[Drug reaction with eosinophilia and systemic symptoms (DRESS): a hypersensitivity reaction with various symptoms]. / "Drug reaction with eosinophilia and systemic symptoms" (DRESS): eine Überempfindlichkeitsreaktion mit vielfältiger Symptomatik.

Drug reaction with eosinophilia and systemic symptoms (DRESS) is an illness which is difficult to diagnose because of its various symptoms. In our case, a patient with small spotted exanthema with nearly erythroderma and eosinophilia presented to the emergency room. Systemic steroid therapy was started on suspicion of a drug reaction. Over the course of time, the patient's general condition deteriorated significantly and the patient developed cholecystitis, Staphylococcus aureus bacteremia, pneumonitis and cytomegalovirus reactivation. With this case report, we want to show that DRESS is a disease that is difficult to treat and can develop after a long delay.

[Pemphigoid gestationis after egg donation : A case report]. / Pemphigoid gestationis bei Eizellspende : Ein Fallbericht.

Pemphigoid gestationis (PG) is a blistering autoimmune skin disease, typically occurring in the second and third trimester of pregnancy. Aberrant expression of major histocompatibility complex (MHC) class II molecules on the chorionic villi seems to lead to antibody production against bullous pemphigoid (BP)-180. We report a case of PG in a woman whose pregnancy was achieved using egg donation. Since the entire fetal genome is allogeneic to the mother, augmented immune reaction in egg-donated pregnancies appears to trigger the occurrence of PG.

[Adult-onset Still's disease : Rare adult-onset autoinflammatory syndrome]. / Adulter Morbus Still : Seltenes autoinflammatorisches Syndrom im Erwachsenenalter.

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by high spiking fever, arthritis, salmon-pink maculopapular rash and multiple organ involvement. We report a case of an adult-onset Still's disease that meets Yamaguchi's criteria and presented with typical clinical manifestations. AOSD is treated with anti-inflammatory medications. Standard therapy includes corticosteroids. Other medications like azathioprine, methotrexate or interleukin-1 or -6 blockers can be used when standard steroid treatment is not adequate.

Reevaluation of established and new criteria in differential diagnosis of Spitz nevus and melanoma.

The histopathologic differentiation between Spitz nevus and melanoma is of particular interest in routine diagnostic procedures of melanocytic tumors. Atypical Spitz nevi are sometimes difficult to distinguish from melanoma. There is still no single criterion that ensures a distinction of melanoma and atypical Spitz nevus. The aim of this study was to reevaluate established and new criteria to differentiate Spitz nevus from melanoma more reliably. We analyzed 25 melanomas with a Breslow index ≥ 1 mm and 18 classical compound Spitz nevi concerning their histopathologic, immunohistochemical and molecular genetic characteristics. Moreover, clinical follow-up data for 5 years were collected. We found statistically significant differences between Spitz nevus and melanoma for the following features: pagetoid spread, atypia, maturation, elastosis, Kamino bodies, p16 expression, and the staining pattern of HMB45. BRAF was positive in 7/21 melanomas and in 1/14 Spitz nevi. Fluorescence in situ hybridization confirmed the histopathologic diagnosis in 36/37 cases. The established clinical, histopathologic, and immunohistochemical criteria to differentiate Spitz nevus and melanoma could be reproduced in our collective. Especially, the expression of p16, BRAF analysis and fluorescence in situ hybridization proved to be helpful tools to improve the differentiation of Spitz nevus and melanoma in our study. Nevertheless, there is-until now-no reliable histopathologic and immunohistochemical parameter which can discriminate Spitz nevus and melanoma with absolute certainty.

Diagnostic approach in lymphoplasmacytic plaque.

BACKGROUND: Lymphoplasmacytic plaque (LPP) is a recently described rare skin disease characterized by a dense dermal lymphohistiocytic infiltrate with polyclonal plasma cells. The clinical picture is distinct with reddish to brownish plaque with a predilection for the lower leg. LPP typically affects children. OBJECTIVE: To define clinical and histologic criteria of LPP and to develop a diagnostic flow chart. METHODS: We investigated six of our own LPP cases. Immunoglobulin light chains, IgG, IgG4, CD31, CD163 as a histiocytic marker were examined by immunohistochemistry. PCR-based molecular studies were conducted for borrelia sp., mycobacterial and leishmania sp. Moreover, 10 cases, which have been reported in the literature, were checked for the same features. RESULTS: We could differentiate three main histological patterns (superficial band-like only, [deep] dermal only and mixed). Acanthosis and interface dermatitis are key features in cases with a superficial band-like or mixed infiltrate. Granulomas and giant cells could be only found in about 30% of the cases. The number of plasma cells was variable accounting for 5-40% of the infiltrate. The number of blood vessels was increased in the majority of the cases. 'Free-floating' collagen bundles surrounded by histiocytes (pseudorosettes) were identified as a new histological feature. An infectious agent could be excluded in all cases. CONCLUSIONS: LPP is a long-standing skin disease, which may also occur in adults and in other body regions than the lower leg. Reproducible clinical and histological criteria allow delineating a diagnostic work-up for LPP.

Steven Kossards postmenopausal frontal fibrosing alopecia (PFFA)--a therapeutic dilemma.

Steven Kossard described a new type of hair loss that he named frontal postmenopausal fibrosing alopecia (PFFA). In some of his patients he observed a symmetric regression of the frontal hair line. The eyebrows of the patients were also often affected. The histology of the lesions showed lichen planopilaris. Several cases of frontal fibrosing alopecia have been described- almost all of them in elderly women. We report a women with postmenopausal frontal fibrosing alopecia of Kossard. In our patient there were no other clinical signs of lichen planus on the rest of the body After systemic and local therapy with corticosteroids we were able to observe a termination in the disease. In the subsequent 6-month control period no regrowth of the hair follicles was found. Even if there is no proof for a hormonal basis of the disease, the effectiveness of finasteride in some patients may indicate that androgens might be partially responsible of the pathogenesis of the disease. The local and systemic medication with corticosteroids are not able to bring to a permanent remission and secondary growth of the hair follicles in the affected areas and this brings to the necessity of more invasive or innovative therapeutic methods, like skin transplantation and additional application of medicaments like blockers of the 5/alpha reductase, which have proven their capacity in the androgenetic male alopecia.

[Cutaneous marginal zone lymphoma (SALT) and infection with Borrelia burgdorferi]. / Kutanes Marginalzonenlymphom (SALT) bei chronischer Borrelia-burgdorferi-Infektion.

A relationship between Borrelia burgdorferi and the development of cutaneous B-cell lymphoma (CBCL) has been long discussed. B. burgdorferi DNA has been detected in patients with CBCL and a response of CBCL to antibiotics has been observed. In our patient with a Borrelia infection, a marginal zone lymphoma (SALT) regressed after ceftriaxone therapy. This further case of a combined appearance of CBCL and B. burgdorferi underlines a possible relationship as an example of an infectious trigger in tumorigenesis.

[Sonography of enlarged lymph nodes: Pathogenetic categorization using contrast enhanced power Doppler sonography]. / Sonographische Diagnostik vergrösserter Lymphknoten: Pathogenetische Kategorisierung mit der kontrastverstärkten Powerdoppler-Sonographie.

BACKGROUND: The purpose of this study was to categorize enlarged superficial lymph nodes as benign or malignant using sonomorphologic features and vascularization pattern. PATIENTS AND METHODS: Enlarged superficial lymph nodes in 57 patients were assessed with B-mode and contrast-enhanced power Doppler sonography. Morphology and vascularization were evaluated. The lymph nodes were categorized as benign or malignant. Correlation was made with histology and follow-up results. RESULTS: In 55 patients, 40 lymph nodes were correctly categorized as benign and 15 lymph nodes correctly as malignant. The most reliable criteria were shape and vascularization pattern. Intact hilar vessels and branching indicated benign enlargement, destruction of the hilum with vessels running peripherally along the capsule indicated metastatic destruction. Two benign lymph nodes were considered malignant (false positive). CONCLUSION: B-mode ultrasound along with contrast-enhanced power Doppler ultrasound is an easy, cost-effective, and reliable tool for differentiation and categorization of enlarged superficial lymph nodes.

Reactive angioendotheliomatosis: is it 'intravascular histiocytosis'?

We report the case of a 68-year-old female with reactive angioendotheliomatosis (RAE). This case highlights the benign course of this condition and suggests that this entity might be an intravascular histiocytosis.

[Anaplastic large cell lymphoma and keratoacanthoma]. / Grosszellig anaplastisches Lymphom und Keratoakanthom.

In CD30 positive lymphoproliferative disorders, e.g. anaplastic large cell lymphoma (ALCL) and lymphomatoid papulosis (LyP), pseudoepitheliomatous hyperplasia may occur in up to 50 percent of the cases. Epithelial tumors are rarely associated with ALCL or LyP, but the presence of single CD30 positive cells among the infiltrate accompanying epithelial tumors such as basal cell carcinomas and keratoacanthomas is well known. A 66-year-old man developed within a plaque of ALCL a rapidly growing epithelial tumor which we classified according to the clinical and histological features as keratoacanthoma. This case underlines the intimate interaction between CD30 positive lymphocytes and epithelial cells.

Cadherin expression pattern in melanocytic tumors more likely depends on the melanocyte environment than on tumor cell progression.

BACKGROUND: Adhesion molecules have been assigned an important role in melanocytic tumor progression. By the loss of E-cadherin, melanocytes might escape the control of neighbouring keratinocytes. Although in vitro data support this hypothesis, there are yet no conclusive immunohistochemical results on cadherin expression in melanocytic tumors. OBJECTIVE: To gain detailed insight in the expression of cadherins and their cytoplasmic binding partners, the catenins, in various types of benign and malignant melanocytic neoplasms. METHODS: Immunohistochemical analysis of the expression of E-, P-, and N-cadherin and alpha-, beta-, and gamma-catenin in compound and dermal nevi, Spitz nevi, blue nevi, ultraviolet B (UVB)-irradiated nevi, and malignant melanomas of various tumor thickness. RESULTS: In both nevi and melanomas, E-cadherin expression in melanocytic cells decreased, following a gradient from junctional to deeper dermal localization. The pattern of E-cadherin expression was more heterogeneous in melanomas than in nevi. In some melanomas, E-cadherin was only weakly positive in the epidermal tumor cells. P-cadherin expression was similar to that of E-cadherin. N-cadherin expression in melanocytic lesions was a rare finding, however, a small percentage of melanomas showed expression in some cell nests. Some Spitz nevi exhibited strong N-cadherin immunoreactivity. Most melanocytic cells were alpha- and beta-catenin-positive and gamma-catenin-negative. UVB irradiation did not influence the expression of cadherins and catenins in melanocytic nevi in vivo. CONCLUSIONS: It is presumed that the gradual loss of E-cadherin expression represents a reaction of melanocytic cells to altered conditions in the dermal environment, e.g. lack of contact to keratinocytes, or new contact with dermal extracellular matrix molecules, respectively. Melanoma cells apparently are less dependent on these environmental factors and, therefore, show a more heterogeneous expression pattern. This might be of importance for the adaptation of the tumor cells to local requirements. However, in view of our results, a causative role of (loss of ) E-cadherin or (gain of ) N-cadherin for melanocytic tumor progression still remains to be proven.

MMP-2, TIMP-2 and MT1-MMP are differentially expressed in lesional skin of melanocytic nevi and their expression is modulated by UVB-light.

BACKGROUND: In malignant melanoma, recent studies have demonstrated an important role of matrix-metalloproteinase 2 (MMP-2), its co-activating enzyme membrane-type matrix-metalloproteinase 1 (MT1-MMP), and the endogenous inhibitor of MMP-2, tissue-inhibitor of matrix metalloproteinase 2 (TIMP-2). Melanocytic nevi are benign neoplasms of the melanocytic lineage, but may exhibit dysplastic features that can be difficult to distinguish from early stage melanoma. As shown in earlier studies, nevi show important morphological and phenotypical changes in response to ultraviolet light (UVB) irradiation. OBJECTIVE: To clarify the role of MMP-2, TIMP-2 and MT1-MMP in UVB-irradiated vs. non-irradiated melanocytic nevi. METHODS: Immunohistochemical comparison of the MMP-2, TIMP-2 and MT1-MMP expression pattern. RESULTS: MMP-2 is expressed by lesional keratinocytes and its expression is up-regulated by UVB-irradiation. MMP-2 expression was not observed in melanocytic cells. TIMP-2, by contrast, is predominantly expressed by melanocytic nevus cells, and its expression is in part down-regulated by UVB-irradiation. MT1-MMP is expressed by basal keratinocytes and to a weaker extent by melanocytic nevus cells. CONCLUSIONS: MMP-2 expression by keratinocytes in nevi probably represents the result of activation of keratinocyte turnover in lesional epidermis. MMP-2 could play a role in the downward movement of junctional nevus cells into the dermis. The reduction of TIMP-2 expression in melanocytic cells by UV-light together with the enhanced expression of MMP-2 in the adjacent epidermis may promote basement membrane degradation. The expression pattern of MT1-MMP in close proximity to epithelial-mesenchymal interfaces underlines the synergistic role of MT1-MMP in this process.

[Keratoacanthoma. A variant of highly differentiated squamous cell carcinoma and its differential diagnosis]. / Keratoakanthom. Eine Variante des hochdifferenzierten spinozellulären Karzinoms und dessen Differenzialdiagnose.

Keratoacanthomas represent epithelial tumors which are characterized by a keratin-filled crater, rapid growth in the proliferation stage, and the potential for spontaneous regression. Histologically, keratoacanthomas display distinct features which must be differentiated from squamous cell carcinomas in the proliferation stage and fully developed tumors and in the regression stage the differential diagnosis of verruca vulgaris must also be considered.

Relationship between season and diagnoses of melanocytic tumours.

The frequency of various diagnoses of pigmented lesions, their distribution on various body sites and the seasonal variations in their diagnoses were studied according to the specimen of pigmented lesions sent to a laboratory for histopathological examination. From the total number of 18,402 investigated lesions, 7,682 were excised from males and 10,720 from females. Melanoma was diagnosed in 2.5% of all excised tumours more often during the summer months. Lighter clothing and greater awareness during summer may explain this finding. Additional factors such as a sun-induced melanocytic activation in naevi cannot be ruled out. Dysplastic naevi, which represented 26% of all naevi in this study, were excised less often during summer. Presuming that the majority of the north German population acquires the highest ultraviolet (UV) doses of the year during summer, it seems unlikely that the histological features of dysplasia in naevi are related to short-term UV activation. The localization of dysplastic naevi did not correspond to the body-site distribution of melanoma according to sex. This finding does not indicate the dysplastic naevus as a possible precursor lesion of malignant melanoma.

Clear cell trichoblastoma in association with a nevus sebaceus.

Nevus sebaceus is a hamartoma that is frequently associated with various neoplasms. Among the neoplasms observed in sebaceus nevi, trichoblastomas are the most common. The present case, to my knowledge, is the first description of a clear cell variant of trichoblastoma.