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.

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.

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.

[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.

Factor XIIIa expression in juvenile xanthogranuloma.

Dermal dendrocytes constitute the largest population among cells of dermatofibromas. In other histiocytic tumours, the exact nature of histiocytic cells is not known. We have searched for the presence of dermal dendrocytes in juvenile xanthogranulomas. The immunohistochemical study was performed on 9 juvenile xanthogranulomas. We used monoclonal or polyclonal antibodies: anti-XIIIa, HAM56, anti-S100, anti-NSE, anti-HLA-DR, anti-CD68 and anti-lysozyme. Phagocytic mononuclear cells (histiocytes, giant cells, Touton cells) did not express Langerhans' cell markers (S100, NSE ou HLA-DR). They weakly expressed markers of macrophages (CD68, lysozyme). There was a very strong binding by HAM56 and anti-XIIIa. This expression was more evident on xanthomatous and newly appeared tumours than on fibrous tumours. The largest population of juvenile xanthogranuloma cells appeared to be constituted by dermal dendrocytes. These cells are perhaps the key-cells of a continuum of benign tumours, from juvenile xanthogranuloma to dermatofibroma, with different stages corresponding to different proportions of dendrocytes, lymphocytes and fibroblasts.

Multiple juvenile xanthogranulomas without foam cells and giant cells.

A 4-month-old boy had numerous nodular lesions. Microscopic findings revealed a histiocytic proliferation without foam cells and giant cells. Immunohistochemical studies showed that the histiocytes belonged to the monocyte-macrophage system and not to the Langerhans cell system. This case is an unusual variant of juvenile xanthogranuloma that, when congenital, may be difficult to differentiate from Hashimoto-Pritzker disease.

[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.

[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.

Serpentine supravenous hyperpigmentation induced by the nitrosourea fotemustine.

Two cases of serpentine supravenous hyperpigmentation developing in the area of fotemustine infusions are reported. Histological features showed an increased melanin synthesis and the presence of melanophages without focal degeneration of basal cells or dermal inflammatory infiltrate. Perls' strain was negative. Hypotheses concerning the mechanisms of increased melanin synthesis over the veins are discussed.

Extraocular sebaceous carcinoma.

A 79-year-old man with no history of previous irradiation presented with a large ulcerated tumour of the cheek. The histological features favoured a true sebaceous carcinoma. Neither squamous nor basal differentiation were seen. Sudan stainings were positive. Tumour cells expressed suprabasal keratins and were negative for carcinoembryonic antigen, vimentin and S 100 protein. Extraocular sebaceous carcinomas occurring without previous irradiation are rare tumours which behave aggressively. Treatment regimens are discussed.

Detection of undegraded fibrin and tumor necrosis factor-alpha in venous leg ulcers.

The pathogenesis of venous leg ulcers is based on the leakage of fibrinogen leading to pericapillary fibrin cuff and plugging of capillaries by white blood cells. Eight patients with venous leg ulcers have been studied with a panel of antibodies reactive for fibrinogen, fibrin, fibrin degradation products, and various cell-associated markers for polymorphonuclear cells, monocytes, and B and T lymphocytes. Our results showed that pericapillary fibrin cuff was mainly composed of undegraded fibrin and that, in the granulation tissue, tumor necrosis factor-alpha and elastase activities were detectable in monocytes and polymorphonuclear cells, respectively. Only few activated lymphocytes were present. On the basis of these results, it is assumed that inflammation generated by activated white blood cells that accumulate under unrelieved pressure is the key event. Tumor necrosis factor-alpha synthesized by activated monocytes may therefore induce the formation of pericapillary fibrin cuffs. Pericapillary fibrin cuffs and toxic metabolites released by polymorphonuclear cells may explain the absence of wound repair.

Epidermal and urethroid penile cyst.

The authors describe a 74-year-old man who presented with a 2-cm nodule on the ventral face of the penis, showing histologically a cyst lined by both epidermal and urethroid epithelium. The authors discuss the various histological forms of raphe median cysts of the penis.

Hyperpigmentation induced by UVB at the application site of estradiol.

We report a case of a 48-year-old woman who presented with chronic essential pruritus and was successfully treated with the transdermal estradiol system because of menopausal discomfort. Suberythemogenic UVB phototherapy was proposed to the patient in order to control her pruritus. She developed hyperpigmentation at the application site of estradiol. To the best of our knowledge, this is the first report suggesting a direct relationship between estrogen and melanin synthesis in humans.