Mizuho Yamada

Subcorneal pustular dermatosis with vesiculo-bullous eruption. Demonstration of subcorneal IgA deposits and a leukocyte chemotactic factor

We report an unusual case of subcorneal pustular dermatosis with widespread vesiculobullous lesions. Reduction of the oral prednisone dosage provoked a severe exacerbation, with erythroderma associated with massive pustule formation. This was effectively controlled by topical PUVA. Subsequent recurrence of skin lesions responded well to oral etretinate.

Lipodystrophia centrifugalis abdominalis infantilis. A follow-up study

To determine the course and prognosis and also to reexamine the clinical and histologic features of lipodystrophia centrifugalis abdominalis infantilis, originally described by Imamura et al in 1971, a questionnaire was sent to 94 dermatology and pediatric departments in Japan. Data on 86 cases collected from 44 departments confirmed the unique and distinct features of this disease: onset by age 3, development of the lesion in the groin, axillae, or their surroundings, presence of slightly inflammatory areas around the depressed one, and histologically, loss of subcutaneous fat in the depressed area with panniculitis in the surrounding area. The depressed lesions were found to enlarge centrifugally for several years and to involve a large part of the abdominal and/or chest wall. However, the enlargement was found to stop spontaneously by age 13, and the depressed area tended to undergo recovery thereafter.

Psoriatic leukotactic factor. Further physicochemical characterization and effect on the epidermal cells

SummaryA leukocyte chemotactic factor with a molecular weight of around 12,500 daltons is found in large amounts in psoriatic scales. Except for complement-activated serum none of the extracts prepared from individual contaminants found in psoriatic scales showed an elution pattern similar to that of the psoriatic leukotactic factor (PLF) by Sephadex G-75 chromatography. Checkerboard experiments showed that PLF was highly chemotactic and mildly chemokinetic for polymorphonuclear leukocytes (PMNs). Purification of PLF yielded a cationic peptide fraction similar to that of anaphylatoxin. Intradermal injection of a potent PLF solution into guinea pigs produced degenerative changes in the epidermis within 24 h, particularly in the differentiated cells of the upper epidermis. It increased epidermopoiesis as assessed by incorporation of 3H-thymidine. Planimetric analysis of the epidermis revealed a marked increase in epidermal thickness after 5 days. Since PLF per se did not influence the proliferative capabilities of isolated basal cells or exert any damaging effect on differentiated epidermal cells in human epidermal cell culture, we speculated that PLF induced the advent of PMNs abound with hydrolytic enzymes and toxic substances, which were responsible for the damage of the upper epidermis resulting in the formation of parakeratosis and of acanthosis.ZusammenfassungEin leukozytenstimulierender chemotaktischer Faktor mit einem Molekulargewicht von annähernd 12500 Dalton kann reichlich aus Psoriasisschuppen isoliert werden. Mit Ausnahme von komplementaktiviertem Serum zeigte keiner der Extrakte aus Psoriasisschuppen bei der chromatographischen Analyse mit Sephadex G-75 ein dem psoriatischen leukotaktischen Faktor (PLF) ähnliches Elutionsmuster. Experimentell konnte gezeigt werden, daß der PLF stark chemotaktisch und mäßig chemokinetisch auf neutrophile Granulozyten wirkt. Eine weitere Reinigung des PLF erbrachte schließlich eine kationische Peptidfraktion ähnlich dem Anaphylatoxin.Bei intradermaler Injektion von PLF an Meerschweinchen zeigten sich innerhalb von 24 h degenerative epidermale Veränderungen, wobei besonders die differenzierten Zellen der oberen Epidermis betroffen waren. Darüber hinaus wurde die Epidermopoese, gemessen an der 3H-Thymidin-Markierung, beschleunigt. Durch Planimetrie konnte eine deutliche Verdickung der Epidermis nach 5 Tagen gezeitt werden. Da der PLF per se weder die Proliferation isolierter Basalzellen beeinflußte noch eine schädigende Wirkung auf Kulturen menschlicher Epidermiszellen ausübte, kann spekulativ vorgeschlagen werden, daß der PLF die Einwanderung von neutrophilen, mit hydrolytischen Enzymen und toxischen Substanzen beladenen Granulozyten bewirkt, wodurch die Schädigung der oberen Epidermis zustande kommt und daraus eine Parakeratose und akanthose resultiert.

S–100 protein-positive cells in hidrocystomas

The histogenesis of hidrocystomas was examined by the use of immunostaining for S–100 protein. In normal sweat glands, S–100 protein was found exclusively in the secretory cells of eccrine glands, whereas this protein was not present in the other parts of eccrine glands or at any levels of the structure of apocrine glands. On the bases of this immunostaining pattern in normal sweat glands, we attempted to correlate the origin of 8 cases of hidrocystoma to the presence of S–100 protein‐positive cells. S–100 protein was detected in the cells of one solitary eccrine hidrocystoma, but not in those of 2 cases of “classic”, multiple‐lesion type of eccrine hidrocystoma. This indicated that the former arose from the secretory portion of the eccrine gland and the latter from the eccrine ductal cells. Two of the 5 cases of apocrine hidrocystoma showed positive staining in a part of the lining cells of the cyst wall, while the other 3 cases were negative to this protein. This finding suggests that some of the tumors diagnosed morphologically as apocrine hidrocystoma differentiate in the direction of eccrine secretory cells. In addition to S–100 protein, we also surveyed for the presence of carcinoembryonic antigen (CEA), and all cases examined were consistently positive to this substance. The detection of S–100 protein was considered to be more helpful in classifying hidrocystomas than that of CEA.

Benign lymphoplasia of the earlobes induced by gold earrings: Immunohistologic study on the cellular infiltrates

We report three patients with peculiar nodules of the earlobes that developed a few months after the wearing of pierced-type gold earrings. Biopsy specimens showed dense infiltration of lymphoid cells in the dermis and subcutaneous tissue associated with the formation of lymphoid follicles. The clinical and histologic pictures were similar to those of lymphocytoma cutis. Immunohistologically, the nodular lesions were characterized by the proliferation of T cells, mainly suppressor/cytotoxic T cells, and the presence of histiocytic cells displaying Leu-3a antigens on the surface. Patch tests of aqueous gold compound induced a strong skin reaction. A histologic study of the patch test reaction sites revealed a picture of ordinary allergic contact dermatitis showing that T cells, including both helper and suppressor T cells, invaded the spongiotic epidermis and the papillary dermis. Eosinophils and a few Langerhans cells were also present in the dermal infiltrates. These findings suggest that pierced-type gold earrings induced a long-term dermal response that resulted in producing benign lymphoplasia in the sensitized individuals.

Can pemphigus vulgaris become pemphigus foliaceus

Among 31 patients with pemphigus, we observed two women with clinical and histologic features characteristic of pemphigus vulgaris that later became those of pemphigus foliaceus. Western blot study indicated that serum from one patient, when she had the cutaneous manifestations of pemphigus foliaceus, contained antibodies reactive with a desmosomal antigen, suggestive of desmoglein 1, the same as recognized by pemphigus foliaceus serum. The present study suggests that two distinct types of pemphigus can occur in the same person simultaneously or separately.

In vivo binding site of pemphigus vulgaris antibodies and their fate during acantholysis

Ultrastructural localization of pemphigus vulgaris antigen-antibody complexes and their fate during acantholysis were studied in epidermal sheets obtained from the area surrounding the bullae and in acantholytic cells in blister fluid. The distribution of pemphigus vulgaris antibodies already bound to the keratinocytes in early acantholytic lesions was detected with ferritin-conjugated goat antihuman IgG. Ferritin particles were observed on the surface of keratinocytes with particular affinity for desmosomal structures. The acantholytic cells in the blister fluid bound only a small number of ferritin particles on their surface. During incubation at 37 degrees C, pemphigus vulgaris antigen-antibody complexes on the surface of separated desmosomes were internalized and recognized in cytoplasmic vesicles. Endocytosis of separated desmosomes also was observed in vivo when freshly obtained epidermal sheets were immediately processed for routine electron microscopic study. These findings suggest that pemphigus vulgaris antibodies are densely located on desmosomes and that the antigen-antibody complexes, together with other serum proteins on the keratinocyte surface, are internalized by a process of endocytosis.

Pemphigus foliaceus, myasthenia gravis, thymoma and red cell aplasia. A case report and indirect immunofluorescence study on 38 patients with myasthenia gravis.

A 68‐year‐old man developed pemphigus foliaceus, myasthenia gravis with a spindle cell thymoma, and later died with red cell aplasia. At autopsy, pemphigus affected the oesophageal mucosa, and this finding was confirmed by direct immunofluorescence.

Analysis of transepidermal leukocyte chemotaxis in experimental dermatophytosis in guinea pigs

SummaryIn dermatophytosis, there is exocytosis of polymorphonuclear leukocytes (PMNs) toward the fungus-laden horny layer. To analyze this mechanism, we studied in vitro leukotactic properties of epidermal extracts prepared from lesions of experimental Trichophyton mentagrophytes infection in guinea pigs as well as fungus-derived chemotactic factors. Trichophyton mentagrophytes was found to release low-molecular-weight PMN chemotactic factors with its growth, like other microorganisms. Furthermore, it activated complement via the alternative pathway to produce complement-derived chemotactic factors in vitro. The epidermal extracts prepared from weakly inflammatory lesions of an early stage of T. mentagrophytes infection showed mild PMN chemotactic activity, which, as in those from irritated skin, was mostly due to the presence of low-molecular-weight chemotactic factors. After 8 days of infection, when prominent PMN migration took place together with the development of immune reactivity to fungus antigens, the epidermal extracts revealed strong leukotactic activity that showed a triphasic pattern by Sephadex G-75 chromatography similar to that observed in complement-activated serum. Since we could not demonstrate any deposition of immunoglobulins and complement on the fungal elements present in the horny layer, actual complement activation in vivo seems to occur after interaction of serum with soluble fungal components in the epidermis through both classic and alternative pathways. We think that the transepidermal migration of PMNs in dermatophytosis, together with contact sensitivity to fungal antigens, is responsible for induction of increased epidermopoiesis with resultant desquamation to eliminate the fungus-laden horny layer.

The speckled epidermal nuclear immunofluorescence of mixed connective tissue disease seems to develop as an in vitro phenomenon

The pathogenesis of speckled epidermal nuclear immunofluorescence in patients with mixed connective tissue disease (MCTD) was studied by reproducing this reaction in guinea‐pigs, using serum samples containing high litre antibody to ribonucleoprotein (RNP). Immunofluorescence studies on specimens obtained from guinea‐pig skin into which scrum samples containing high titre RNP antibody had been injected intradermally, revealed positive epidermal nuclear staining for IgG. This speckled immunofluorescence was demonstrable immediately after injection and remained so for 24 or 48 h. The pattern of fluorescence was similar in all cases, and there was no penetration of RNP antibody through the cell membrane. The epidermal nudear fluorescence was not detected with sera at a dilution of 1:100 or more. These results provide strong evidence that the epidermal nuclear immunofluorescence observed in patients with high titre antibody to RNP develops as an in vitro phenomenon.