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At least 19 recordsLinked to original sources
PubMed1987

Immunohistology of pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica. Evidence for their interrelationship with lymphomatoid papulosis.

Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica are idiopathic, papular eruptions that exhibit certain clinicopathologic similarities to each other and to lymphomatoid papulosis. In order to determine if these disorders are also similar immunologically, we studied the immunopathology of five biopsy specimens from three cases of pityriasis lichenoides et varioliformis acuta and three biopsy specimens from three cases of pityriasis lichenoides chronica. We then compared them to our prior immunohistologic study of nine cases of lymphomatoid papulosis. Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica both exhibited a dermal and epidermal infiltrate of CD4+ and CD8+ T cells expressing activation antigens. These were admixed with numerous macrophages. The lesional epidermis was diffusely human lymphocyte antigen (HLA)-DR+ and contained decreased CD1+ dendritic cells. Endothelial cells were also HLA-DR+. Cells bearing the phenotypes of B cells, follicular dendritic cells, or natural killer/killer cells were essentially absent. Except for the lack of large atypical cells, the results resembled those described previously for lymphomatoid papulosis. These findings indicate that pityriasis lichenoides chronica, pityriasis lichenoides et varioliformis acuta, and lymphomatoid papulosis share several immunohistologic features. Together with certain clinicopathologic similarities, they are consistent with the hypothesis that these three disorders are interrelated.

Acute Disease↗
PubMed2002

Medium-dose ultraviolet A1 therapy for pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica.

BACKGROUND: Ultraviolet A1 (340-400 nm) was found to be effective in the treatment of cutaneous T-cell-mediated diseases. OBJECTIVE: The purpose of the present study was to assess the efficacy of UVA1 phototherapy for pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC). METHODS: Eight patients (3 with PLEVA and 5 with PLC) received 60 J/cm(2) UVA1 daily until remission. Four patients also had lesions inaccessible to UVA1 that were used as control lesions. Immunocytologic studies of skin infiltrates and circulating T cells were done. RESULTS: Six patients showed complete clinical and histologic recovery. Two patients with PLC had a partial improvement. Unirradiated control lesions never improved. Serious short-term adverse effects were not encountered. No effects on circulating lymphocytes were reported. CONCLUSION: UVA1 therapy is an effective and well-tolerated treatment for PLEVA and PLC. The therapeutic activity seems to be related to direct effects on cutaneous inflammatory infiltrates because the lesions in nonexposed cutaneous areas did not respond.

Adult↗
PubMed1991

[Lichenoid pityriasis (parapsoriasis guttata) in children. Report of 17 cases].

Seventeen cases of pityriasis lichenoides diagnosed over a nine-year period in children under 15 years of age are reported. Patients with this benign disease develop papular skin lesions covered with thick, coherent scales which detach in a single piece (reminiscent of sealing wax). Pruritus is not marked. Lesions may be necrotic (Mucha Habermann's small pox-like form, n = 6) or mild (leukodermic form, n = 2). Half of the patients studied developed several episodes and total duration of the disease exceeded two years in one third of cases. Recovery occurred after one or two episodes in half the children. Scars developed in some patients with severely necrotic lesions. None of the patients developed lymphoma. All patients with lymphomatoid papulosis progressing to lymphoma reported in the literature were adults. Pathogenesis of pityriasis lichenoides remains unknown but may involve lymphocytic vasculitis. No truly effective therapy is available. However, oral macrolides can be used especially in patients with early manifestations suggesting an infectious disease. Emollients, heliotherapy and ultraviolet therapy may also be recommended.

Adolescent↗
PubMed1977

[Lichenoid pityriasis. Clinical study of 13 cases].

13 patients with Pityriasis Lichenoides are studied clinical and histologically, showing a clinical polymorphism of the lesions, mainly in the papulous, vesiculous, and necrotic ones. The data about age, sex, evolution and response to the treatment in the present study are similar to those found by other authors. Constantly, we found, a variable degree of vasculitis. In almost all the cases there was a damage of the epithelium --exoserosis and exocytosis--, as well as presence in some cases, of red cells extravasated within the epidermis. In upper dermis we found in all biopsies, divers degrees of perivascular cell infiltration mainly composed of lymphocytes and histiocytes with predominance of the last ones, in five cases. In the majority of our cases, there was a strong relationship between the clinical and the histological aspects, but in some cases, mild lesions showed an acute microscopical picture. We are of the opinion that Pityriasis Lichenoides must be considered as a different entity from Parapsoriasis. In addition, we think that PL, is a clinical picture that manifests itself as a chronic or an acute form, and both types can be seen in the disease evolution. Finally, we could not find an evident influence and a positive response to the treatment in our patients with the classical therapeutics.

Adult↗
PubMed2002

The clonal nature of pityriasis lichenoides.

BACKGROUND: Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) are benign lymphocytic infiltrates of the skin that classically present as either a recurrent papulonecrotic eruption (PLEVA) or a persistent, scaling, papular eruption (PLC). Observations of both types of lesions present on individual patients have led to speculation that both entities are related. Previous studies evaluating the DNA of biopsy specimens from patients with PLEVA and PLC revealed clonal T-cell receptor beta gene rearrangements. OBJECTIVE: To analyze and compare the T-cell populations between lesions of PLEVA and PLC. DESIGN: Retrospective and prospective analysis of patient tissue samples, classified by histologic analysis. Extracted DNA from 13 skin biopsy specimens with the diagnosis of PLC and 14 skin biopsy specimens with the diagnosis of PLEVA was analyzed by polymerase chain reaction/denaturing gradient gel electrophoresis (PCR/DGGE). SETTING: Molecular diagnostic laboratory at an academic medical center. PATIENTS: Twenty-seven tissue samples were obtained from patients with a histologic diagnosis of PLEVA or PLC. These samples were analyzed by PCR/DGGE. MAIN OUTCOME MEASURE: The presence or absence of T-cell receptor gene rearrangements on PCR/DGGE analysis corresponding to a clonal population of T cells. RESULTS: Of 14 PLEVA specimens, 8 (57%) demonstrated monoclonal T-cell receptor gene rearrangements; 1 (8%) of 13 PLC specimens showed a gene rearrangement (P =.008, Fisher exact test). CONCLUSIONS: Our results demonstrate the polyclonal nature of the lymphocytic infiltrate found in almost all of the PLC specimens, which contrasts with the monoclonal nature found in most of the PLEVA specimens. These differences may represent different stages of the clinical evolution of a single entity that results from varying host immune responses to pathogenic factors. Specifically, we propose that PLEVA is a benign clonal T-cell disorder in which the clone arises from a subset of T cells in lesions of PLC. The host immune response to this clone determines the clinical and histologic findings in PLEVA.

Adolescent↗
PubMed1997

The relation between toxoplasmosis and pityriasis lichenoides chronica.

Pityriasis lichenoides chronica (PLC) is a rare skin disease of uncertain aetiology. Many infectious agents have been incriminated as the cause of the disease. One of these agents is toxoplasmosis. The aim of this work was to find out if there is a relationship between toxoplasmosis and PLC. Twenty two patients (17 males and 5 females) diagnosed clinically and histopathologically as PLC were chosen for this study. Also twenty apparently healthy individuals free from skin lesions were included as a control group. Patients and controls were examined clinically for signs of toxoplasmosis and submitted for indirect haemagglutination (IHA) and indirect immunofluorescent antibody (IFA) tests in our Parasitology laboratory for serodiagnosis of toxoplasmosis. Toxoplasmosis was diagnosed in 8 (36.36%) and 3 (15%) in PLC patients and controls respectively by both tests. Using pyrimethamine and trisulfapyrimidine in treating PLC patients, showed subsidence of skin lesions in five patients with toxoplasmosis within two months from the beginning of therapy. The remaining patients showed no response to treatment. On conclusion, toxoplasmosis appears to play a role in the aetiology of PLC and serological tests for diagnosing toxoplasmosis should be performed in all PLC patients.

Adolescent↗
PubMed2005

The role of cytotoxic skin-homing CD8+ lymphocytes in cutaneous cytotoxic T-cell lymphoma and pityriasis lichenoides.

BACKGROUND: Pityriasis lichenoides (PL) is a rare cutaneous lymphoproliferative disorder of unknown origin. Malignant transitions of PL have been described, but are very rare. We recently observed the fatal course of a 26-year-old patient who presented with a clinical picture resembling PL but had cytotoxic CD8+ T-cell lymphoma of the skin (CxCTL). This case prompted us to reinvestigate the role of cytotoxic T lymphocytes in PL and its relationship to antiviral immunity. METHODS: Skin biopsy specimens of 11 patients with PL and two biopsy specimens of CxCTL were included. In all, 5 biopsy specimens taken from healthy skin and 5 samples of varicella-zoster virus (VZV) skin lesions were analyzed for control purposes. The inflammatory infiltrate was characterized by immunohistochemistry using monoclonal antibodies against CD3, CD4, CD8, CD20, cutaneous lymphocyte-associated antigen (CLA), CCR4, CXCR3, Granzyme B, Tia-1, and MxA. Flow cytometry was used to analyze the expression of chemokine receptors on peripheral blood mononuclear cells in CxCTL. RESULTS: The CxCTL skin lesions were dominated by a dense infiltration of CD8+ cytotoxic lymphocytes with a skin-homing CLA+ CCR4+ phenotype. PL and VZV skin lesions were also characterized by a predominantly CD8+ T cellular infiltrate with strong expression of the cytotoxic molecules Granzyme B and Tia-1 and the skin-homing molecules CLA and CCR4. Coexpression analyses confirmed that skin CLA+ CD8+ cytotoxic T cells are present in CxCTL, VZV, and PL skin lesions. Strong lesional production of the antiviral protein MxA, which is specifically induced by type I interferons, could be found in all investigated disorders. The study was based on histologic, immunohistologic, and flow cytometric analyses in a limited number of patients, because of the rareness of the investigated diseases. CONCLUSION: Our results revealed a striking similarity between the immunohistologic picture of malignant CxCTL, benign PL, and VZV skin lesions. Strong expression of the antiviral protein MxA in all disorders supports the view that a common antiviral immune response pattern leads to aberrant skin recruitment of CLA+ CCR4+ cytotoxic T lymphocytes in PL and CxCTL.

Adult↗
PubMed1988

[Lichenoid pityriasis. Immunologic study of 10 children].

Ten children clinically and histologically diagnosed as having pityriasis lichenoides (PL), have been studied by direct immunofluorescence (DIF). Circulating immune complexes (CI) have also been studied in four children. Granular deposits of IgM, located in the walls of the dermal vessels have been observed in two cases, but they have never been found at the dermo-epidermal junction. Granular deposits of C3 have been observed in three children, both in the walls of the dermal vessels and at the dermo-epidermal junction. The search for immune complexes gave negative results in all cases. The hypothesis of some authors that PL is an immune complex disease cannot be confirmed by our findings.

Antigen-Antibody Complex↗
PubMed2004

[Photochemotherapy for pityriasis lichenoides: 3 cases].

BACKGROUND: Pityriasis lichenoides is a rare cutaneous disorder of unknown origin that mainly affects children and young people. The disease consists of two variants: pityriasis lichenoides et varioliformis acuta, characterized by papular lesions evolving into necrosis and pityriasis lichenoides chronica characterized by scaly papules that may last from several months to several years. Various types of therapy have been proposed for pityriasis lichenoides, to our knockledge, photochemotherapy has never been used. We report the first 3 cases of pityriasis lichenoides treated by photochemotherapy. CASE REPORTS: Three patients, 6, 15 and 18 years old, presented respectively with a 1 month, 2 years and 3 months history of numerous, papular and necrotic lesions or squally papular lesions. Clinical appearance and histopathologic examination of biopsy were consistent with pityriasis lichenoides et varioliformis acuta (patients 1 and 3) and with pityriasis lichenoides chronica (patient 2). They had received different treatments without significant effect: topical corticosteroids, antibiotic, UVB therapy and dapsone. Acitretin associated with PUVA were dramatically effective within few weeks. DISCUSSION: Pityriasis lichenoides can be a severe scarring disease with significant social, psychological and physical consequences. In our opinion, photochemotherapy, which has never been used in pityriasis lichenoides, is effective. Therapeutic modalities have to be define.

Acitretin↗
PubMed2001

Differentiation and clonality of lesional lymphocytes in pityriasis lichenoides chronica.

BACKGROUND: Pityriasis lichenoides chronica (PLC) and pityriasis lichenoides et varioliformis acuta (PLEVA) are benign T-cell diseases that share several overlapping clinicopathologic features, leading many to believe that they exist as a spectrum rather than as single entities. Previous molecular studies have shown that PLEVA is a clonal lymphoproliferative disorder. To further characterize the immunohistologic features of PLC and to determine whether PLC demonstrates clonality, we studied 6 cases of PLC using a frozen section-immunoperoxidase technique and polymerase chain reaction/denaturing gradient gel electrophoresis. OBSERVATIONS: All 6 cases showed a mild to moderate superficial and deep perivascular infiltrate composed predominantly of CD4(+) T cells, admixed with Langerhans cells and macrophages; most were associated with an HLA-DR(+) epidermis. Three of 6 cases involved monoclonal T-cell receptor gamma (TCR gamma) gene rearrangements detected by V gamma 1-8/J gamma 1-2 and V gamma 9/J gamma 1-2 primers. CONCLUSIONS: Our findings enhance existing data showing that PLC shares many immunohistologic features with PLEVA and indicating that PLC is frequently a clonal T-cell disease. This provides further evidence that PLC and PLEVA are interrelated processes within the larger group of T-cell lymphoproliferative disorders.

Adult↗
PubMed2006

Pityriasis lichenoides and its subtypes.

Pityriasis lichenoides represents a unique group of inflammatory skin disorders that include pityriasis lichenoides et varioliformis acuta (PLEVA), febrile ulceronecrotic Mucha-Habermann disease (a subtype of PLEVA), and pityriasis lichenoides chronica. The history, epidemiology, clinical features, pathophysiology, and treatment of this group of conditions are reviewed in this manuscript.

Humans↗
PubMed2003

Infectious causes of pityriasis lichenoides: a case of fulminant infectious mononucleosis.

Pityriasis lichenoides is a rare cutaneous eruption of unknown cause that spans a spectrum of clinical severity. Infectious agents have long been suspected as etiologic factors. The present case is the first to demonstrate a known EBV-mediated process evolving and resolving in concert with pityriasis lichenoides. Epstein-Barr virus, Toxoplasma gondii, and HIV are the most frequently reported infectious triggers of pityriasis lichenoides. Pityriasis lichenoides may arise secondary to EBV-mediated acute infectious mononucleosis.

Adult↗
PubMed1992

Pityriasis lichenoides and lymphomatoid papulosis.

The clinical features, histopathology, immunopathology, and management of pityriasis lichenoides and lymphomatoid papulosis are discussed, with particular emphasis on the pediatric aspects of these conditions. The difficulties in logically separating pityriasis lichenoides into an acute (pityriasis lichenoides et varioliformis acuta) and a chronic (pityriasis lichenoides chronical) form are addressed. The development of lymphoreticular malignancy in patients with lymphomatoid papulosis has been well documented, but pityriasis lichenoides has characteristically been regarded as a benign condition. However, recent reports of the development of large plaque parapsoriasis in patients with pityriasis lichenoides have led to a reconsideration. Some of these patients were in the pediatric age group. Although there are significant clinical, histopathological, and immunopathological differences between pityriasis lichenoides and lymphomatoid papulosis, the demonstration of similar clonal T cell receptor gene rearrangements and the confirmation of the potentially premalignant nature of both suggests that there may indeed be an interrelationship between these two controversial entities. Close follow-up of patients with both of these conditions is recommended, with observation being discontinued only when the patient has been free of lesions for several years.

Child↗
PubMed2001

Pityriasis lichenoides et varioliformis acuta in pregnancy.

Pityriasis lichenoides et varioliformis acuta is an uncommon disease, especially during pregnancy. In review of the obstetric literature, there was no report of pityriasis lichenoides et varioliformis acuta during pregnancy. A 25-year-old female was seen at 24 weeks of gestation for consultation about a cutaneous disease. She was admitted at 30 weeks of gestation because of threatened premature labor, and some active cutaneous papules presented themselves at that time. After the treatment, cutaneous papules remitted. But at 35 weeks of gestation, she had spontaneous labor. Both the mother and infant were doing well at 5 months postpartum. If pityriasis lichenoides et varioliformis acuta exists in the vagina or cervical bone of the uterus, it is due to infections from lymphatic vasculitis and necrosis. It may cause threatened premature labor and premature rupture of the membrane.

Adult↗
PubMed2002

Cytotoxic mycosis fungoides evolving from pityriasis lichenoides chronica in a seventeen-year-old girl. Report of a case.

Pityriasis lichenoides chronica and its acute form, pityriasis lichenoides et varioliformis acuta, are skin diseases of unknown origin, that probably represent a hypersensitivity reaction to an infective agent. Pityriasis lichenoides is often a benign disorder but, because of the presence of a clonal T cell population detected both in the chronic and acute forms, some authors have suggested that it may belong to the group of primary cutaneous T cell lymphomas. Although various studies have clearly documented no significant association between pityriasis lichenoides and malignant lymphomas, cases of long-standing pityriasis lichenoides evolving into mycosis fungoides have been described. Herein we report the case of a girl suffering from pityriasis lichenoides since the age of 11 years, subsequently developing a CD45RO+, CD8+, TIA-1+ mycosis fungoides.

Adolescent↗
PubMedDate not supplied

Pityriasis lichenoides and idiopathic thrombocytopenic purpura in a young girl.

Pityriasis lichenoides is an inflammatory skin disorder characterized by erythematous, desquamative papules and plaques. An acute form, pityriasis lichenoides et varioliformis acuta, and a chronic form, pityriasis lichenoides chronica, represent the two ends of the spectrum of this disorder. Most commonly seen in children and young adults, its etiology is unknown. We describe a young patient with concurrent pityriasis lichenoides and idiopathic thrombocytopenia purpura, a previously unreported association.

Biopsy, Needle↗
PubMed2006

UVB therapy of pityriasis lichenoides--our experience with 29 patients.

BACKGROUND: Pityriasis lichenoides comprises a clinical and pathological spectrum of disorders. So far no highly effective treatment has been reported. Previous small studies have suggested that ultraviolet B (UVB) is a good alternative. METHODS: This is a retrospective analysis of 29 pityriasis lichenoides patients treated in our institution with broad- or narrow-band UVB during the period 1996-2002. Twenty-one of these patients had one or more previous unsuccessful treatments. RESULTS: Complete response was achieved in 93.1% in both treatment groups, with 73% of them still relapse free after a mean follow-up of 58 and 38 months in broad- and narrow-band UVB treatment groups, respectively. Mild side-effects were observed in about one-third of the patients. CONCLUSION: We believe both forms of UVB are a good option for pityriasis lichenoides and should be considered as the first line in generalized cases interested in treatment.

Adult↗