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W Kempf

Publications and source records attributed to W Kempf.

At least 37 records · Page 2Linked to original sources
PubMed2003

Pyogenic lymphoma of the skin: a peculiar variant of primary cutaneous neutrophil-rich CD30+ anaplastic large-cell lymphoma. Clinicopathological study of four cases and review of the literature.

Systemic anaplastic large-cell lymphoma (ALCL) in human immunodeficiency virus (HIV)-infected individuals showing an extensive infiltrate of neutrophils has been reported and referred to as 'neutrophil-rich' CD30+ ALCL. Secondary cutaneous involvement has been found in a subset of these cases. We report the clinicopathological features of four immunocompetent patients with primary cutaneous neutrophil-rich ALCL and present a new histological subtype with a dissolute growth pattern of CD30+ tumour cells. Four HIV-negative patients presented with rapidly growing solitary or multiple tumours located on the face. Ulceration of the lesions with purulent discharge was a typical finding. Various inflammatory dermatoses were considered clinically in all cases. The histological hallmark was a large number of neutrophils in the infiltrate that masked neoplastic CD30+ anaplastic cells. In two cases, a dissolute growth pattern of anaplastic tumour cells was observed. In two cases, a strong correlation between tumour growth and interleukin (IL)-8 cytokine pattern as well as the production of IL-8 by tumour cells was demonstrated. The diagnosis of neutrophil-rich ALCL is challenging clinically and histologically as the tumour cell compartment is masked by an extensive inflammatory infiltrate of neutrophils and other reactive cells such as histiocytes which may be mainly due to release of IL-8 by tumour cells. The term 'pyogenic' designates the typical feature of this distinct neutrophil-rich ALCL, namely abscess formation ('pyo-') by cytokines (IL-8) produced by tumour cells ('-genic'). The clinical behaviour of this type is the same as in primary cutaneous CD30+ ALCL with classical histological presentation.

Abscess↗
PubMed2003

Blastic natural killer-cell lymphoma of the skin associated with myelodysplastic syndrome or myelogenous leukaemia: a coincidence or more?

We report three patients with blastic natural killer (NK)-cell lymphoma of the skin associated with myelodysplastic syndrome (MDS) (two patients) or subacute myelomonocytic leukaemia (one patient). In two patients MDS was diagnosed before skin lesions; the patient with leukaemia initially presented with skin lesions. Our patients had several clinical features in common, namely multiple skin lesions with a bruise-like appearance, involvement of the oral mucosa, and good general status at presentation but very rapid deterioration in the course of the disease. All patients died of disease 4-14 months after the diagnosis. Histopathologically, there were cutaneous infiltrates of slightly pleomorphic medium-sized cells expressing CD4, CD56, terminal deoxynucleotidyl transferase (focally) and being negative for surface CD3, cytotoxic molecules, B-cell-associated markers and myelomonocytic markers. Erythrocyte extravasation was seen in all patients. T-cell receptor genes were in germline configuration. MDS was classified as refractory cytopenia with multilineage dysplasia and ring sideroblasts and refractory anaemia with transition to refractory anaemia with excess of blasts. We reviewed similar cases reported in the literature showing coexistence of blastic NK-cell lymphoma/leukaemia and MDS or myelogenous leukaemia. We conclude that given an overall limited number of reported cases of blastic NK-cell lymphoma/leukaemia, its association with various myelodysplastic/myeloproliferative disorders seen in a subset of patients ( approximately 15-20%) is more than coincidental and may indicate their common origin.

Aged↗
PubMed2003

HHV-8 DNA sequences in the peripheral blood and skin lesions of an HIV-negative patient with multiple eruptive dermatofibromas: implications for the detection of HHV-8 as a diagnostic marker for Kaposi's sarcoma.

BACKGROUND: Multiple eruptive dermatofibroma (MEDF) is a rare disorder seen in immunocompromised patients, simulating Kaposi's sarcoma (KS). Whereas KS is strongly associated with human herpesvirus 8 (HHV-8), the virus has never been detected in MEDF until now. OBJECTIVE: To present a patient with MEDF who showed no signs of immunodeficiency but was seropositive for HHV-8 antibodies and demonstrated HHV-8 DNA both in the peripheral blood and lesional skin of MEDF. METHODS: Clinical, histological and serological investigations were performed as well as polymerase chain reaction (PCR) studies and in situ hybridization (ISH). RESULTS: A 35-year-old white man with suspected KS was referred for evaluation of multiple pigmented nodules and patches. Biopsies revealed features of dermatofibroma, superficial fibrosing dermatitis and scar. One of the nodular lesions harbored HHV-8 DNA sequences. A faint amplification product was detected in the superficial fibrosing dermatitis lesion, while no HHV-8 sequences were found in normal skin and scar. Whole-blood samples and serum were positive for HHV-8. None of the skin lesions shown to harbor HHV-8 DNA sequences by nested PCR displayed a signal for HHV-8 RNA by ISH. Repetitive peripheral blood examinations did not reveal any serum antibodies against or antigens of HIV. Serum antibodies against the HHV-8 capsid antigen orf 65.2 were detected. CONCLUSION: Results of PCR studies and ISH indicate that the presence of HHV-8 in the lesional tissue was probably blood-borne due to viremia and not due to viral replication in tumor cells. The presence of HHV-8 is not fully restricted to KS. The differential diagnosis of KS and its simulators should be based on an integrative analysis of all available clinicopathological and molecular data and should not rely exclusively or predominantly on the presence or absence of HHV-8.

Adult↗
PubMed2003

Posttransplant CD30+ anaplastic large cell lymphoma with skin and lymph node involvement.

Posttransplant (i.e. status with the transplant present) lymphoproliferative disorders (PTLD) are common conditions in transplant recipients. Most examples are of B cell origin, and CD30+ T cell PTLD are very rare. We report a CD30+ anaplastic large cell lymphoma (ALCL) in the skin of the right lower leg and in draining lymph nodes of the right inguinal region in an immunosuppressed 59-year-old male who had received a renal graft 9 years previously. Unlike the vast majority of PTLD, an incomplete T cell immunophenotype was observed, and there was evidence of T cell lineage at the genetic level reflected by a rearranged T cell receptor gamma gene. The neoplastic cells were non-reactive to the anaplastic lymphoma kinase (ALK) 1 protein. In addition, Epstein-Barr virus and human herpesvirus 8 sequences were absent. Arguments against a primary cutaneous ALCL, which is also ALK-1 negative, include systemic presentation at the time of initial diagnosis and immunoreactivity of the neoplastic cells to epithelial membrane antigen. Typically, our rare example of a posttransplantation systemic ALCL showed an aggressive behaviour and a poor response to both chemotherapy and local irradiation.

Biopsy, Needle↗
PubMed2003

Unusual manifestation of specific cutaneous involvement by B-cell chronic lymphocytic leukemia: spontaneous regression with scar formation.

We report a patient with specific cutaneous involvement by B-cell chronic lymphocytic leukemia, who demonstrated unusual clinical features during the course of the disease, namely several spontaneous regressions of skin lesions with the formation of scars. In addition, histologically proven keratoacanthoma was found. During the follow-up period of approximately 1.5 years, the patient experienced several recurrences of skin lesions and their partial spontaneous regression. The scars persisted and remained unchanged. We hypothesize that vascular injury combined with edema could have accounted for dermal ischemia and the subsequent development of the scarring lesions.

Aged↗
PubMed2003

Treatment of verrucous carcinoma with imiquimod and CO2 laser ablation.

An 82-year-old female patient presented with a large perianal hyperkeratotic tumor extending into the anal canal. Staging did not reveal any metastatic spread. Diagnosis of verrucous carcinoma or Buschke-Löwenstein tumor, respectively, was based on typical clinical and histologic features. Moreover, human papillomavirus 6b DNA sequences could be detected by PCR. Surgical excision could not be performed due to the general health status of the patient; thus, alternative therapy methods were necessary. Treatment with imiquimod cream 5% (Aldara), a topical immune response modifier applied once a day and left for 12 h, led to significant partial tumor regression and clear demarcation of the tumor. The remaining tumor, now feasible for treatment with CO2 laser, was removed in two sessions in local anesthesia. In a third session, tumor parts in the anal canal were vaporized. This case demonstrates that the combination of imiquimod and CO2 laser ablation is an effective treatment option for verrucous carcinoma.

Aged↗
PubMed2003

Therapy of cutaneous lymphoma--current practice and future developments.

Cutaneous lymphomas include various types of clonal lymphoproliferative disorders. The adequate treatment approach depends on the exact diagnosis and should be non-aggressive in most cases. In early stages, local approaches such as UV or radiotherapy are preferred. In advanced stages, systemic drugs such as interferon-alpha or bexarotene can be administered. Experimental approaches for cutaneous lymphomas include vaccination and gene therapy.

Humans↗
PubMed2002

Primary subcutaneous follicular centre cell lymphoma with involvement of the galea: a case report and short review of the literature.

Primary cutaneous follicular centre cell lymphoma (FCCL) is a distinct subtype of cutaneous lymphoma that originates from germinal centre cells. Histologically, the disease is typified by a bottom-heavy infiltrate with a diffuse or follicular growth pattern situated in the mid or deep dermis. In some cases, the neoplastic infiltrate may involve the underlying subcutaneous tissue, but so far primary subcutaneous FCCL has not been reported. We report the first case of primary FCCL located primarily in the deep subcutis with extension into the galea and review the literature on primary subcutaneous B-cell lymphomas.

Female↗
PubMed2002

T-zone lymphoma with cutaneous involvement: a case report and review of the literature.

T-zone lymphoma (TZL) is a rare subtype of nodal peripheral T-cell lymphoma characterized by a clonal expansion of T-zone lymphocytes accompanied by a proliferation of other T-zone constituents. Non-specific cutaneous alterations are seen in about one-third of all cases, but specific cutaneous involvement is extremely rare. We present a case of TZL with secondary skin infiltration, review the literature on cutaneous manifestations of TZL and discuss the differential diagnosis of TZL.

Adult↗
PubMed2002

A case of Sézary's syndrome associated with granulomatous lesions, myelodysplastic syndrome and transformation into CD30-positive large-cell pleomorphic lymphoma.

Sézary's syndrome (SS) is a leukaemic variant of mycosis fungoides, a cutaneous T-cell lymphoma showing distinct clinical, histological, immunological, and genotypic features. We report a 10-year follow-up of a patient with SS exhibiting unusual features such as granulomatous skin lesions, transformation to a CD30-positive large-cell pleomorphic T-cell lymphoma, and development of myelodysplastic syndrome and review the cases of SS reported in the literature with these unusual and rare complications.

Fatal Outcome↗
PubMed2002

Monoclonal rearrangement of the T cell receptor gamma-chain in lichenoid pigmented purpuric dermatitis of gougerot-blum responding to topical corticosteroid therapy.

Lichenoid pigmented purpuric dermatitis of Gougerot-Blum belongs to a group of closely related disorders which are termed pigmented purpuric dermatoses. It clinically manifests itself with grouped lichenoid papules in association with purpuric lesions. We report a case of lichenoid pigmented purpuric dermatitis of Gougerot-Blum with a heavy band-like CD4-positive lymphocytic infiltrate and clonal rearrangements of the gamma-chain of the T cell receptors as detected by polymerase chain reaction/denaturing gradient gel electrophoresis. Monoclonal expansion of T cells in combination with certain histological features of mycosis fungoides (MF) might support a biological relationship between lichenoid pigmented purpuric dermatitis of Gougerot-Blum and MF. However, prompt clinical response to topical steroid therapy supports the benign clinical nature of our case.

Administration, Topical↗
PubMed2002

Superficial acral fibromyxoma: report of two cases.

Superficial acral fibromyxoma (SAFM) is a rare soft tissue tumor that has recently been delineated as a separate entity. We report 2 cases of SAFM and discuss its pathological features and differential diagnosis. Both patients had lesions on the toe. In 1 patient, the tumor was found after nail extraction, which had been performed for the treatment of onychomycosis, whereas in the other patient the tumor itself was the reason for seeking dermatological assistance. Biopsies from both cases demonstrated similar features. There was a moderately circumscribed, non-encapsulated tumor extending through the whole dermis. The neoplasm was composed of spindle and stellate cells with slight nuclear atypia arranged in a loose storiform, partly fascicular growth pattern. In 1 case, strands of cells with rather wavy nuclei were seen at the periphery of the tumor. Mitotic figures were scarce. The neoplastic cells were embedded in a myxoid stroma with increased numbers of small blood vessels and scattered mast cells. Immunohistochemically, the tumor cells showed weak focal positivity for CD34 and stained negatively for S-100 protein and alpha-smooth muscle actin. In 1 case epithelial membrane antigen (EMA) was negative, whereas in the second case focal expression of EMA by neoplastic cells was seen. Alcian blue staining revealed abundant mucinous material within the stroma. In conclusion, SAFM represents a distinct entity in the spectrum of cutaneous myxoid tumors. The differential diagnosis of SAFM includes various myxoid neoplasms and tumors with a predilection for distal parts of the extremities.

Aged↗
PubMed2002

Trichogerminoma: a rare cutaneous adnexal tumor with differentiation toward the hair germ epithelium.

In 1992, Sau and colleagues described 14 cases of a rare cutaneous adnexal tumor with differentiation toward the hair germ epithelium. All cases in their study displayed a unique constellation of histological features which allowed the authors to consider the neoplasm to be a distinct entity and to designate it as 'trichogerminoma' (TG). We present a case of an adnexal tumor identical to that described as TG and report the immunophenotypical features of this neoplasm for the first time. A biopsy from a 41-year-old man revealed a well-demarcated epithelial tumor composed of multiple lobules located in the deep dermis without connection to the overlying epidermis and surrounded by a fibrous pseudocapsule. The tumorous lobules were composed of uniform basophilic cells with vesicular nuclei, dispersed chromatin and prominent nucleoli showing no prominent peripheral palisading. Some of the lobules had the appearance of densely packed 'cell balls' with peripheral condensation of the tumor cells. The stroma showed a moderate number of fibroblasts and mast cells. Reticulum staining revealed fine reticulum fibers surrounding the tumor aggregates with accentuation of the 'cell balls'. Immunohistochemically, the tumor cells expressed AE1/AE3, CK5/8, CK5/6, CAM5.2 and stained negatively for CK20, CK7, calretinin, Lu-5 and Thomsen- Friedensreich antigen. There was no increase in the numbers of CK20-positive Merkel cells in the epidermis overlying the tumor; however, a few Merkel cells were scattered in some tumor lobules. In addition, we stained 6 trichoblastomas (TBs) and found a particular pattern of calretinin expression in this tumor which was not observed in our case of TG. We conclude that pathological features allow the delineation of TG as a distinct adnexal neoplasm. Histological differential diagnosis includes basal-cell carcinoma (pilar type), large nodular TB, trichoblastic fibroma, trichoepithelioma, tricholemmoma, pilomatricoma and matrical carcinoma.

Adult↗
PubMed2002

Primary cutaneous B-cell lymphomas and pseudolymphomas: review of histopathological features.

The terminology and classification of lymphoproliferative disorders in the skin remains a source of confusion for clinicians and pathologists. More than 25 types of malignant lymphoid neoplasms may involve the skin. These either arise primarily or represent secondary cutaneous involvement due to the spread of node-based lymphomas, other extracutaneous lymphomas or leukemias. In addition, there is a variety of cutaneous pseudolymphomatous reactions. This article addresses the histopathological features of primary cutaneous B-cell lymphomas and B-cell pseudolymphomas and presents a modern approach to the diagnosis of these lymphoid neoplasms.

Humans↗
PubMed2001

[Drug reaction to terbinafine simulating an acute generalized exanthematous pustulosis].

Oral terbinafine (Lamisil) is a widely used and effective therapy of dermatophyte infections. A 20-year-old patient was treated with oral terbinafine for an inguinal-scrotal fungal infection and developed an acute generalized exanthematic pustulosis (AGEP) and fever. Histological examination of skin biopsy revealed subcorneal and spongiform pustules. The diagnosis of an AGEP induced by terbinafine was established based on the history, clinical manifestations, and laboratory findings. Clinical features, differential diagnosis, causative drugs, and the therapy of AGEP are discussed and the literature on cases of terbinafine-induced AGEP is reviewed.

Adult↗
PubMed2001

[Granulomatous slack skin: partial remission following intralesional administration of interferon-alpha and PUVA].

Granulomatous slack skin (GSS) is a rare cutaneous T-cell lymphoma which typically runs a protracted and indolent course. On histopathological assessment lymphoid infiltrates with multinucleated giant cells in the dermis and subcutis with elastophagocytosis can be observed. Skin lesions are characterized by pendulous folds. We report on the successful response of the lesions to intralesional interferon alpha combined with PUVA.

Adult↗