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PubMed1978

[Treatment of mycosis fungoides by PUVAtherapy. Report on 18 cases (author's transl)].

A group of 18 patients with mycosis fungoides (M.F.) was treated by PUVAtherapy. According to VanScott classification, they have been set in:--5 parapsoriasis in large plaques = 5 stages I;--6 stages II;--3 stages III;--2 stages IV;--1Sézary syndrom.--1 erythrodermia. There was complete clearing of 12 patients (66 p. 100); 2 patients (11 p. 100) improved cutaneous lesions without a complete clearing, and there was no response to treatment for 3 patients (22 p. 100) (1 with erythrodermia, 1 with Sézary syndrom and 1 stage IV, and 1 forsaking). The method applied here was different in several points: orally administered methoxalen were given according to the body area (mg/m2) and some of the patients had twice a day puvatherapy. Puvatherapy seems to be the least dangerous and most effective treatment for the patients in the early stages of mycosis fongoides (I, II) and nitrogen mustard, electron therapy, corticoids and even mono- or polychimiotherapy could be associated to puvatherapy, if necessary, for the stages III and IV.

Aged↗
PubMed1983

Stimulation of monocyte-mediated, antibody-dependent cytotoxicity in mycosis fungoides.

Antibody-dependent cytotoxicity mediated by purified blood monocytes were determined in untreated patients with plaque-stage mycosis fungoides (MF), parapsoriasis en plaques, exfoliating erythroderma, and noncutaneous malignant lymphoma. Compared with healthy control subjects and control subjects with varicose ulcers, patients with MF had increased monocyte cytotoxicity. In the other dermatoses, normal monocyte cytotoxicity was found. Patients with noncutaneous malignant lymphoma showed normal or decreased monocyte cytotoxicity. There was no evidence that abnormal monocyte cytotoxicity in cutaneous and noncutaneous malignant lymphoma were caused by interaction with lymphocytes or serum. The presence of increased monocyte cytotoxicity in patients with plaque-stage MF may be a clinically useful finding when this diagnosis is suspected clinically but not proved histopathologically.

Adult↗
PubMed1983

Disciform erythrasma.

A case of disciform erythrasma is presented. This unusual manifestation of a common cutaneous infection may mimic other dermatologic disorders, including lichen sclerosus et atrophicus and plaque-type parapsoriasis. The condition is characterized by an atrophic appearing surface, located in nonintertriginous areas. Appropriate diagnostic procedures easily differentiate disciform erythrasma. These include Wood's light examination, potassium hydroxide preparation, and skin scrapings or tissue sections stained with Gram stain, periodic acid-Schiff, Giemsa, or methylene blue.

Aged↗
PubMed1978

[Woringer and Kolopp's disease (author's transl)].

Woringer and Kolopp's disease is a rare skin disease. Erythemato-squamous, slightly infiltrated lesions with round-shaped islets of normal skin are the clinical hall-marks of the disease. The histological picture is highly specific: a dense epidermal infiltrate disrupts the stratum spinosum, contrasting with a spared dermis. Three different aspects of the disease have been reported: a localized benign type as in Woringer and Kolopp's patient; a disseminated lethal type without visceral involvement and without preexisting lesions, and a disseminated lethal type in which lesions appear on erythematous patches clinically and histologically similar to "parapsoriasis en plaques". It is the opinion of the authors that the third type is not Woringer and Kolopp's disease but an extremely epidermotropic variant of mycosis fungoides. The two other types seem to be variants of the same disease. Several hypotheses about the nature of that disorder are discussed. Most authors think that Woringer and Kolopp's disease is a cutaneous lymphoma because of clinical, histological and ultrastructural similarities with mycosis fungoides. We are presenting evidence favoring the hypothesis of a Merkel cell proliferative disease.

Adolescent↗
PubMed1981

Attempts to enhance light microscopic diagnosis of cutaneous T-cell lymphoma (mycosis fungoides).

Precise pathologic criteria for the diagnosis of mycosis fungoides (MF) remain controversial. With the use of a specific counting technique and defined criteria for cell types, we attempted to differentiate between a series of slides from patients with eczematous dermatitis, large plaque parapsoriasis, and atypical dermatitis with features that suggest MF, the plaque stage of MF, and the tumor stage of MF. This could not be done on the basis of cellular density in e defined field in the papillary dermis or on the basis of the percentage of atypical lymphocytes in the fields counted. Furthermore, individual investigator variance was shown to be highly significant despite efforts to minimize this factor.

Cell Count↗
PubMed1981

Sacroiliac joints in chronic dermatoses.

In 34 patients with different chronic skin diseases (including eczemas, lichen ruber, erythrodermia, pyodermia, pemphigus erythematosus, vasculitis, parapsoriasis varioliformis, scleroderma adultorum Buschke, prophyria cutanea tarda, rosacea, dermatomyositis, erythematodes chronicus discoides and indurative tuberculosis), X-ray studies revealed sacroiliac-changes consistent with a low-grade inflammation. This oteoarthritis slightly differs from the patterns disclosed earlier by the same authors in psoriatic patients, lacking any clinical bone or joint symptoms. So far, it has not been possible to explain their nature, cause or development.

Adult↗
PubMed1994

[Lymphomatoid papulosis and anaplastic giant-cell lymphoma].

INTRODUCTION: The association between lymphomatoid papulosis and malignant Hodgkin or non-Hodgkin lymphoma is well known but still raises the problem of nosology between these two pathologies. Is lymphomatoid papulosis a pseudolymphoma, a prelymphomatous state or a true skin lymphoma? CASE REPORT: We observed a patient who had lymphomatoid papulosis and anaplastic large-cell lymphoma within an interval of 8 years between. This case was particularly interesting because identical immunophenotypes were observed in the atypical large-cells of the skin and the lymphomatous cells of the lymph nodes (positive for CD43, CD45, CD25, CD30, CD15, EMA). DISCUSSION: This case points out that atypical large-cells of lymphomatoid papulosis express the CD15 antigen which is only expressed by atypical large-cells in half of the cases of lymphomatoid papulosis. In addition, EMA is classically expressed in primary lymph node lymphomas rather than in primary cutaneous anaplastic large cell lymphomas which could predict extracutaneous dissemination of lymphomatoid papulosis. Furthermore, the demonstration that the skin lesions and the lymph nodes responded differently to the same treatment would suggest that there are other unrecognized biological differences. Lymphomatoid papulosis appears to be a range of disorders of the lymphoproliferation of activated T-cells and could include varioliform parapsoriasis and cutaneous lymphoma.

Humans↗
PubMed1995

Cutaneous T-cell infiltrates: analysis of T-cell receptor gamma gene rearrangement by polymerase chain reaction and denaturing gradient gel electrophoresis.

In cutaneous T-cell infiltrates, the demonstration of a clonal T-cell receptor (TCR) gene rearrangement has been considered helpful to distinguish Cutaneous T-cell lymphomas from reactive lymphoproliferation. Hence, a polymerase chain reaction (PCR) method using GC-clamp primers and denaturing gradient gel electrophoresis has been developed in our laboratory to analyze the TCR gamma locus configuration. Two hundred eleven cutaneous samples from 155 patients were analyzed. A detectable clonal TCR gamma rearrangement was significantly associated with cutaneous T-cell lymphomas as defined by morphologic and immunologic criteria. A clonal TCR gamma rearrangement was also detected frequently in lymphomatoid papulosis, never in reactive lymphocytic infiltrates and B-cell lymphomas, and rarely in parapsoriasis en plaque and cutaneous lymphoid hyperplasia. Forty five patients had both a cutaneous and a peripheral blood sample. Fifteen had a detectable clonal rearrangement in the two samples and 22 were negative. Six patients had a positive skin sample and a negative blood sample, whereas two patients had a positive blood sample and a negative skin sample. Four lymph node samples were analyzed and the PCR results were the same as in the skin. Finally, 21 patients had sequential samples of recurrent skin lesions. The PCR results were concordant in all and, when detectable, the clonal TCR gamma rearrangement remained unchanged in a given patient. Because of its simplicity and accuracy, the newly designed PCR procedure improves the monitoring of diagnosis, staging, and follow-up in cutaneous T-cell infiltrates.

Base Sequence↗
PubMed1994

Molecular biology techniques for the diagnosis of cutaneous T-cell lymphoma.

The molecular biologic analysis of TCR gene rearrangements by Southern blot analysis and various PCR-based assays has contributed significantly to the understanding of CTCL. It is now known that CTCL is a monoclonal T-cell disorder like other T-cell neoplasms and that the same tumor clone is generally present in all sites of tissue involvement. Relative to histopathologic examination, the enhanced sensitivity of molecular biologic assays has allowed the diagnosis of CTCL at an early stage in many cases. In fact, molecular biologic analysis of TCR gene rearrangements suggests that CTCL may contain a dominant monoclonal tumor cell population from the time of its earliest clinically recognizable lesions, such as the cutaneous patches once termed large plaque parapsoriasis and now generally regarded as early CTCL. Furthermore, available data indicate that, at least in some cases, tumor cells are distributed widely among cutaneous and extracutaneous tissues at a time long before this involvement can be appreciated morphologically. It is apparent that, in addition to their value in the early diagnosis and staging of cutaneous lymphomas, these molecular biologic assays are valuable in monitoring the response to therapy, detecting early relapse, and improving understanding of the compartmentalization and trafficking of tumor cells. In order to reap the full clinical benefit from this new information, however, it is important to perform prospective long-term studies designed to determine the clinical significance of molecular biologic data. In addition, the complexity of cutaneous lymphoproliferative disorders dictates that molecular biologic clonality data should never be interpreted in a vacuum. In skin disease, dominant clonality does not always equate with clinical malignancy. The proper diagnosis of CTCL and other cutaneous lymphoproliferative diseases requires the thoughtful integration of molecular biologic data with the clinicopathologic and immunophenotypic findings.

Blotting, Southern↗
PubMed1993

Molecular diagnosis of lymphocytic infiltrates of the skin.

BACKGROUND: Advances in our understanding of the molecular genetics of lymphocyte antigen receptors (B-cell immunoglobulin and T-cell antigen receptor), have led to the application of molecular biologic techniques to molecularly characterize lymphocytic infiltrates of the skin. Molecular diagnosis refers to the application of these techniques as a diagnostic aid in the clinicopathologic evaluation of cutaneous lymphocytic infiltrates. OBSERVATION: Molecular studies have clinical application in the determination of lineage and detection of retroviruses in cutaneous lymphoid neoplasms, distinguishing between lymphoproliferative and reactive infiltrates, and staging and monitoring response to therapy in cutaneous T-cell lymphoma. Southern blot analysis of immunoglobulin and T-cell antigen receptor gene rearrangements may fail to aid the clinician in establishing a diagnosis of a cutaneous malignancy due to the limits of detection sensitivity in minimally infiltrated lesions (eg, parapsoriasis and patch-stage mycosis fungoides) or the still uncertain prognostic significance of clonality in benign cutaneous diseases (eg, follicular mucinosis, pityriasis lichenoides et varioliformis acuta, lymphomatoid papulosis, and cutaneous lymphoid hyperplasia). CONCLUSIONS: Molecular studies have enormous research value, providing new means to explore the pathogenesis and clonal evolution of lymphoproliferative skin diseases. Presently, however, they have limited applications as an independent diagnostic tool. As our understanding of the clinical and biologic significance of the molecular detection of clonal lymphocyte populations in the skin expands and as the application of polymerase chain reaction amplification provides us with greater detection sensitivity and specificity, the clinical utility of molecular diagnosis of lymphocytic infiltrates of the skin will be enhanced.

Antibodies, Monoclonal↗
PubMed1995

[Mycosis fungoides in the child. Three cases].

INTRODUCTION: Cutaneous lymphoma is unusual in children but according to data in the literature, approximated 5 p. 100 of the cases observed would begin in childhood. CASE REPORT: We retrospectively studied 3 cases of mycosis fungoides where the first manifestations occurred before 10 years of age. In one of the patients, the diagnosis was not definitively confirmed until adulthood. DISCUSSION: Diagnosis in these forms which begin in childhood is usually achieved after a long delay. Clinically, these lymphomas form a homogeneous group. In approximately 15 p. 100, guttate parapsoriasis occurs before mycosis fungoides. Biopsy is indicated if the lesions change in aspect or become atypical. The most frequent presentation in children or young adults is vitiligoid hypo-pigmented macules. Histologically, childhood forms do not differ from the adult forms and also respond to local treatment as in adults. Prospective studies conducted conjointly by paediatricians and dermatologists would be needed to describe the natural history of these cutaneous lymphomas in light of progression to aggressive lymphoma of Hodgkin's disease described in certain cases.

Antigens, CD↗
PubMed1995

[Conjugal mycosis fungoides].

INTRODUCTION: The pathophysiology of mycosis fungoides remains uncertain but HTLV I or a similar virus could be involved. We observed a couple who developed mycosis fungoides suggesting the infectious hypothesis might indeed be valid. CASE REPORT: A 70-year-old man who had often travelled in foreign countries developed parapsoriasis en plaques, lymphomatoid papulosis and mycosis fungoides successively over a thirty year period. Several years after the first manifestation of mycosis fungoides, his wife also developed a single plaque of mycosis fungoides. The diagnosis was confirmed on pathology slides and immunohistochemistry tests as well as on the basis of T-receptor gene rearrangement in both patients. Search for HTLV I was negative using serology tests and PCR on circulating lymphocytes. COMMENTS: The epidemiological situation in our observation (several trips in foreign countries and the delayed development of mycosis fungoides in the wife) favours the hypothesis of an infectious mechanism. Search for HTLV I was unsuccessful with classical virology methods. Certain recent work suggests a virus similar but different from the HTLV I virus could be involved in the pathogenesis of mycosis fungoides.

Aged↗
PubMed1996

[Health effects of plutonium pollution--assessment after the Thule disaster in 1968].

On 21 January 1968 a B-52 airplane with four nuclear weapons crashed on the ice close to the Thule air base in northern Greenland. Approximately six kilograms of plutonium was dispersed in the surroundings. Since 1968, the possible health impacts among the Danish civilian workers who assisted at the clean-up have been discussed regularly. A Nordic expert group was appointed by the Danish government to examine the evidence. The group concluded in May 1995 that no conclusive evidence of damage to the health of the worker as a result of plutonium pollution had been reported. However, they recommended continued follow-up of record linkage studies on cancer and a closer investigation of the rare skin disease parapsoriasis en plaque.

Accidents, Aviation↗
PubMed1977

Failure to induce tolerance to mechlorethamine hydrochloride.

In view of the contradictory results reported in the literature regarding induction of specific immunologic tolerance to mechlorethamine hydrochloride (HN2), the problem was reinvestigated using a "tolerogenic" schedule that had been reported to be effective. Mechlorethamine hydrochloride, 200 microgram, intravenously, was given weekly for five weeks before beginning topical therapy with it. In the test group, five of 13 patients (11 with mycosis fungoides and two with psoriasis) became contact sensitized to mechlorethamine. In another patient, what was probably a contact urticarial reaction developed. In the control group, five of 13 patients (12 with mycosis fungoides, one with parapsoriasis) became contact sensitized to mechlorethamine. Thus, 38% of the patients in both groups became contact sensitized to mechlorethamine. It is concluded that this tolerogenic schedule, just as others previously tried, was not effective in inducing specific tolerance to mechlorethamine.

Dermatitis, Contact↗
PubMed1997

Genotypic analysis of cutaneous T-cell lymphoma: a comparative study of Southern blot analysis with polymerase chain reaction amplification of the T-cell receptor-gamma gene.

The diagnosis of early cutaneous T-cell lymphoma (CTCL) is a difficult point in dermatology. Recently, Southern blot analysis (SBA) and polymerase chain reaction (PCR) have been used to detect clonality in initial lesions in which clinical and histological findings are unspecific. Forty-one samples from 25 patients with CTCL were investigated for the presence of T-cell receptor-gamma gene rearrangement using a nested PCR technique and analysed by polyacrylamide gel electrophoresis (PAGE). Conventional SBA was also performed on 28 samples from 20 of these patients. In addition, 20 samples corresponding to patients with large plaque parapsoriasis (LPP), cutaneous B-cell lymphoma (CBCL) and eczema were analysed by PCR in the same way as were the CTCL specimens. Most of the CTCL specimens (81%) showed clonality on PCR analysis. Among patients with mycosis fungoides, 71% of initial patch lesions and 100% of plaques and tumours showed clonal disease. Clonality could be detected in three of four histologically negative post-treatment lesions. Clonal rearrangement was detected in one of three patients with LPP and in three of 10 patients with CBCL. None of the samples corresponding to patients with eczema showed positive results. SBA was significantly less sensitive than PCR in detecting clonality in CTCL patients (42% among early disease and 60% among advanced cases). The results indicate that this PCR/PAGE technique is a reliable and useful method for the detection of clonality in early skin lesions of CTCL patients and probably in the identification of silent extracutaneous involvement.

Adolescent↗
PubMed1998

Recognition of Streptococcus pyogenes and skin autoantigens in guttate psoriasis.

BACKGROUND: Guttate psoriasis is associated with infections by Streptococcus pyogenes and cross-reactions between skin and streptococcal antigens have been reported, suggesting an autoimmune component in the disease. METHODS: In this work, the authors looked for antibodies against S. pyogenes M-5 antigens by immunoblot in 52 sera of psoriasis patients and in 52 sera of normal individuals. Histological and immunohistochemical analysis in skin biopsies from lesions of another group of 16 clinically diagnosed guttate psoriasis patients and four healthy controls were also carried out. RESULTS: All guttate psoriasis patients studied (11) had IgG antibodies that intensively recognized three different proteins of 70, 60 and 14 kDa, as compared to sera from patients with other forms of psoriasis or from healthy controls. The diagnosis of psoriasis was confirmed in 14 of the patients by hematoxylineosin staining. Of the other two patients, one was diagnosed as parapsoriasis and the other as liquen. By indirect immunofluorescence (IFI), all 14 psoriatic patients had autoantibodies against their own lesional skin that did not recognize normal skin from control subjects or from the two non-psoriatic patients. The parapsoriatic and the liquen patients did not have autoantibodies. A rabbit immune serum against S. pyogenes antigens reacted with lesional skin from the 14 guttate psoriatic patients, but not with normal skin from controls or with lesional skin from the 2 non-psoriatic patients. CONCLUSIONS: The recognition by immunoblot of streptococcal antigens by serum of guttate psoriasis patients, the presence of autoantibodies against their own skin, and recognition of the same skin antigens by anti-streptococcal rabbit antibodies confirm the participation of the immune system and of streptococcal infections in guttate psoriasis.

Adult↗
PubMed1999

Approach to lymphoproliferative infiltrates of the skin. The difficult lesions.

Primary cutaneous lymphomas (CLs) are the second most common group of extranodal non-Hodgkin's lymphomas. Cutaneous T-cell lymphomas (CTCLs) account for 60% to 65% of all primary CLs, whereas 20% to 25% of CLs are of B-cell origin. Besides the most common and well-known forms of CL, such as mycosis fungoides and follicular B-cell lymphomas, there are some rare and unusual variants of CL that represent about 10% of lymphoproliferative skin infiltrates. Discussed in detail are T- and B-cell pseudolymphomas as simulators of CL, the group of parapsoriasis and early mycosis fungoides, circumscribed CTCL, such as pagetoid reticulosis and syringolymphoid hyperplasia, granulomatous variants of CTCL, the group of primary CD30+ CTCL and the controversies in the classification and some rare, but distinct cutaneous B-cell lymphomas. The final diagnosis in CL is based on a constellation of clinical, histopathologic, and molecular-biologic criteria.

Humans↗