[Inflammatory skin diseases and hypereosinophilia]. / Hautentzündungen und Hypereosinophilien.

Among the dermatoses associated with tissue eosinophilia are pemphigus vegetans, bullous pemphigoid, granuloma facial and Wells' syndrome. Eosinophilic spongiosis can occur in the early stage of pemphigus. Pemphigus herpetiformis, a recently introduced term, does not represent a well defined entity but develops into either pemphigus vulgaris or pemphigus foliaceus. Granuloma facial is basically vasculitis. Eosinophilic cellulitis or Wells' syndrome shows numerous eosinophils which degranulate and through confluence of their granules form the so-called flame figures.

Cell surface carbohydrates in proliferative epidermal lesions. II. Masking of peanut agglutinin (PNA) binding sites in solar keratoses, Bowen's disease, and squamous cell carcinoma by neuraminic acid.

Seventy-six skin biopsies of proliferative lesions were studied by using 4 different lectins and an avidin-biotin-peroxidase complex. In solar keratosis, Bowen's disease and squamous cell carcinoma, malignant-appearing keratinocytes exhibited loss of membrane staining with Concanavalia ensiformis agglutinin (Con A), but revealed cytoplasmic staining. When incubated with peanut agglutinin (PNA), the malignant keratinocytes did not stain. However, the PNA binding sites were not absent, but masked by sialic acid. Following cleavage of the sialic acid with neuraminidase, free PNA binding sites could be demonstrated in the plasma membranes. In contrast, the keratinocytes in keratoacanthomas showed membrane staining with Con A and also contained free PNA binding sites. These histochemical findings confirm and extend our earlier observations regarding cell surface carbohydrates in premalignant and malignant epidermal lesions.

Cell-surface carbohydrates in proliferative epidermal lesions. Distribution of A, B, and H blood group antigens in benign and malignant lesions.

The distribution of A, B, and H blood group antigens was studied by means of peroxidase-antiperoxidase technique in normal skin and in lesions of carcinomas in situ (solar keratoses, Bowen's disease), squamous cell carcinoma, keratoacanthomas, and verrucae. In normal skin, the epidermis of persons of blood group O showed H antigens throughout the epidermis; of blood group A, H and A antigens; and of blood group B, H and B antigens. In lesions of solar keratoses, there were no antigens of blood groups in the irregular downward proliferations. In five of 11 cases of Bowen's disease, there were no antigens of blood groups in the epidermis. In eight out of 10 cases of squamous cell carcinoma, no antigens of blood groups were found in the islands of the neoplastic process, but in two cases they were present in a patchy distribution. In the benign lesions examined, the antigens of A, B, and H blood groups were always present, although in verrucae the staining was confined to the upper layers of the epidermis only.

Cell surface carbohydrates in psoriasis. Defective cytoplasmic transport by glycoconjugates carrying fucose residues suggested by lectin staining.

Eleven biopsy specimens of normal skin and twenty-four biopsy specimens of psoriatic lesions were examined histochemically by using several lectins (Ulex europaeus, UEA-1; Dolichos biflorus, DBA; Bandeirea simplicifolia, BS-I; Concanvalia ensiformis, Con A; Triticum vulgaris, WGA; Ricinus communis, RCA; Arachis hypogoea, PNA) in order to evaluate the presence and distribution of various carbohydrates in normal and psoriatic keratinocytes. The findings revealed that keratinocytes from psoriatic lesions are distinguished by a different composition of carbohydrate residues incorporated in their plasma membranes. In particular, the intracellular transport of alpha-L-fucose, alpha-D-mannose, and alpha-D-glucose to the plasma cell membrane is impeded, whereas their synthesis in the cytoplasm of the psoriatic keratinocytes is largely unaltered. In addition, due to the lack of terminal alpha-L-fucose, the alpha-D-N-acetyl-galactosamine and alpha-D-galactose residues cannot be transferred to the plasma membranes and, therefore, the antigens for blood groups A and B remain incomplete in psoriatic epidermis. On the basis of these findings and in comparison with previous findings of our group on hyperproliferative, malignant keratinocytes, it is concluded that particularly the disordered cytoplasmic transport of alpha-L-fucose-carrying glycoconjugates may represent a specific defect in psoriasis, possibly linked with the pathogenesis of this disease.

Treatment of pemphigus vulgaris. Results obtained in 84 patients between 1961 and 1982.

A review of the treatment of 84 patients with pemphigus vulgaris between 1961 and 1982 showed that 47 of the 84 patients were free of lesions and not receiving treatment as of Jan 1, 1983. Twenty-two of the patients had been without lesions and treatment for more than five years. There had been no disease- or treatment-related fatalities since 1976. Two types of treatment have been used in recent years: in severe cases an initial course of large daily doses of prednisone (200 to 400 mg) was given for six to ten weeks. This was followed by combined treatment with small, alternate-day doses of prednisone plus a daily dose of an immunosuppressant agent, usually azathioprine. In mild, relatively stable cases the combined form of treatment was given from the beginning. The use of intermediate doses of prednisone, ie, doses between 40 mg every other day and 200 mg/day, is discouraged as potentially hazardous.

Distribution of carbohydrate residues in normal skin.

Sections of biopsies of normal skin obtained from 11 individuals were incubated with 8 lectins using an avidin-biotin complex (ABC). All sections when incubated with the appropriate lectin showed the presence of the following carbohydrate residues: L-fucose, beta-(1-4)-D-GlcNAc)2 (N-acetylglucosamine), acetylneuraminic acid, Gal-beta-(1-3)-GalNAc (N-acetyl-galactosamine), beta-D-galactose, alpha-D-glucose, and alpha-D-mannose. In addition, sections of individuals with blood group A showed alpha-D-GalNAc and sections of individuals with blood group B showed alpha-D-galactose. In the stratum (str.) basale, carbohydrates were present in small quantities, but as the cells matured and moved upward, the incorporation of carbohydrates into the cell membranes increased considerably. In the str. granulosum, lectin reactivity was absent in many sections, probably due to masking by phospholipids. The dark cells in the eccrine glands showed reactivity with all lectins except in the one nonsecretor with blood group A1, whose dark cells showed no L-fucose and alpha-D-GalNAc. The endothelial cells of the blood vessels showed lectin reactivity except when incubated with concanavalin A. The sebaceous glands showed both cytoplasmic and membrane staining when incubated with various lectins.

Squamous cell carcinoma arising in a linear epidermal nevus.

An isolated, pruritic lesion developed in the right breast of a 17-year-old girl during a period of four months. The lesion was well circumscribed, erythematous, indurated, and markedly hyperkeratotic in the center. The histologic diagnosis was well-differentiated squamous cell carcinoma arising in a linear epidermal nevus. The development of squamous cell carcinoma in a linear epidermal nevus is a rare event, previously reported only in long-standing, extensive lesions of middle-aged to elderly patients. This case seems to be unique in regard to the patient's young age, the short duration of the nevus, and the lack of identifiable predisposing factors.

Correlation of antibodies in skin and serum with disease severity in pemphigus.

Direct and indirect immunofluorescence (IF) testing was performed on 63 patients with active or inactive pemphigus in order to determine the reliability of these tests for diagnosis and for monitoring disease severity. Direct IF was positive in 58 of 63 patients with pemphigus. The five patients with negative direct IF had been free of lesions for more than a year. Thus, the direct IF test is a reliable diagnostic procedure that shows positive findings early in the disease. Indirect IF is inferior to direct IF as a diagnostic test because it may be negative in early cases, as observed in four patients. Furthermore, indirect IF, contrary to claims, is not reliable for evaluating the status of the disease. Thus, in patients with lesions and receiving treatment, 41% of the determinations showed a negative titer; whereas in patients free of lesions, 45% of the determinations showed a positive titer. In particular, seven patients without lesions and without treatment for more than a year had a positive titer.

Immunosuppressants and prednisone in pemphigus vulgaris: therapeutic results obtained in 63 patients between 1961 and 1975.

Evaluation of the treatment in 63 patients with pemphigus vulgaris showed that six patients (9.5%) died of the consequences of high-dosage prednisone treatment, while 24 patients (38.1%) were free of lesions and not receiving treatment. Immunosuppressants (methotrexate, cyclophosphamide, and azathioprine) are of value in patients with severe pemphigus vulgaris since they often reduce the maintenance dose of prednisone required after high-dosage prednisone treatment. Immunosuppressants are of even greater value in patients in the early, stable stage of pemphigus vulgaris. Of 16 such patients initially treated with a combination of an immunosuppressant and maintenance doses of prednisone not exceeding 40 mg on alternate days, 13 at no time required treatment with higher doses of prednisone. The good results obtained with this method emphasize the importance of diagnosing and treating pemphigus vulgaris in its early stage.

Localized mycosis fungoides with prominent epidermotropism: Woringer-Kolopp disease.

A patient had a single, large, sharply demarcated lesion on one leg. Under the mistaken diagnosis of malignant melanoma in situ, the lesion was excised and the area grafted in 1956. There has been no recurrence in the past 20 years. Histologic reevaluation led to a diagnosis of mycosis fungoides with pronounced epidermotropism of the cellular infiltrate. This case has close clinical and histologic resemblance with three cases published in Europe and regarded as a special form of reticulosis as first described by Woringer and Kolopp. Mycosis fungoides with marked epidermotropism is not always a benign localized disease but may be widespread and lead to death.