[Malignant atrophic papulosis (Köhlmeier-Degos disease). Failure to respond to interferon alpha-2a, pentoxifylline and aspirin]. / Maligne atrophische Papulose (Morbus Köhlmeier-Degos) Kein Ansprechen auf Interferon alpha-2a, Pentoxifyllin und Azetylsalizylsäure.

A 45 year old female patient presented with the cutaneous manifestations of malignant atrophic papulosis (Köhlmeier-Degos disease) for two years. The typical papules with central porcelain-white atrophy correspond histologically to wedge-shaped necrosis of the connective tissue due to thrombotic occlusion of small vessels in the corium. The pathogenesis of malignant atrophic papulosis and effective treatment modalities are unknown. A slow virus infection has been suggested by some authors. Therefore, we attempted an immune therapy with interferon alpha-2a over a period of 11 months, but failed to cause a significant effect on the appearance and progression of the skin lesions. Furthermore, we could not confirm the effectiveness of a recently reported treatment modality with pentoxifylline and aspirin administered to our patient over a period of 5 months.

[Eccrine spiradenocarcinoma with unusual histiocytic giant cell components. Case report and review of the literature of a rare sweat gland tumor]. / Ekkrines Spiradenokarzinom mit ungewöhnlicher histiozytärer Riesenzellkomponente. Fallbericht und Literaturübersicht eines seltenen malignen Schweissdrüsentumors.

We report the case of a spiradenocarcinoma (malignant eccrine spiradenoma), a rare variant of a malignant sweat gland tumor, which occurred in the scalp of a 63-year-old woman. Some years before the current tumor occurred, a tumour of unknown histology had been excised from the same localization. The new tumor presented with rapid growth and exulceration. Malignant eccrine spiradenoma is characterized by the coincidence of a benign and a malignant portion within one specimen. We report case example 47 of this entity. The peculiarity of this case is the enormous heterogeneity with foci of atypical histiocytes like an atypical fibroxanthoma and an extensive foreign body reaction with cholesterol needles as in a xanthogranuloma. Static DNA cytophotometric analysis revealed different degrees of aneuploidy in the three tumor portions. The follow-up of 23 months is without recurrent tumor. A review of the literature is given.

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.

[Erysipelas carcinomatosum in tubular adenocarcinoma of the stomach]. / Erysipelas carcinomatosum bei tubulärem Adenokarzinom des Magens.

Erysipelas carcinomatosum is an inflammatory infiltration of dermal lymphatic vessels by tumor cells of a metastatic adenocarcinoma mimicking common erysipelas. It is most often due to adenocarcinomas, especially breast cancer. A 51 year-old male patient developed erysipelas carcinomatosum on his right chest wall due to a tubular adenocarcinoma of the stomach.

[Cutaneous sinus histiocytosis (Rosai-Dorfman disease)]. / Kutane Sinushistiozytose (Rosai-Dorfman-Erkrankung).

Sinus histiocytosis is as a rule a benign disease of lymph nodes, infiltrated by large histiocytes. These cells show typical cytophagocytosis, particularly lymphophagocytosis. Other organs may be also involved by this disease, often including the skin. Exclusive cutaneous sinus histiocytosis without infiltration of lymph nodes seems to be very rare. To exclude other non-X histiocytoses or histiocytosis-X, it is advisable to use immunohistochemistry. We report on a patient with sinus histiocytosis and discuss the problems of differential diagnosis.

[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.

[Acrokeratosis verruciformis-like changes in Darier disease]. / Acrokeratosis-verruciformisähnliche Veränderungen beim Morbus Darier.

In the literature, a number of observers reported that acrokeratosis verruciformis (Hopf) often evolves in cases of keratosis follicularis. The relationship of these verruciform manifestations to keratosis follicularis is discussed. In this paper, we present three patients with Darier's disease, who have verruciform papules on the dorsal surfaces of the hands and feet. Histopathologically, the papules showed the distinctive structures of Darier's disease. Therefore, these lesions are regarded as an abortive form of keratosis follicularis. It is necessary to examine carefully the punch biopsies histomorphologically. We also recommend having a closer look at the development of other typical Darier's skin lesions in the follow-up of patients.

Identification of aneuploidy in recurrent clear cell hidradenoma by DNA image cytometry (ICM-DNA).

Since malignant clear cell hidradenoma (CCH) is often characterized by only slight and sometimes by absent nuclear anaplasia, even in metastases, definitive differentiation from its benign counterpart by light microscopy may be very difficult. Herein, we report the case of a 72-year-old woman suffering from a CCH on the back, which showed local recurrence following incomplete excision. By light microscopy no unequivocal signs of malignancy were found either in the primary or the recurrent tumor. However, unusual deep extension to the subcutis with some architectural disorder was seen in the latter. Identification of prospective malignancy in such cases of borderline histopathological features is one of the main indications for diagnostic DNA image cytometry (ICM-DNA). Application of this method to enzymatic cell separation specimens of the recurrence detected marked DNA-aneuploidy with a stemline ploidy of 2.60 c and single events up to 6.7 c, whereas a nearly exact diploid DNA-stemline was found in the primary. We suppose from our results that a prospective malignant CCH with aneuploid DNA-stemline has developed out of its DNA-diploid counterpart. The need for total surgical removal of apparently benign clear cell hidradenomas is further stressed by this observation. Criteria for the diagnosis of malignancy in CCH are reviewed.

[Chronic sclerodermiform graft versus host disease (GvHD)]. / Chronische sklerodermiforme Graft-versus-Host disease (GvHD).

We report on a 41-year-old patient who developed extensive chronic sclerodermatous graft-versus-host disease (GvHD) subsequent to an allogeneic bone marrow transplantation from his HLA-identical sister. Chronic GvHD is to be expected in 30% of bone marrow transplantations. It is a serious multisystem disorder which leads to a polymorphic clinical picture with lichenoid or sclerodermatous skin changes. It may also affect nails, hair and mucous membranes. In the absence of causal therapy GvHD should be managed with symptomatic local treatment and systemic immunosuppression.

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.

[Necrobiotic xanthogranuloma in IgG kappa plasmacytoma and Quincke edema]. / Nekrobiotisches Xanthogranulom bei IgG-kappa-Plasmozytom und Quincke-Odem.

Necrobiotic xanthogranuloma is a non-X histiocytosis with unknown pathogenesis. It is associated with paraproteinaemia, and in rare cases with multiple myeloma. A decreased level of C1 inhibitor has been found in several cases without clinical manifestations of Quincke oedema. We report on a patient with necrobiotic xanthogranuloma and myeloma, in whom we found a decreased level of C1 inhibitor and recurrent episodes of manifest Quincke oedema. The indirect detection of auto-antibodies against the paraprotein with development of immune complexes is regarded as an explanation for the consumption of the C1 inactivator and the manifestation of Quincke oedema. The possibility of a causal relationship between paraproteinaemia and necrotic xanthogranuloma is discussed.

[A lupus-vulgaris like atypical mycobacteriosis caused by Mycobacterium xenopi (lupus xenopi)]. / Lupus-vulgaris-artige atypische Mykobakteriose durch Mycobacterium xenopi (Lupus xenopi).

A case of lupus-vulgaris-like infection caused by Mycobacterium xenopi in a 62-year-old immunocompetent female patient is presented. A large cutaneous infiltration was seen in the right periorbital region. Histological examination revealed a granulomatous reaction of epithelioid cells and giant cells. M. xenopi was isolated from biopsy material and tuberculosis could be excluded. Isoniazid was effective in healing the lesion within a year. Such infections are well known for other mycobacteria but to our knowledge had not yet been described for M. xenopi. The characteristics of human infections with M. xenopi are summarized in a review of the literature and criteria for the diagnosis of atypical cutaneous mycobacterioses are proposed.

[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.

[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.

[Pigmented spindle cell tumor]. / Der pigmentierte Spindelzellentumor.

The pigmented spindle cell tumor first described by Reed et al. is a benign melanocytic lesion of the skin that frequently remains unrecognized. The tumor is a heavily pigmented macule or plaque that usually appears on the extremities of young patients. The clinical and histological features and the differential diagnosis of pigmented spindle-cell tumor are described with reference to two clinical cases.

[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.

[Epidermolytic leukoplakia: an unusual precancerous condition]. / Epidermolytische Leukoplakie: Eine ungewöhnliche Präkanzerose.

We report on a rare type of leucoplakia of the lip and the prepuce showing the morphological features of epidermolytic hyperkeratosis. The development of squamous cell carcinoma in one of the patients strongly suggests that epidermolytic leucoplakia may represent a precancerous disorder of the transitional epidermis.