Bibliothek

Notizen: The emission properties of a new phosphor based on LiTaO3:Tb3+ are described. It is shown to be a storage phosphor, i.e., after previous x-ray exposure, Tb3+ emission in green-red region of spectrum can be photostimulated by light in 400–800 nm spectral range. Emission centers (Tb3+ ions), electron storage centers (oxygen vacancies) and competing nonradiative recombination centers (oxygen ion and Li vacancy couples), are recognized as the key elements in the energy transfer processes and overall emission efficiency of LiTaO3:Tb3+. © 1996 American Institute of Physics.

Notizen: Introduction: B-cell chronic lymphocytic leukemia (B-CLL) represents a low grade B cell lymphoproliferative disease with overlapping features with small lymphocytic lymphoma. The neoplastic cell is an autoreactive CD5 CD23 B lymphocyte. B-CLL may involve the skin, typically in the context of known disease. We present a case of subclinical B-CLL presenting initially in the skin. Results: A 73-year-old male developed a lesion on his right cheek in April 2003 compatible with basal cell carcinoma. The re-excision specimen contained a well differentiated atypical lymphocytic infiltrate consistent with B-CLL along with residual carcinoma. Subsequent laboratory studies revealed slight thrombocytopenia and mild peripheral blood lymphocytosis. A diagnosis was made of stage 0 CLL. Chromosomal studies on peripheral blood showed a deletion at 13q14.3. Conclusion: This case represents a cutaneous presentation of subclinical B-CLL. There are two prior reports describing B-CLL presenting initially in the skin. In one case the infiltrates were incidental on a re-excision specimen. The second report suggests 16% of B-CLL patients have cutaneous manifestations as the first sign of disease. Data indicates that 13q14 deletion in B-CLL in early stage disease may portend a more aggressive course, possibly explaining its early presentation in the skin in our case.

Notizen: Background: Dermatomyositis, a connective tissue disease syndrome where antibodies to the endothelium of the microvasculature of the skin, muscle and lung are implicated in lesional propagation, is characterized by photodistributed erythema, heliotrope rash, Gottron’s papules, muscle weakness and interstitial pulmonary fibrosis. Endotheliotropic viruses and underlying neoplasia are among the inciting triggers. Uncommonly drugs, namely the lipid lowering agents, have been implicated in dermatomyositis. Case Report: The patient, a 57-year-old male, developed a photodistributed rash and muscle weakness following treatment with the antifungal medication Lamisil. A skin biopsy was performed, showing an atrophying interface dermatitis with pandermal mucinosis and striking vasculopathic changes including endothelial cell necrosis with denudement and basement membrane zone reduplication. Direct immunofluorescent testing showed prominent staining of C5b-9 along the dermal- epidermal junction and within the vasculature. Conclusion: We have shown a temporal association between use of Lamisil and the development of dermatomyositis. Terbinafine, the active agent in Lamisil, promotes apoptosis of human endothelial cells in culture. Enhanced endothelial cell apoptosis results in the displacement of various cellular antigens creating a state of neo-antigenicity; its attendant sequelae has held to be one of antiendothelial cell antibody formation, a defining pathogenetic event in the evolution of dermatomyositis.

Notizen: Introduction: Most primary cutaneous T cell lymphomas are derived from mature T lymphocytes. We encountered a case of primary cutaneous T cell lymphoma with an undescribed phenotypic profile as defined by CD3+, CD8+ lymphocytes that co-expressed CD10, CD20 and CD30. Results: The patient, a 65-year-old woman, presented in 2001 with a thigh mass that was biopsied and held to represent a lymphoblastic T cell lymphoma based on CD10 expression. Two years later the patient developed a recurrence. The pathology showed a large cell lymphoma manifesting a CD8 phenotype although with CD10/CD20 expression. Conclusion: The importance in this case lies in the novel phenotype, namely a mature post thymic T cell lymphoma exhibiting CD10 and CD20 positivity. CD10 expression in primary cutaneous T cell lymphoma in the literature is largely limited to two case reports. CD10, a neutral endopeptidase, cleaves proteins with inflammatory or proinflammatory activity released by dying malignant and benign T cells including those of post thymic origin and may also degrade cytokines which inhibit T-cell apoptosis. Another unique aspect of our case was CD20 positivity whereby only a small number of prior citations exist, all with a CD8 phenotype as was the case in our patient.

Notizen: Various oral medications including nonsteroidal anti-inflammatory drugs (NSAIDs) have been associated with pseudoporphyria, although the pathogenetic basis has not been elucidated. A novel NSAID nabumetone (Relafen) has become popular because of its minimal gastrointestinal side effects, Its association with pseudoporphyria is not reported save for its listing in the Physician's Desk Reference (PDR) as a possible side effect. Biopsies of lesional skin from 4 patients manifesting blisters and erosions on the hands and face within 4 months of starting nabumetone were submitted for light microscopic and immunofluorescent (IF) studies. Histories and serology were obtained. Two patients had rheumatoid arthritis (RA), 1 had mixed connective tissue disease (MCTD), and I received diltiazem. All 4 had antinuclear antibodies. Characteristic clinical, light microscopic and IF features in the absence of elevated urine porphyrin levels confirmed a diagnosis of pseudoporphyria in all 4 patients. Biopsies in three patients showed features attributed to underlying connective tissue disease (CTD), including ectasia of the superficial vascular plexus, mild leukocytoclastic vasculitis, superficial and deep perivascular lymphocytic infiltrates with dermal mucinosis, granular deposition of IgM along the dermoepidermal junction indicative of a positive lupus band test, and of IgG and C5b-9 within keratinocytes. Nabumetone (Relafen) can provoke pseudoporphyria; an underlying CTD diathesis may be a predisposing factor.

Notizen: We present 20 patients in whom drug therapy was associated with interstitial histiocytic infiltrates with variable degeneration of collagen and elastic fibers mimicking early lesions of granuloma annulare (GA). Most patients had a reproducible clinical presentation comprising erythematous-to-violaceous, nonpruritic plaques, often with an annular pattern, predominantly involving inner aspects of the arms, medial thighs and intertriginous areas. The most frequent clinical differential diagnoses inchided cutaneous T cell lymphoma, erythema annulare centrifigum (EAC), GA, and lupus erythematosus. A drug reaction was suspected in only 3 cases. The implicated drug classes included calcium channel blockers, angiotensin converting enzyme inhibitors, beta-blockers, lipid-lowering agents, antihistamines, anticonvoilsants and antidepressants. Patients were often on two or more of these drugs; all have been associated with pseudolymphomatotis infiltrates of the skin, the presumptive basis of which is iatrogenic pertubation of immune function. The defining histomorphology was diffuse infiltration of the interstitium by lymphocytes and histiocytes with piecemeal fragmentation of collagen and elastic fibers in concert with a vacuolar interface dermatitis. Ten cases showed intermediate and transformed lymphocytes with hyperchromatic convoluted nuclei disposed interstitially within the dermis or along the dermoepidermal junction with variable epidermotropism. In the 15 patients who discontinued the implicated drug, lesional resolution occurred. We propose the designations interstitial granulomatous drug reaction for this novel cutaneous reaction pattern.

Notizen: The authors report the occurrence of lichenoid and/or subacute lupus erythematosus-like eruptions in a group of patients receiving agents with antihistaminic properties. In 1 of the patients the eruption clinically resembled lichen planus, while in 5 patients the eruption resembled subacute cutaneous lupus crythematosus (SCLE). At a light microscopic level all cases showed a lichenoid dermatitis and in 4 cases the lesions were interpreted as representing subacute lupus-like eruptions by virtue of the presence of alternating cell poor and lichenoid interface dermatitis, suprabasilar lymphocytosis around degenerating keratinocytes, and dermal mucinosis. Despite the resemblance at a clinical and light microscopic level to SCLE, anti-Ro seropositivity could not be established in any of the cases. One case, however, did demonstrate antihistone antibodies in concert with a high antinuclear antibody titer. A causal association was implicated by virtue of lesional resolution following drug withdrawal. The association of cutaneous eruptions resembling SCLE with antihistamine intake is seemingly a novel one. The possible pathogenetic basis of the eruptions is discussed.

Notizen: Pityriasis rubra pilaris (PRP) is an idiopathic erythematous scaling eruption which can be difficult to distinguish from psoriasis. We explored the clinical features, including background medical illnesses and potential triggers in patients with PRP, and also its histomorphologic spectrum. Patients with PRP were selected by natural language search of dermatopathology databases containing 250 000 cases accessioned over a 3-year period. A detailed medical history was obtained on each patient via office chart review and patient interviews. We compared the histologic findings in skin biopsies from patients with PRP to those of biopsies matched for site and age from control patients with psoriasis. Certain aspects of the clinical presentations encountered in the study group patients have not been previously emphasized, including eruptions compatible with a vesiculobullous disorder in 4 patients. Several patients had evidence of underlying iatrogenic or systemic immune dysregulatory states and certain triggers could be isolated such as emotional stress and prior exposure to microbial pathogens. Histomorphologic features predictive of PRP included follicular plugging, an increased granular cell layer, and acantholysis. While seen in a minority of biopsies of PRP, morphologic discriminators supportive of a diagnosis of psoriasis included neutrophils in the parakeratotic scale crust, and dermal papillae capillary ectasia with vessels lying in direct apposition to the epidermis. Other morphologic features were encountered in biopsies of PRP which have received little emphasis in the dermatopathology literature, including epithelial atrophy, significant dermal inflammation, the presence of eosinophils and plasma cells within the inflammatory infiltrates, an abnormal granular cell layer, and epidermolytic hyperkeratosis, albeit none were statistically signficant predictors of PRP over psoriasis. Nevertheless, their identification emphasizes the varied histologic spectrum of PRP. The almost ubiquitous finding of acantholysis and the presence of an abnormal granular cell layer in many biopsies of PRP suggests that abnormal keratins or defects in the retinol signalling pathway may be of pathogenetic importance.

Notizen: Abstract: Ichthyosis bullosa of Siemens (IBS) is a rare disorder of cornification characterized by blister formation in the upper suprabasal layers of the epidermis. Molecular analysis of IBS has identified mutations in the keratin 2e (K2e) gene, which is located in the type II keratin gene cluster on chromosome 12q. We have studied two IBS families and have identified heterozygous point mutations in codon 493 of the K2e gene in both families. Whereas a non-conservative amino acid substitution at position 117 of the 2B region of K2e (E117K) was associated with a severe phenotype in family 1, family 2 showed mild clinical features as a result of a conservative substitution (E117D). These data suggest a phenotype–genotype correlation in these families.

Notizen: Background:  The histologic hallmark of most arthropod bite reactions is a deep, wedge-shaped perivascular and interstitial infiltrate comprising lymphocytes, neutrophils, and eosinophils.Methods:  We present a case series of six patients in whom tick bite reactions, when examined microscopically, were found to mimic mixed cryoglobulinemic vasculitis.Results:  Though different in histology, clinically these lesions were indistinguishable from typical tick bite reactions.Conclusion:  As five of our six biopsy specimens were found to still harbor retained tick parts, it is possible that the actual retention of tick parts was involved in evoking this localized cryoprecipitate reaction.