorescence on sodium chloride-split skin in differential diagnosis of subepidermal autoimmune bullous dermatoses.

OBJECTIVE: To determine the diagnostic value of indirect immunofluorescence on sodium chloride-split skin (SSS) in differentiating the pemphigoid group of subepidermal autoimmune bullous dermatoses, including bullous pemphigoid (BP), cicatricial pemphigoid, and pemphigoid gestationis, from epidermolysis bullosa acquisita (EBA). DESIGN: Serum samples were tested using immunofluorescence on SSS and immunoblot assay on epidermal and dermal extracts, a recombinant protein corresponding to the C-terminal end of the 230-kd BP antigen, and purified laminin-5. SETTING: An immunodermatology laboratory. PATIENTS: One hundred forty-two serum samples from patients with BP (n = 98), cicatricial pemphigoid (n = 23), pemphigoid gestationis (n = 10), EBA (n = 10), and anti-type IV collagen (n = 1). MAIN OUTCOME MEASURES: Binding sites of serum to the epidermal and/or dermal sides of SSS were correlated with their antigenic specificities. RESULTS: Epidermal staining on SSS was highly specific for pemphigoid. Alternatively, a poor correlation was found for the dermal-reacting serum samples and the diagnosis of EBA; of the 19 serum samples with dermal staining on SSS, only 10 reacted with the EBA antigen. The remaining serum samples were from patients with cicatricial pemphigoid having antibodies to the alpha 3 or beta 3 chains of laminin-5 (n = 5) or patients with BP having antibodies to the 180-kd BP antigen (n = 2). One sample recognized exclusively a 185-kd dermal antigen corresponding to type IV collagen. One more BP serum sample with dermal staining did not recognize any dermal or epidermal antigen. CONCLUSION: In case of immunofluorescent dermal staining, the precise diagnosis should be confirmed by identification of the involved antigen, since it may reveal antibodies to laminin-5 or type XVII or IV collagen, in addition to the EBA antigen.

Café au lait spots in ataxia-telangiectasia (A.T.). Histochemical and ultrastructural study in one case.

Café au lait spots appear to represent one of the cutaneous features of Ataxia-telangiectasia (A.T.). At the cellular level, they are characterized by an epidermal hypermelanosis with a normal number of melanocytes. At the subcellular level, two basic abnormalities are observed: (1) an increase in the synthesis of melanosomes; (2) a modification of their distribution within the epidermal keratinocytes. Numerous pendulous melanocytes project into the upper dermis. Altered melanocytes are also observed. These show mitochondrial dilatation, melanosomal autophagic vacuoles, as well as lipid droplets. These abnormalities are not observed in the normal surrounding skin. The significance of these observations is discussed and stress is placed on the difficulty in establishing a differential diagnosis of café au lait spots, based on their histoenzymological and ultrastructural features.

Epidermodysplasia verruciformis induced by a new human papillomavirus (HPV-8). Report of a case without immune dysfunction. Effect of treatment with an aromatic retinoid.

A new case of epidermodysplasia verruciformis (EV) is reported in a 35-year-old man characterized by multiple common warts, flat warts, psoriasis-form lesions, and pityriasis-versicolor-(PV) like lesions. There was no familial history, no mental retardation, and no malignant changes. Human papilloma virus type 8 was identified in PV-like lesions. Immunologic studies detected no abnormalities: Delayed skin tests, DNCB sensitization, levels of immunoglobulins, complement components, circulating immune complexes, number of circulating lymphocytes, lymphocyte membrane markers, proliferative responses to mitogens, Ts activity, and Ts subpopulations as detected by monoclonal antibodies were considered to be within normal limits. A treatment with a retinoid aromatic (RO 10-9359) improved the clinical status of the patient and did not modify the immune parameters.

Treatment of cutaneous T-cell lymphoma with a new aromatic retinoid (Ro 10-9359).

A 77-year-old woman developed a diffuse nodular eruption with histologic, ultrastructural, and biologic evidence of cutaneous T-cell lymphoma (CTCL) limited to the skin. She was treated with a new aromatic retinoid Ro 10-9359 (1 mg/kg/day). After 34 days, the lesions flattened completely and the mononuclear cell infiltrate decreased significantly. No clinical recurrences occurred after a 4-month survey. Discontinuation of the aromatic retinoid led to a relapse with identical clinical and histologic features.

Detection of herpes simplex virus DNA in a cutaneous squamous cell carcinoma by in situ hybridization.

A case of a cutaneous squamous cell carcinoma (SCC) occurring at the site of a long-standing recurrent HSV infection is described. No deficit of the cell-mediated immunity was recorded. HSV2 was isolated in several viral cultures. HSV DNA was visualized in the SCC by in situ hybridization with biotinylated probes in paraffin-embedded tissue. The samples were negative for HPV2, 5, 16, and 18 probes. A causal relationship between HSV infection and cutaneous SCC is hypothesized.

Diagnosis of cutaneous vasculitis.

Vasculitis represent a broad spectrum of disorders involving vessels of different types and characterized by various clinical manifestations. The diagnosis of cutaneous vasculitis is usually easy and based on clinical and histological features. A number of classification systems of vasculitis have been proposed. In this review we define small vessel necrotizing vasculitis, large vessel necrotizing vasculitis, overlap syndromes and miscellaneous vasculitis. This classification for diagnostic purposes is more than an academic interest because prognostic and therapeutic differences can be demonstrated among these various groups of vasculitis.

Immunocytological characterization of the mycosis fungoides tumour cell.

The immunocytological identification of the mycosis fungoides cell was carried out on cells extracted from the tumorous nodules of a patient suffering from typical mycosis fungoides. Various techniques, such as E and IgM-EAC rosettes and examination for surface membrane immunoglobulins, were performed on the peripheral blood cells and the tumour cell. Membrane staining with a specific anti-T lymphocyte serum conjugated with peroxidase confirmed the thymodependent origin of the mycosis fungoides cell. However, the immunolabelling (with Fab peroxidase conjugate was found constantly negative.

[Immunotherapy of multiple recurring warts. II. Reassessment of the use of squaric acid dibutylester (SADBE) (author's transl)]. / Traitement des verrues multiples et récidivantes par induction d'une hypersensibilité retardée. II. Etude critique de l'utilisation du dibutylester de l'acide squarique.

The treatment of recalcitrant multiple verrucae by sensitization to DNCB has been successfully used as shown in a previous study. Owing to the mutagenic effect of DNCB in Ames test, we tried a new non-mutagenic compound, SADBE to treat patients with multiple recurring warts. The study comprised 18 patients whose informed consent was obtained. They were sensitized to SADBE with a 2 p. 100 solution applied once and then treated with a 0.1 p. 100 solution weekly. The solution was applied by means of a swab on the warts. The concentration was adjusted to the patient's reactivity in order to maintain a mild contact dermatitis. The duration of treatment ranged from 4 to 13 weeks. Our results showed tat complete resolution of warts occurred in only 2 out of 18 patients. Side effects were negligible. In conclusion, we do not support the use of SADBE for treating multiple recurring warts.

[Squamous cell carcinoma and recessive dystrophic epidermolysis bullosa]. / Carcinome spino-cellulaire et épidermolyse bulleuse récessive dystrophique.

We report a case of spindle cell carcinoma developed in a patient with recessive dystrophic epidermolysis bullosa. This association is classical but rare since only 46 cases have been found in the literature. The various possible treatments are discussed.

[Prospective study of factors associated with leukocytoclastic vasculitis]. / Etude prospective de facteurs associés aux vascularites leucocytoclasiques.

The origin of leukocytoclastic vasculitis (LV) being often difficult to determine, we have undertaken since 1980 a prospective study of factors associated with LV. We selected 53 patients whose LV was clinically predominant, and excluded patients in whom LV was an expected phenomenon in a known autoimmune or infectious disease. Twenty-eight of the 53 patients presented with a typical Gougerot-Ruiter disease, 15 with a bullous or necrotic form of the disease and 10 with urticarial lesions. Detail of the prospective laboratory tests performed is given in table I. Correlations between laboratory values and LV-associated factors were significant with the decrease of complement but not with the presence of circulating immune complexes, rheumatoid factor, cryoglobulin or direct immunofluorescence test positivity. Most of the associated factors in our series were infectious agents (streptococci, hepatitis virus), immunological agents (rheumatoid factor, cryoglobulin) or drugs known to be potential LV-inductors; other factors were less common or quite recently described (enterovirus, Yersiniae, cirrhosis, primary liver cancer, Chlamydiae, refractory anemia with an excess of myeloblasts. We do not feel that a large series of laboratory tests should be performed in every case of LV. The clinical context and simple laboratory tests, such as blood cell count, complement assay, plasma electrophoresis and a search for rheumatoid factor should be enough to guide the clinician and help him decide whether further investigations are needed. However, it should be noted that in some cases without clinical pointers only full virological evaluation enabled us to determine that enteroviruses may be involved in the pathogenesis of LV.

[Pyoderma gangrenosum associated with seronegative polyarthritis. Report of a case. Review of the literature (author's transl)]. / Pyoderma gangrenosum associé a une polyarthrite séro-négative. A propos d'un cas. Revue de la littérature.

Pyoderma gangrenosum developed in a 45-year-old caucasian woman who had an 18 year past history of seronegative polyarthritis without any visceral or haematologic associated abnormalities. A literature review revealed only 12 reported cases. Extensive laboratory evaluation failed to detect alterations of the immune functions. The presence of a vasculitis with C3 deposits suggested that levamisole may have played a role in eliciting the lesion. Clofazimine, administered for a period of one month, was a relative failure.

[Immunotherapy of multiple recurring warts. Reassessment of the use of dinitrochlorobenzene (author's transl)]. / Traitement des verrues multiples et récidivantes par induction d'une hypersensibilité retardée. Etude critique de l'utilisation du dinitrochlorobenzène (DNCB).

The treatment of recalcitrant multiple verrucae vulgaris remains puzzling. Recent studies have demonstrated the relationship between the DNCB-induced cell mediated immunity and the resolution of warts. Our study consisted of 27 patients. Among them, 22 were sensitized to DNCB. A complete resolution of warts occurred in 81 p. 100 with minor side effects (pruritus, oedema and occasional bullae). However, the degradation of DNCB in dinitrophenol compounds in vivo, may be considered as a main drawback of this method. Since there is little information concerning the mutagenicity and toxicity of DNCB as yet, we suggest to prefer the use of other chemical compounds, such as dibutyl ester squarate, that act the same way as the DNCB does.

[Behavior on de-epidermized human skin of unspecified reconstituted collagen tissue]. / Comportement sur peau humaine désépidermisée d'un tissu collagène reconstitué déspécifié.

The aim of our study was to assess the behaviour of a despecified collagen tissue laid on desepidermized human skin (taking area of skin graft). The collagen tissue was prepared according to Bell's method. The collagen was latticed, contracted by fibroblasts, chemically treated (formaldehyde or glutaraldehyde) to stabilize the fibrils and then despecified by cialit treatment. This tissue laid on superficial, non infected wounds very rapidly took a necrotic appearance and was totally lysed after 10 to 12 days without any modification of the healing course. No bacterial infection was observed. Histological and ultrastructural studies showed desorganization of collagen fibre bundles and tissue invasion by inflammatory cells. Circulating antibodies to collagen were absent at day 30. This model lacks interest as a substitute for superficial tissue replacement, but a healing function could be assigned to its high chemotactic power for polynuclears and macrophages and should allow its use in cases of deep tissue loss.

[Facial hemiatrophy, homolateral cervical linear scleroderma and thyroid disease]. / Hémiatrophie faciale, sclérodermie en bande cervicale homolatérale et thyréopathie.

A case of facial hemiatrophy and homolateral cervical band of scleroderma, complicated by hypothyroidism is reported. This case raises two problems: one is the problem of distinction between Romberg's disease and facial hemiatrophy due to a genuine localized scleroderma; the other concerns the relationship between localized scleroderma and dysthyroidism. The generalized scleroderma-dysthyroidism association has now been recognized, but the coexistence of thyroid disease and localized scleroderma has not yet been reported. Several pathogenetic hypotheses on this association are discussed.

[Adult papular acrodermatitis (Gianotti's disease). Report of 3 cases]. / Acrodermatite papuleuse de l'adulte. A propos de 3 cas

The authors report three cases of adult papular acrodermatitis with circulating HBs antigen. The eruption was followed by a benign icteric hepatitis which lasted from 30 to 45 days. In two cases, HBs antigen disappeared in a one month period, in one case the antigen has been present for more than three months. Direct immunofluorescence staining exhibits c3 deposits in the vessels of the dermal lesions, without any deposition of immunoglobulins or fibrinogen. We were unable to demonstrate the presence of HBs and " e " antigens in the skin lesions (using FITC conjugated specific antiserums). Serum protein concentrations of complement components C1q, C4,C3, C3PA were normal as measured by radial immunodiffusion. The percentage of circulating B and T cells was normal, as essayed by E-RFC, EAC-RFC and sIg. Thus, adult papular acrodermatitis, as well as the infantile form, does represent a sign of invasion of a benign viral hepatitis.

[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)

[Bullous manifestations of Kaposi disease associated with AIDS]. / Manifestations bulleuses d'une maladie de Kaposi associee au SIDA.

INTRODUCTION: We report the apparently first description of bullous Kaposi's sarcoma in a patient with AIDS. CASE REPORT: Kaposi's sarcoma was observed in a HIV positive homosexual who had developed AIDS. The delay between the development of Kaposi's sarcoma and the appearance of bullae was 6 months. Bullae occurred after oedema and necrosis of the subepidermic derma. DISCUSSION: This unusual clinical presentation of Kaposi's syndrome in an AIDS patient led to rapid tumor extention and would thus suggest a rapidly unfavourable prognosis.

[Thymoma and lupus: apropos of a case with recurrences and histological dedifferentiation]. / Thymome et lupus: à propos d'un cas avec rechutes et dédifférenciation histologique.

A case of thymoma associated with chronic lupus erythematosus is reported. The course of the lupus was parallel to that of the thymoma, with several successive relapses. Histologically, the thymoma became increasingly undifferentiated. Chemotherapy only resulted in a few months of remission.