Variable course of patients with plaque parapsoriasis [correction of psoriasis]: lack of transformation into tumorous mycosis fungoides.
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Biomedical subjects
Publications and source records attributed to M Mempel.
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A 53 year old female patient developed a keratoacanthoma, present on the left side of her nose for two months. After repeated unsuccessful trials with cryotherapy, she was treated with intralesional injection of methotrexate performed twice with a weekly internal. This led to complete resolution within 5 weeks. Although controlled studies on the effect of methotrexate in keratoacanthoma are not available, the rapid regression and the good cosmetic result suggest that this therapy may play a beneficial role in treating these lesions especially in problem locations.
The T-cell-reactive (eg, tuberculoid and reversal) forms of leprosy represent a well-defined granulomatous reaction pattern against an invading pathogen. The immune response in cutaneous sarcoidosis is a granulomatous condition that pathologically is very similar to T-cell reactive leprosy. However, it lacks a defined causative agent. In view of the role of NKT cells in murine granulomas induced by mycobacterial cell walls, we have searched for the presence of NKT cells in the cutaneous lesions of both leprosy and sarcoidosis. These cells were present in T-cell-reactive leprosy but were undetectable in cutaneous sarcoidosis. We have also studied the TCR Valpha repertoire in the two diseases. In addition to Valpha24(+) NKT cells, all patients with T-cell-reactive leprosy showed a very restricted T-cell-reactive Valpha repertoire with a strong bias toward the use of the Valpha6 and Valpha14 segments. Valpha6 and Valpha14(+) T cells were polyclonal in terms of CDR3 length and Jalpha usage. In contrast, most sarcoidosis patients showed a diverse usage of Valpha chains associated with clonal or oligoclonal expansions reminiscent of antigen-driven activation of conventional T cells. Thus the origin and perpetuation of the two kinds of granulomatous lesions appear to depend on altogether distinct T-cell recruiting mechanisms.
BACKGROUND: UVA1 (340-400 nm) therapy proved to be highly effective in patients with severe atopic dermatitis. The optimal dose regarding therapeutic efficacy and possible side effects is still to be evaluated. In vitro cell culture as well as in vivo animal studies recently indicated that a correlation between UVA irradiation and photoaging, skin carcinogenesis, or melanoma induction may exist. Therefore it seems appropriate to focus research activities on reducing the UVA1 dose applied during phototherapeutic regimens minimizing nonbeneficial side effects. OBJECTIVE: The present study was performed to evaluate the therapeutic effectiveness and long-term efficacy of medium-dose UVA1 irradiation in patients treated for acute exacerbated atopic dermatitis. METHODS: Thirty-two patients underwent a medium-dose UVA1 therapy consisting of 15 treatments applied from Monday to Friday for a period of 3 weeks. The applied dose per treatment was 50 J/cm(2) resulting in a cumulative dose of 750 J/cm(2). Clinical severity was assessed according to the SCORAD index before and after irradiation as well as in monthly intervals up to 3 months after cessation of phototherapy. RESULTS: Medium-dose UVA1 phototherapy is effective for alleviating acute exacerbated atopic dermatitis as shown by a significant reduction of SCORAD ratings (P <.001) at the end of the active UV treatment period. A significant skin improvement was still present 1 month later (P <.001). However, at the end of the 3-month posttreatment observation period the skin condition had reached the pretreatment level. CONCLUSION: According to our data, medium-dose UVA1 phototherapy is a highly effective, nonsteroidal, therapeutic alternative for treatment of acute exacerbated atopic dermatitis. However, effectiveness is merely short term, limited, and is followed by recurrence of symptoms within a 3-month observation interval.
Fourteen patients suffering from acute, exacerbated atopic eczema were screened for changes in collagen I and collagen III metabolism in serum (n = 11), urine (n = 11) and skin biopsies (n = 9) before and after medium-dose ultraviolet (UV) A1 phototherapy (15 exposures of 50 J/cm2 over a 3-week period, total dose 750 J/cm2). Mature collagen I and, to a lesser extent, mature collagen III were found to be decreased after the therapy in skin samples from the irradiated patients. As markers of collagen I degradation, the cross-links pyridoline and deoxypyridoline were analysed in urine using high-performance liquid chromatography. Both cross-links were found to be mildly increased after UVA1 phototherapy, without reaching statistical significance. As markers of de novo collagen synthesis we screened for the procollagen I-carboxyterminal peptide (PICP) and procollagen III-aminoterminal peptide (PIIINP) levels in serum and skin. The ratio of PICP to PIIINP in serum dropped significantly after the UVA1 phototherapy, suggesting a different impact of UVA1 on the two collagens. These findings were paralleled by a diminished ratio of PICP to PIIINP in tissue samples. Staining for matrix metalloproteinase 1 (MMP-1) and its specific counterpart, tissue inhibitor of MMP-1 (TIMP-1), showed slight increases for both proteins by therapeutic UVA1; this was also seen in serum for TIMP-1 but not MMP-1. In our study, high-energy UVA1 doses induced changes of the skin collagens in patients with atopic eczema which are measurable by their metabolites in serum and urine.
Hepatosplenic gammadelta T-cell lymphoma is a recently identified entity in which lymphoma cells bearing the gammadelta T-cell receptor (TCR) infiltrate the sinusoids of the liver and the sinuses of the splenic red pulp and bone marrow, without lymph node involvement. It is also characterized by a recurrent cytogenetic finding, isochromosome 7q (i7q10). The authors report a case of hepatosplenic lymphoma of alphabeta T-cell phenotype that shares the same clinical, histologic, and cytogenetic characteristics of the previously described hepatosplenic gammadelta T-cell lymphoma. Fluorescent in situ hybridization performed with chromosome 7 probes showed the typical pattern of isochromosome 7q. Genomic analysis of the TCR gamma locus failed to detect a clonal rearrangement. This unique case of hepatosplenic lymphoma of alphabeta T-cell phenotype supports the possibility that lymphoid populations of different alphabeta or gammadelta phenotype that share similar homing and presumably functional properties could give rise to lymphomas displaying similar clinical and pathologic findings.
Unilateral laterothoracic exanthem a (ULE) is a self-limited, probably infectious-allergic skin disease predominantly affecting small children. We describe five such cases. The typical unilaterally located or at least unilaterally dominant exanthem usually starts in the axillary region and is characterized by red, partly confluent papules and fine scales. Two of the children presented with atypical manifestations of ULE. Due to its asymptomatic course, therapy is not necessary in the majority of cases.
Six patients (3 children and 3 adults) with acute exacerbated atopic eczema were treated with basic emollients in combination with chlorhexidine-soaked dressings over a period of three days using the "wet-pyjama" technique. Improvement of eczema was documented with the severity score "Scoring of Atopic Dermatitis" (SCORAD); most pronounced changes were found for the subjective parameters itch and sleep loss. Paralleling skin improvement a reduction of Staphylococcus aureus colonisation was noted. Improvement of skin changes lasted beyond the active treatment period. Wet-wrap dressings are an effective treatment modality for atopic eczema without use of corticosteroids and can be used easily on an outpatient basis when manufactured dressings are used.
A 26-year old patient who suffered from purpura for four years evolved into the complete clinical picture of Henoch-Schönlein-purpura in the past two years. A causative agent was never found. Multiple therapeutic approaches including systemic steroids, azathioprine as well as pentoxyfylline failed to control the arthralgias, intestinal symptoms and skin lesions. Therefore dapsone therapy was initiated at a dosage of 100 mg daily after checking the glucose-6-phosphate-dehydrogenase and with regular control of the blood methemoglobin level. Within two weeks, the patient's symptoms completely cleared, he has now been in remission for the past six months. As noted elsewhere, dapsone is an effective therapy for severe cases of Henoch-Schönlein- purpura.
Two children are described with the combination of aplasia cutis congenita (ACC) and transverse limb defects known as Adams-Oliver syndrome. Whereas in the first child the typical features of ACC, syndactyly and transverse nail dystrophy were only mildly expressed and associated defects of the central nervous system and cardiac malformations were absent, the second child suffered from a very severe expression of the syndrome, with a combination of ACC, syndactyly, cutis marmorata telangiectatica congenita and multiple cardiac and central nervous system malformations which resulted in fatal central respiratory insufficiency.
Mechanisms for the increased Staphylococcus aureus colonization in atopic eczema are only partially known. From the aspect of the bacterium, the presence of various extracellular matrix components seems important. In the host epidermal lipid deficencies disturbing barrier dysfunction are important. Staphylococcus aureus' immunological and inflammatory effects include the release of superantigens, additional exotoxins and exoenzymes and perhaps bacterial DNA-triggered mechanisms. Therapeutic possibilities include the use of systemic antibiotics in cases of generalized superinfected atopic eczema, the use of corticosteroids and specific antibiotic-antiseptic combinations in cases of localised superinfected atopic eczema and the wide-spread use of topical antiseptics in cases of microbial-laden atopic eczema.
Skin colonization with Staphylococcus aureus is a characteristic feature of atopic dermatitis with more than 90% of patients being colonized. Extracellular matrix proteins are important for the adherence of S. aureus to human keratinocytes. The bacterium interferes in the inflammatory process of atopic dermatitis in various ways, among which the ability to release superantigens in a high percentage of clinical isolates is of great importance. As the colonization correlates significantly with the severity of eczema, anti-staphylococcal treatment measurements are widely used. In cases of atopic dermatitis exacerbation with wide-spread weeping lesions, a systemic antibiotic treatment is warranted, with erythromycin no longer being recommended due to an increased resistance rate. In localized superinfected lesions the topical application of an antibiotic-glucocorticoid preparation may offer advantages to the mere steroid application. Based on efficacy and resistance data, fusidic acid is the antibiotic of choice. There is evidence that phototherapy in atopic dermatitis may be even more effective when combined with anti-staphylococcal measurements. In the future new therapeutical options may be available.
Colonization of human skin with Staphylococcus aureus is a common feature in a variety of dermatologic diseases. In order to reproducibly investigate the adherence of Staphylococcus aureus to human epidermal cells, an in vitro assay was established using the biotin/streptavidine labeling system and the HaCaT cell line. This assay was used to define the role of several Staphylococcus aureus surface proteins with regard to their function in the staphylococcal adhesion process. Our studies included the standard laboratory strain Newman as well as its genetically constructed mutants DU5873, DU5852, DU5854, and DU5886 generated by allele replacement or transposon mutagenesis, which are deficient in the elaboration of staphylococcal protein A (spa), clumping factor (clfA), coagulase (coa), and the fibronectin-binding proteins A and B (fnbA/B), respectively. In comparison with strain Newman all mutants showed remarkably reduced adherence to the HaCaT keratinocyte cell line in our assay, yielding only between 43% and 60% of the adherence capacity of strain Newman after 60 min. Bacterial adherence could be re-established by introducing the cloned wild-type genes for the surface proteins on shuttle plasmids into the chromosomally defective mutants, thus suggesting a pathogenetic role of these proteins in the attachment of Staphylococcus aureus to human keratinocytes. Bacterial adherence was additionally enhanced by alkaline pH-values that are characteristic for skin conditions with epidermal barrier dysfunction. The use of Staphylococcus aureus mutant strains, deficient in the elaboration of defined proteins, allows specific investigation of colonization and virulence factors of this dermatologic relevant microorganism.
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Gianotti-Crosti syndrome (GCS), a self-limiting papulovesicular acrodermatitis often associated with underlying viral infection, is mainly described in children. Nine children with GCS were evaluated with dermatologic examination and serologic tests for viral infections. Therapy was modified according to the subjective symptoms of patients, which included characteristic acrolocated papulovesicles, generalized skin eruption, and mild to severe pruritus. Results of serologic investigations revealed Epstein-Barr virus, Coxsackie A virus, parvovirus B19, and parainfluenza virus 1/2. In three children no underlying viral infection was found. Therapeutic interventions included topical clioquinol lotion 1 percent, topical application of corticosteroids, systemic antihistaminic therapy, and systemic methylprednisolone. Skin lesions resolved after 2 to 4 weeks in treated as well as in nontreated children. Although GCS in children often lacks close association with a causative viral infection, such severe infections as hepatitis B and human immunodeficiency virus must be considered. Whole-body involvement seems to correlate with severe pruritus and additional general symptoms requiring more intensive therapy.