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Background
Pityriasis lichenoides has an acute and chronic phase. The acute form is called Pityriasis lichenoides et varioliformis acuta (PLEVA) or Mucha-Habermann disease. The chronic form is usually designated as pityriasis licehnoides chronica. These two diseases from a spectrum of a self-limited dermatosis with the acute form starting as a maculopapular, erythematous eruption which heals to form superficial variable scars. The lesions occur in crops over several weeks and may continue for months to years. The chronic form is more scaly and less hemorrhagic. There is a predilection for males in the second and third decades. They are most common on the anterior trunk and flexor surfaces of the proximal portions of the extremities.
It is debatable whether PLEVA can transform into a cutaneous T-cell lymphoma although there are cases reports as discussed in the outline below.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS PLEVA
Mucha-Habermann DiseaseAGE RANGE-MEDIAN PLEVA usually affects young individuals in 2-3rd decades CHILDHOOD
Department of Dermatology, Northwestern University's Feinberg School of Medicine, Chicago, IL 60611-2941, USA.
BACKGROUND: Pityriasis lichenoides (PL) occurs in all age groups, although predominantly in younger individuals.
OBJECTIVE: We sought to study the clinical features of PL in children followed up at our institution.
METHODS: The records of 124 children who were given the diagnosis of PL at our institution between 1993 and 2003 were retrospectively reviewed.
RESULTS: PL chronica (PLC) was recorded in 37% of the cases, PL et varioliformis acuta (PLEVA) in 57.3%, and clinical features of both disorders were seen simultaneously in the remaining. The median age of onset was 60 months (range: 6-180 months), although the median age of onset of PLEVA (median: 60 months) was significantly younger than that of PLC (median: 72 months) (P = .03). The age distribution showed peaks at 2 to 3 years (24.8%) and 5 to 7 years (32%). A history of infection or drug intake preceded the skin manifestations in 30% and 11.2% of patients with PLC and PLEVA, respectively. The disease began most commonly during winter (35%) or fall (30%). The median duration was 20 months (range: 3-132 months) in patients with PLC and 18 months (range: 4-108 months) in patients with PLEVA. Involvement was diffuse in 74.2% of the patients, peripheral in 20.2%, and central in the remainder. The disease was recurrent in 77% of the patients (n = 80). Of the patients, 59% had pruritus, whereas 32% reported no symptoms; the remainder had fever, arthralgia, or both. Erythromycin estolate or ethylsuccinate was administered to 79.7% of the affected children; 66.6% of these showed at least a partial response.
LIMITATIONS: The analyzed data were collected retrospectively and biopsies were not performed in all patients.
CONCLUSIONS: PL is not an uncommon disease in childhood, with age peaks in the preschool and early school-age years. It is usually recurrent, and shows a seasonal variation with onset most often in the fall or winter. In childhood PL, erythromycin is an effective initial treatment choice.
PATHOGENESIS CHARACTERIZATION CLONALITY Arch Dermatol 2000;136:1483-1486
Heteroduplex analysis of 20 cases on paraffin embedded tissue13/20 cases (65%) revealed the presence of a dominant T-cell clone
This suggests that:
PLEVA may be cutaneous lymphoproliferative disorder
May be closely related to LyP OR
Dominant T-cell clone represents a clonal immunological response to an unknown antigen or infectious agentIn a follow-up of 2 1/2 years, there was no evidence of a lymphoid malignancy
Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University New York Presbyterian Hospital-Cornell Campus, New York, NY 10021, USA.
Pityriasis lichenoides (PL) has traditionally been classified as a benign papulosquamous disease. However, there is an increasing literature precedent that suggests that PL should instead be considered a form of cutaneous lymphoid dyscrasia.
We prospectively encountered 46 patients with a diagnosis of PL and used immunohistochemical and multiplex polymerase chain reaction fragment size analysis to assess for phenotypic abnormalities and for T-cell clonal restriction, respectively.
We categorized them into 2 groups based on the molecular profile, namely, those cases that showed a monoclonal and/or a restricted oligoclonal profile versus those cases that were polyclonal. Half of all the cases studied showed a monoclonal and/or an oligoclonal restricted T-cell repertoire. From a clinical perspective, 2 cases in this group manifested skin lesions compatible with mycosis fungoides (MF). All of the other cases demonstrated a persistent but nonprogressive clinical course characterized by periods of regression and recurrence. In any case in which there were multiple biopsies, the same T-cell dominant clonotypes, be it in the context of representing a true monoclonal and/or oligoclonal pattern, were implicated over time and at different biopsy sites, including 2 cases in which there was a subsequent evolution to MF. Substantial losses of CD7 and CD62L were seen in both monoclonal/oligoclonal and polyclonal cases of PL, although both values of percentage reduction were greater in the monoclonal/oligoclonal cases. A dominance of CD8 lymphocytes was seen in more than half of all the cases of PL and held to be reactive in nature, potentially directed against clonally restricted CD4 cells. CD4/CD25+ (Foxp3+) T cells averaged 24% in the polyclonal cases; it was 12% in the monoclonal variants of PL.
We conclude that PL is a form of indolent cutaneous T-cell dyscrasia. The limited propensity for progression to MF may reflect internal countercheck mechanisms of controlling clonally restricted CD4+ T-cell proliferations via CD8 and CD4/CD25+ regulatory T cells.Differentiation and Clonality of Lesional Lymphocytes in Pityriasis Lichenoides Chronica Arch Dermatol. 2001;137:305-308 Abstract quote
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) gene rearrangements detected by V1-8/J1-2 and V9/J1-2 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.
The clonal nature of pityriasis lichenoides.Weinberg JM, Kristal L, Chooback L, Honig PJ, Kramer EM, Lessin SR.
Department of Dermatology, St Luke's-Roosevelt Hospital Center, 1090 Amsterdam Ave, Suite 11D, New York, NY 10025.
Arch Dermatol 2002 Aug;138(8):1063-7 Abstract quote 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.
Pityriasis lichenoides: a clonal T-cell lymphoproliferative disorder.Magro C, Crowson AN, Kovatich A, Burns F.
Department of Pathology Ohio State University Medical Center, Columbus, OH 43210, USA.
Hum Pathol 2002 Aug;33(8):788-95 Abstract quote Pityriasis lichenoides (PL) is a papulosquamous disorder often considered a form of reactive dermatosis and classified with small plaque parapsoriasis (digitate dermatosis). However, some patients with PL have developed large plaque parapsoriasis (LPP) and mycosis fungoides (MF), and lymphoid atypia and T-cell clonality have been reported in lesions of PL.
We set out to explore the possibility that PL is a form of T-cell dyscrasia. Cases were selected by natural language search from an outpatient dermatopathology database; 35 cases were reviewed and clinicians and patients were contacted. Hematoxylin and eosin-stained sections were examined and immunophenotyping was carried out on paraffin-embedded, formalin-fixed tissue using antibodies to CD2, CD3, CD4, CD5, CD7, CD8, CD20, CD30, and CD56. In paraffin-embedded tissue, T-cell receptor (TCR)-gamma chain rearrangement was sought through polymerase chain reaction single stranded conformational polymorphism analysis. There were 14 males and 21 females with a mean age of 40 years held clinically to have PL chronica (PLC) (28 cases) and/or PL et varioliformis acuta (PLEVA) (7 cases). Five patients developed large atrophic poikilodermatous and/or annular plaques compatible with MF and/or LPP in a background of typical PLC. All biopsies showed tropism of lymphocytes to an epidermis manifesting psoriasiform hyperplasia, dyskeratosis, parakeratosis, and intraepithelial collections of Langerhans' cells and lymphocytes mimicking Pautrier's microabascesses. Epidermal atrophy, dermal fibroplasia, poikilodermatous alterations, and a dominance of intraepidermal cerebriform cells were seen only in patients with chronic persistent disease (i.e., PLC) and in some cases corresponded with clinical progression to MF. All cases had a T cell-dominant infiltrate, with a CD7 deletion in 21 of 32 biopsies examined; the CD7-negative cells were typically the largest and most atypical forms, often in a cohesive array within the upper layers of the epidermis. In 17 biopsies in which a CD4 stain was satisfactory for evaluation, 50% or more of the intraepidermal population was CD4 positive in 8 biopsies, whereas in 11 biopsies 50% or more of the dermal infiltrate was CD4 positive. The CD4-positive cells frequently had a cerebriform nuclear morphology and were CD7 negative. Most cases had an admixture of CD8-positive lymphocytes in excess of 40% or more of the intraepidermal and/or dermal infiltrate; it was the dominant intraepidermal infiltrate in 10 cases. The CD8-positive cells, typically small, round, and CD7 positive, showed a directed pattern of migration into acrosyringia and suprapapillary plates, with satellitosis around CD4-positive/CD8-negative/CD7-negative atypical lymphocytes. CD56 positivity was seen among the intraepidermal lymphoid cells and roughly paralleled the CD8 profile.
In general, CD8-positive lymphocytes dominated in cases of PLEVA, whereas CD4-positive lymphocytes were very conspicuous and composed the dominant intraepidermal populace only in those biopsies of progressive PL/PLC. Clonality was shown in 25 of 27 biopsies in which amplifiable DNA was obtained. Intraepithelial atypical lymphocytes, phenotypic abnormalities, and TCR-gamma rearrangements suggest that PLC and PLEVA are a form of T-cell dyscrasia. Lesions may follow a recalcitrant course characteristic of MF and premycotic disorders such as LPP. The aberrant phenotype cell is similar to that defining MF: a CD4-positive T lymphocyte with a CD5 and CD7 deletion. Directed epidermal migration seen in biopsies procured from incipient lesions along with occasional temporal association to viral or drug exposure suggests that an abnormal immune response to an antigenic trigger may be the inciting event.
PARVOVIRUS B19 Pityriasis lichenoides: a cytotoxic T-cell-mediated skin disorder. Evidence of human parvovirus B19 DNA in nine cases.
Tomasini D, Tomasini CF, Cerri A, Sangalli G, Palmedo G, Hantschke M, Kutzner H.
Department of Dermatology, Hospital of Busto Arsizio, Busto Arsizio, Italy.
J Cutan Pathol. 2004 Sep;31(8):531-8. Abstract quote
Background: Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) probably represent the polar ends of the same pathologic process, i.e. pityriasis lichenoides (PL), with intermediate forms in between. Previous studies have demonstrated that the inflammatory infiltrate in PLEVA is composed of cytotoxic suppressor T cells, whereas in PLC the helper/inducer T-cell population drives the immunological answer. Furthermore, monoclonal rearrangement of the T-cell receptor-gamma (TCR-gamma) genes was repeatedly found both in PLEVA and PLC.
Methods: Forty-one formalin-fixed, paraffin-embedded tissue specimens of 40 cases of PL were retrieved from the files of the authors. Immunophenotyping for cytotoxic granular proteins (Tia-1/GMP-17 and Granzyme B) and T-cell-related antigens (n = 41), TCR-gamma chain gene analysis (n = 30) and molecular investigations for parvovirus B19 (PVB19) DNA (n = 30) were performed.
Results: Overlapping immunophenotypes were observed in PLEVA and PLC. The dermal and epidermal T cells predominantly expressed CD2, CD3, CD8, and Tia-1 with a variable positivity for CD45RA, CD45RO, and Granzyme B. A monoclonal rearrangement pattern of the TCR-gamma genes was detected in three cases (10%). PVB19 DNA was found in nine cases (30%). T-cell monoclonality in conjunction with genomic PVB19 DNA was present in one case.
Conclusions: Our results demonstrate that PL is a skin disorder mediated by the effector cytotoxic T-cell population. The identification of PVB19 DNA in nine cases may be interpreted ambiguously: PVB19 as a true pathogen or as an innocent bystander.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION General
Minneapolis Veteran's Affairs Medical Center, USA.
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.VARIANTS Clinical and histologic features of pityriasis lichenoides et varioliformis acuta in children.
Longley J, Demar L, Feinstein RP, Miller RL, Silvers DN.
Department of Pathology, College of Physicians and Surgeons of Columbia University, New York.
Arch Dermatol 1987 Oct;123(10):1335-9 Abstract quote
Pityriasis lichenoides et varioliformis acuta (PLEVA) is commonly thought of as a disease of young adults, yet we identified five cases, involving patients who were 3, 5, 6, 8, and 11 years of age, among 13,000 consecutive specimens submitted to a general dermatopathology laboratory during a 15-week period.
The clinical and histologic features of PLEVA in our cases were similar to those reported for adults, except that no lesions were observed on the scalp or mucous membranes of children.
A high index of suspicion and biopsy specimens of suspected lesions are often needed to differentiate PLEVA from other papular and crusted eruptions seen in the pediatric age group. These include reactions to arthropods, Gianotti-Crosti syndrome, varicella, and erythema multiforme. Histologically, papular eczema and pityriasis rosea may be misdiagnosed as PLEVA.
Pityriasis lichenoides in children: clinicopathologic review of 22 patients.
Romani J, Puig L, Fernandez-Figueras MT, de Moragas JM.
Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Pediatr Dermatol 1998 Jan-Feb;15(1):1-6 Abstract quote
Pityriasis lichenoides (PL) is a cutaneous disease of unknown origin, with an autoinvolutive course, that can occur in pediatric patients. Traditionally, acute and chronic variants have been described, but other special forms of presentation have been reported.
We reviewed the clinical records and histopathologic specimens of all pediatric patients diagnosed with PL in our hospital from 1980 to 1995 to assess the clinicopathologic features of this disorder in our environment. Twenty-two of the 118 cases reviewed were pediatric patients less than 15 years old (12 males and 10 females, 18.6% of all patients). Their ages ranged from 3 to 15 years, with a mean of 9.3 years. Most of the patients (72%) had the chronic variant of the disease, while the remainder had an acute course. One patient suffered from acute ulceronecrotic PL. Systemic treatments prescribed were erythromycin in eight patients, PUVA in five patients, and methotrexate in one patient. Three patients had a prolonged course with more than two episodes. Acute and chronic PL are polar extremes, but individual cases cannot be classified only on the basis of histopathologic data, since coexistence of lesions in different stages of evolution can lead to sampling bias.
Acute ulceronecrotic forms and the presence of a variable degree of cellular atypia in the infiltrate are liable to cause differential diagnostic problems with lymphomatoid papulosis (LP), which cannot be completely resolved on the basis of T-cell receptor clonal rearrangement detection.
Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta. Dermatology 1994; 189 Suppl 2:50-53
An unusually severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) with a fatal outcome
82-year-old woman
After a period of a mild eruption, extensive polymorphous, papular and ulcerohemorrhagic skin lesions developed, associated with intermittent high temperature and constitutional symptomsDead of disease
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