Effect of neutrophil-derived eosinophil chemotactic factor (ECF) in human and guinea pig skin.

The in vivo effectiveness of eosinophil chemotactic factor (ECF), secreted in vitro by human neutrophils (PMN) during phagocytosis, is tested in 2 model systems. Injection of ECF into guinea pig ears causes a preferential attraction of eosinophils with time that is more marked in animals with preexisting eosinophilia. In the same model system, activated serum attracts fewer eosinophils and more PMN. In human skin windows, ECF and activated serum are equally effective on a quantitative basis, but ECF is more selective for eosinophils in patients with eosinophilia. With normal controls, ECF and activated serum attract PMN equally well. The studies confirm previous in vitro observations on the properties of ECF and suggest a potentially significant role of this factor during pathological processes.

Genetic linkage of the keratin type II gene cluster with ichthyosis bullosa of Siemens and with autosomal dominant ichthyosis exfoliativa.

Ichthyosis bullosa of Siemens is an autosomal dominant disease characterized by mild hyperkeratosis and blistering. Autosomal dominant ichthyosis exfoliativa is a recently described disease with clinical features similar to ichthyosis bullosa of Siemens, but in contrast to ichthyosis bullosa of Siemens no histologic signs typical for epidermolytic hyperkeratosis are observed. We used linkage analysis to test whether keratin gene mutations might underlie both diseases. This analysis showed linkage of both disorders with the region of chromosome 12 in which the keratin type II gene cluster is located. The keratin type I gene cluster on chromosome 17 is excluded. These data, combined with clinical observations, strongly suggest that the genes coding for keratin 1 or keratin 2e, both expressed in the suprabasal compartment of the epidermis and located in the type II gene cluster, are candidate genes for ichthyosis bullosa of Siemens and ichthyosis exfoliativa.

Clinical features of superficial spreading melanomas with zones of regression.

To assess the significance of spontaneous regression in superficial spreading melanoma (SSM), 36 patients with clinical signs of regression in their primary tumor were compared to 200 patients with regular SSMs (controls). SSMs with regression were found to have the following, distinctive clinical features, which were significantly different from controls (P less than 0.05): (1) male predominance (69%), (2) preferential localization on the trunk (80.6%), (3) lower tumor thickness (Breslow), (4) clustering in Clark levels II and III, and (5) a larger surface area. The incidence of metastases was lower in patients with regressing SSMs (13.9%) compared to controls (20.5%) although the time until relapse was slightly shorter (20.6 months versus 28.1 months for controls). These prognostic parameters were not significantly different. However, of the patients who died, 2 of 4 with zones of regression had thin melanomas (less than or equal to 1.5 mm), compared to only 1 of 27 without regression zones (P less than 0.05). SSMs with regression therefore have unique clinical features, which may be related to their pathogenesis, and they may have some prognostic significance.

Monoclonal gammopathy in scleredema. Observations in three cases.

A monoclonal gammopathy was observed in three patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. Deposition of monoclonal immunoglobulins in the skin was not detected by direct immunofluorescence microscopy. In contrast to scleromyxedema (lichen myxedematosus), from which scleredema can be distinguished clinically and histologically, the monoclonal immunoglobulins in two cases were of IgG2-kappa and IgG3-kappa type. Only one of the three patients had IgG1-lambda paraproteinemia, which is frequently seen in scleromyxedema. Our findings suggest that diffuse scleredema may be characterized by paraproteinemia but that the possible role of monoclonal immunoglobulins in the pathogenesis of this disease has yet to be resolved.

An ultrastructural study of epidermolytic leukoplakia.

A leukoplakic lesion of the lip showing the histologic features of epidermolytic hyperkeratosis above solar elastosis was investigated by electron microscopy. The ultrastructural alterations observed in the upper epidermal layers corresponded in the main with those of epidermolytic hyperkeratosis in other skin diseases. The keratinocytes showed irregularly formed tonofilaments, a marked intracellular edema, and premature cornification. In addition, there were discrete subcellular signs of premalignancy in the cells of the basal and suprabasal layers. These alterations suggest that the epidermolytic leukoplakia represents a rare histopathologic variant of actinic cheilitis.

Type 2 segmental manifestation of Hailey-Hailey disease: poor therapeutic response to dermabrasion is due to severe involvement of adnexal structures.

In autosomal dominant skin conditions, two different types of segmental manifestation can be distinguished. Type 1 represents heterozygosity for a postzygotic mutation, resulting in a degree of severity similar to that of the nonmosaic phenotype. Type 2 reflects loss of heterozygosity and shows an excessively pronounced involvement superimposed on the ordinary nonsegmental phenotype. We describe the clinical, histopathological and therapeutic aspects of the first case of type 2 segmental manifestation of Hailey-Hailey disease (HHD). A 24-year-old woman with a family history of HHD comprising four generations, presented with lesions of erythema and blistering arranged in a unilateral pattern following the lines of Blaschko. The disorder was first noted at the age of 3 months. At the age of 24 years, additional scattered symmetrical lesions involving the axillary and inguinal folds were noted. Histopathological examination of the severely involved linear skin areas revealed pronounced acantholysis within the deep adnexal structures, whereas clinically unaffected skin showed the typical histopathological features of the heterozygous phenotype with suprabasal clefting and acantholysis sparing the adnexae. Dermabrasion was performed in the areas of segmental involvement. During a follow-up period of one year, no recurrence was noted, but 18 months after dermabrasion a recurrence was present in the left submammary and left perianal regions. This therapeutic resistance to dermabrasion may be explained by the presence of acantholysis within the adnexal structures of the skin as found in type 2 segmental HHD.

[Syringolymphoid hyperplasia with alopecia]. / Syringolymphoide Hyperplasie mit Alopezie (SLHA).

Syringolymphoid hyperplasia with alopecia is a chronic patchy skin eruption consisting of hairless, anhidrotic, reddish-brown papules associated with pseudofollicular hyperkeratosis. Skin biopsies show dense lympho-histiocytic infiltrates aggregated in the dermis and around the hyperplastic sweat ducts and glands, imitating epithelial islands. Occasionally, obliteration of blood vessels may be found. This case report deals with a 52-year-old man suffering since 9 years ago from this distinctive skin disease.

[Neuropathia mucinosa cutanea]. / Neuropathia mucinosa cutanea.

A 29-year-old man developed progressive, reticular livid skin lesions with neuropathy and hypertrophy of intradermal nerves encased by mucinous material, which has not previously been reported. The name of neuropathia mucinosa cutanea is suggested.

[Pilar sheath acanthoma]. / Haarscheidenakanthom.

Pilar sheath acanthoma is a rare, benign follicular hamartoma. A case is reported, and the histological and clinical features of this skin tumor are described. The tumor is elevated and characterized by a central punched-out area filled with amorphous whitish material. In contrast to the keratoacanthoma, which has a similar clinical appearance, pilar sheath acanthoma shows no spontaneous regression.

Relapsing linear acantholytic dermatosis.

A 5-year-old girl with a unilateral linear systematized skin lesion showing the clinical, histological and ultrastructural features of Hailey-Hailey disease is described. We suggest that this relapsing linear acantholytic dermatosis represents a new distinct entity within the spectrum of unilateral linear skin diseases.

[Bilateral segmental neurofibromatosis]. / Bilaterale segmentale Neurofibromatose.

Segmental neurofibromatosis is a rare type of neurofibromatosis. We report a case of bilateral manifestation, review the literature on this extremely uncommon variant, and discuss the possible causative mechanisms and the genetic risk of segmental neurofibromatosis.

[Erythrokeratodermia anularis migrans--a new genetic dermatosis?]. / Erythrokeratodermia anularis migrans--eine neue Genodermatose?

An unusual type of erythrokeratodermia in an 11-year-old boy is presented. It can be distinguished from the well-known classic types of erythrokeratodermia by clinical criteria, such as the persisting, very slowly migrating, annular lesions, and also by immunohistochemical and ultrastructural findings. In view of the distinct clinical features of this new genodermatosis, the term erythrokeratodermia annularis migrans is proposed.

Autosomal dominant ichthyosis exfoliativa.

An unusual ichthyosiform dermatosis with circumscribed areas of blistering hyperkeratoses as well as scaly areas was observed in five consecutive generations of a German family. Light and electron microscopical studies revealed oedema of the keratinocytes in the upper epidermis but no defects of tonofilaments and keratohyalin granules. We suggest that this type of ichthyosis exfoliativa inherited as an autosomal dominant trait represents a new and distinct entity.

[Delayed type allergic reaction to red azo dye in tattooing]. / Allergische Spättypreaktion auf roten Azofarbstoff in Tätowierungen.

Allergic reactions in tattoos are comparatively rare. In most cases the reactions are caused by different red pigments. While in the past these reactions have been ascribed to mercury salts (cinnebar) and cadmium sulphide, now synthetic inorganic azo dyes have also been found to be responsible for such reactions. A 42-year-old man presented with an allergic reaction in the red parts of his tattoos. Histologically a chronic granulomatous, partly fibrous inflammation with transfollicular elimination of pigment granules was found. Spontaneous regression in a part of the inflammatory reaction was observed, simultaneously with depigmentation and scarring of the overlying skin. The pigment used for tattooing was found to be an aromatic azo derivative. In addition to a positive cutaneous reaction to the dye, the patient also showed a positive patch test to Napthol AS, used for the coupling of different dyes in the textile industry.