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[Lymphomatoid papulosis in a child].

We report a case of a 3-year-old boy who developed crops of papules and ulcerating nodules on the limbs in April 1992. Periodically, new lesions continue to erupt, while others resolve spontaneously. This course is characteristic for rhythmic paradoxical eruptions. This course and the clinical picture, supported by the histopathological and immunohistochemical findings, led to the diagnosis of lymphomatoid papulosis. Lymphomatoid papulosis is extremely rare in childhood. All published cases of lymphomatoid papulosis in children under 10 years of age are reviewed. The differential diagnosis of lymphomatoid papulosis in childhood includes arthropod assaults, pityriasis lichenoides et varioliformis acuta, primary cutaneous Hodgkin's disease, Ki-1 large cell anaplastic lymphoma and other lymphomas and pseudolymphomas in children.

Biopsy↗

Gene rearrangements and T-cell lymphomas.

BACKGROUND: Cutaneous T-cell lymphomas comprise a broad spectrum of neoplasia ranging from indolent to highly aggressive types. To determine subset lineage and malignant vs benign nature, morphologic analysis, immunophenotyping, and flow cytometry have been used. However, given the shortcomings of these methods, molecular genetic techniques, which take particular advantage of the clonal nature of malignancy, are now being applied to better characterize and diagnose these lymphomas. RESULTS: Each antigen-specific T cell and its clonal progeny has a unique rearrangement of its T-cell receptor gene such that it can recognize very specific antigenic epitopes. By visualizing these particular T-cell receptor gene rearrangements, Southern hybridization techniques and polymerase chain reaction amplification can detect clonal populations of T cells in the skin, blood, and lymph nodes of patients with T-cell leukemias and lymphomas. Clonal T-cell populations have also been found in cases of benign disorders such as lymphomatoid papulosis and pityriasis lichenoides et varioliformis acuta. Although these disorders usually have a benign outcome, they may represent dysplastic clonal lymphoid expansions with a high incidence of spontaneous regression. CONCLUSIONS: Molecular genetic techniques have added to our ability to diagnose, characterize, and monitor the course of T-cell lymphomas and leukemias. In addition, they may provide insight into the pathogenesis of certain benign disorders.

Cloning, Molecular↗

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↗

[Septicemia caused by Malassezia furfur].

A case of septicaemia caused by Malassezia furfur in a newborn patient receiving intravenous nutrition is presented. M. furfur, the well known cause of pityriasis versicolor, is a strict lipophilic yeast, with predilection for indwelling catheters conducting lipid solutions. Since the diagnosis of M. furfur is hampered by the slow growth and the requirement of lipid containing growth media, one must keep the organism in mind when signs of septicaemia occur in a patient receiving parenteral nutrition, especially in premature infants.

Catheterization, Central Venous↗

Epidermodysplasia verruciformis: report of a case with palmar pits.

A case of epidermodysplasia verruciformis is reported. This rare disease is characterized by a generalized eruption of flat warts and lesions resembling pityriasis versicolor. Epidermodysplasia verruciformis is considered to represent a distinctive pattern of abnormal reactivity to the human papillomavirus. Our patient, in addition to showing the classic skin lesions, exhibited pitlike lesions of the palms.

Adult↗

[Naftifin--laboratory and clinical experience with a new antimycotic from the allylamine group].

The authors evaluated the effectiveness of naftifin on a broad spectrum of 42 types of agents causing mycoses. Using the microdilution method, the authors assessed minimal inhibiting concentrations (MIC) of this antimycotic agent in 107 clinical isolates. Naftifin displayed a selective antifungal activity: excellent sensitivity was found in dermatophytes (MIC 90% = 0.39 mg.l-1), Aspergillae were medium sensitive (MIC = 0.09-12.5 mg.l-1). The majority of yeasts and filamentous fungi from the group of Zygomycetes was resistant to naftifin. The therapeutic results in 57 subjects with dermatomycoses corresponded to the results of in vitro tests of the antimycotic agent. Most successful as treatment of dermatophytoses at extra-intertriginous sites and treatment of pityriasis versicolor; manifestation of candidoses were not affected. The commercial preparation used--Fetimin cream--is considered by the authors a suitable alternative of hitherto used local antimycotics, in particular preparations from the azole series.

Allylamine↗

Ecchymotic Kaposi's sarcoma.

We report two patients with ecchymotic patches that did not suggest the diagnosis of Kaposi's sarcoma. The principal complaint of one patient was facial and periorbital edema with bilateral periorbital ecchymosis. On his trunk were patches resembling pityriasis rosea-like Kaposi's sarcoma. Both types of lesions proved to be Kaposi's sarcoma on histologic examination. The second patient had scattered ecchymotic lesions, with typical lesions of Kaposi's sarcoma elsewhere on his body. The ecchymotic lesions showed a large amount of extravasated red blood cells and no evidence of amyloid. This variant has not been described previously to our knowledge.

Adult↗

Common disorders of pigmentation: when are more than cosmetic cover-ups required?

The many types of pigmentation disorders may present in diverse forms and distributions and have various causes. They can be inherited (eg, vitiligo, familial periorbital hyperpigmentation), acquired (eg, postinflammatory pityriasis alba, idiopathic guttate hypomelanosis, Becker's nevus, melasma), infectious (eg, tinea versicolor), benign and self-limiting (eg, isolated café au lait spots, photocontact dermatitis), or a sign of more serious underlying disease (eg, multiple café au lait spots, malignant acanthosis nigricans). Primary care physicians see many patients with skin complaints and can often accomplish the early recognition and appropriate treatment that is paramount to cost-effective medicine. In many cases, an important aspect of patient care is education toward realistic expectations, because even with referral and use of extensive treatment, cosmetic results may be disappointing. Assuring patients that the disorder is not dangerous and providing tips on sunscreen and cosmetic use may be the best approach in some cases.

Humans↗

Diagnosis and treatment of tinea versicolor.

Tinea versicolor (pityriasis versicolor) is a common superficial fungal infection of the stratum corneum. Caused by the fungus Malassezia furfur, this chronically recurring disease is most prevalent in the tropics but is also common in temperate climates. Treatments are available and cure rates are high, although recurrences are common. Traditional topical agents such as selenium sulfide are effective, but recurrence following treatment with these agents is likely and often rapid. Currently, therapeutic interest is focused on synthetic "-azole" antifungal drugs, which interfere with the sterol metabolism of the infectious agent. Ketoconazole, an imidazole, has been used for years both orally and topically with great success, although it has not been approved by the Food and Drug Administration for the indication of tinea versicolor. Newer derivatives, such as fluconazole and itraconazole, have recently been introduced. Side effects associated with these triazoles tend to be minor and low in incidence. Except for ketoconazole, oral antifungals carry a low risk of hepatotoxicity.

Administration, Oral↗

[Superficial mucocutaneous mycosis].

Superficial mycosis are very common. The frequency in France is 10 to 15% of the population. The reason for the high incidence of this disease is the change of the moral values, rise in promiscuity and increase sports activities. The agents responsible are divided in four groups. Dermatophytes involve keratin of the skin, hairs and nails but never the mucous membranes. They can cause glabrous skin lesion, tinea corporis, tinea capitis or onychomycosis. Yeast are divided in two groups: the candidiasis and Malassezia furfur, the etiologic agent of pityriasis versicolor. Oral mucosa candidiasis is very common in HIV infected patients.

Candidiasis, Cutaneous↗

[Cutaneous reactions to gold salts].

Gold salts are used for rheumatoid arthritis, and also in resistant corticosteroid dermatoses such as pemphigus. Gold salts inhibit the expression of endothelial cell adhesion molecules, but activity varies from one molecule to another; thiomalate alone gives the same effect. Patients given gold salts have as high risk of cutaneous reactions, and a provisional diagnosis of "gold dermatisis" is insufficient. The mechanisms of cutaneous reactions are unknown and vary according to the molecules. Smokers, HLA Bw35 patients and perhaps atopic states are more prone to gold drug reaction. Inflammation at the site of injections is frequent but with no consequence. Accumulation (chrysiasis) may be observed with long-term treatment. The main problem is its diagnosis as it may mimic numerous dermatoses. Immunological adverse events are the most frequently encountered. Pruritus is frequently observed, more often with oral salts. Exanthemas are common and may disclose an associated visceral disease. Drug hypersensitivity is rare, but severe. All these types necessitate drug interruption although prescription has been continued after development of pityriasis rosea-like and eczematous eruptions in some series without worsening. Lichenoid eruptions require withdrawal, but the skin disease may continue. Oral presentation is frequent, either as a taste abnormality, or as stomatitis. Contact dermatitis may flare in patients sensitized to gold. Rare non-immunological skin diseases have been also observed. Careful dermatological assessment correlated with an imputability method and search of visceral side-effects could lead to a better choice for the patient. Skin tests are not reliable.

Animals↗

[Cutaneous and neurologic vasculitis disclosing EBV-selective immunodeficiency].

INTRODUCTION: Purtilo's syndrome or X-linked lymphoproliferative syndrome (XLP) is a rare genetic disorder affecting boys who have a selective immunodeficit towards Epstein Barr Virus (EBV) and who develop extremely severe forms of EBV infection, of which there are four major types: severe or fatal infectious mononucleosis (60 p. 100), lymphoma (23 p. 100), acquired hypo- or agamaglobulinemia (25 p. 100) and anemia or pancytopenia. We report a case of vasculitis (cutaneous and neurologic) which led to the discovery of a selective immunodeficit towards EBV, similar to Purtilo's syndrome. CASE REPORT: A 17 year-old male with no significant past medical history presented with an eruption initially felt to be consistent with pityriasis lichenoid. Treatment with erythromycin was initiated, this did not prevent the subsequent eruptions of cutaneous vasculitis lesions which were severe, prolonged, debilitating, and associated with fever and general deterioration of the patient condition. All etiologic studies were negative. A course of systemic corticosteroids was begun, but the cutaneous eruptions persisted; and in addition the patient developed signs of polyneuropathy in the lower extremities secondary to neurologic vasculitic lesions. New studies revealed an abnormal EBV serology (absence of anti-EBNA antibodies) as well as hypogammaglobulinemia, suggestive of a selective immunodeficit towards EBV resembling Purtilo's syndrome. DISCUSSION: In our patient, the development of an extensive vasculitis, characterized histologically by an intense lymphocytic infiltrate, positive for EBV, associated with hypogammaglobulinemia, and with abnormal serology suggests an anomaly in the immune response to EBV. Although the age of the patient and absence of family history make the Purtilo's syndrome uncertain, the nature of the immunodeficit is very similar and the patient could well develop a lymphoma. This case is significant in that the disease initially manifested itself as a cutaneous vasculitis, which was not been described previously.

Adolescent↗

Malassezia furfur: a fungus belonging to the physiological skin flora and its relevance in skin disorders.

Malassezia furfur is an anthropophilic fungus that belongs to the physiological skin flora. The fungus can grow in a yeast phase as well as in a mycelial phase; on nonaffected skin the fungus is mainly prevalent in the yeast phase. The organism has complex lipid requirements for growth, which also explains its occurrence on the skin. This also leads to the requirement for specially supplemented media for in vitro cultivation. Malassezia furfur is the causative agent of pityriasis versicolor. It also seems to be associated with seborrheic dermatitis and dandruff formation, folliculitis, confluent and reticulate papillomatosis, and the provocation of psoriatic lesions. Many substances for topical application, such as azole antimycotics, ciclopirox olamine, piroctone-olamine, zinc pyrithione, or sulfur-containing substances are effective in the treatment of these diseases. In recent years rare cases of systemic infections and fungemias caused by Malassezia have been reported.

Dermatitis, Seborrheic↗

Hidradenitis suppurativa following acanthosis nigricans. Report of two cases.

Two overweight male patients developed acanthosis nigrican of the axillae. Seven to nine months later, the patients developed hidradenitis suppurativa deep within the areas of acanthosis nigricans. On examination of biopsy specimens, the hyperkeratosis of acanthosis nigricans was found to extend into the follicles. In previous reports, Fox-Fordyce disease and pityriasis rubra pilaris have preceded the onset of hidradenitis suppurativa.

Acanthosis Nigricans↗

The effects of Malassezia on pro-inflammatory cytokine production by human peripheral blood mononuclear cells in vitro.

Malassezia spp., the causative agents of pityriasis versicolor, are members of the normal human cutaneous microflora. Utilizing a combination of both enzyme-linked immunosorbent assay (ELISA) and bioassay, we have investigated the ability of both formalin-preserved and viable Malassezia (serovars A, B and C) to modulate pro-inflammatory cytokine (IL-6, IL-1beta and TNF-alpha) release by human peripheral blood mononuclear cells (PBMNC) in vitro, over a 48-h co-incubation period. The results demonstrated that formalin-preserved Malassezia (serovars A, B and C) at mid-exponential phase were generally able to induce a pro-inflammatory cytokine response at a yeast cell to PBMNC ratio of 1:1. In addition, the results consistently demonstrated that at a yeast cell to PBMNC ratio of 20:1, formalin-preserved Malassezia, irrespective of serovar, growth phase or PBMNC donor, were capable of significantly (P<0.05) decreasing the release of both immunochemical IL-6 and IL-1beta plus bioactive IL-1beta and TNF-alpha below that of unstimulated culture medium control values. This was apparent following 24- and 48-h co-incubation times, where maximal cytokine production was detected after 24 h. Similar results were obtained for the effect of viable Malassezia on pro-inflammatory cytokine release by PBMNC. Our results suggest that a possible inhibitory component, present perhaps within the cell wall of Malassezia, was responsible for this depressive effect on pro-inflammatory cytokine production.

Biological Assay↗