Pitiriasis liquenoide varioliforme aguda posterior al inicio de eritropoyetina / Acute varioliform lichenoid pityriasis following the initiation of erythropoietin

Resumen La pitiriasis liquenoide varioliforme aguda (PLEVA) pertenece a un grupo de enfermedades categorizadas como pitiriasis liquenoides, junto con la enfermedad febril úlcero-necrótica de Mucha-Habermann y la pitiriasis liquenoide crónica (PLC). Se caracteriza por la aparición aguda de múltiples pápulas eritemato-violáceas con posterior necrosis, discromía residual y cicatrices varioliformes. Dentro de las teorías patogénicas propuestas se encuentra el posible papel de agentes infecciosos, trastornos linfoproliferativos, complejos inmunes e incluso, asociación a medicamentos. Se presenta un casode una mujer adulta con un cuadro típico de PLEVA con confirmación histopatológica, cuyas lesiones aparecieron posteriormente al inicio de eritropoyetina.

Abstract Pityriasis lichenoides et varioliformes acuta (PLEVA) is part of a group of diseases clustered as pityriasis lichenoides, next to febrile ulceronecrotic Mucha-Habermann disease and pityriasis lichenoides chronica. It's characterized by a sudden onset of multiple erythematous and violaceous papules which develop necrosis, leaving residual dyschromia and varioliform scars. It's been hypothesized the possible role of infectious agents, lymphoproliferative diseases, immune complexes and drugs. We present the case of a woman with a typical PLEVA with histopathological confirmation, whose lesions appeared after therapy with erythropoietin.

Clinicopathological analysis of 81 cases of parapsoriasis / 中华皮肤科杂志

Objective@#To analyze clinicopathological features of parapsoriasis.@*Methods@#Clinical and pathological data were collected from 81 patients with parapsoriasis in Department of Dermatology, Peking University First Hospital between January 2016 and May 2018, and analyzed retrospectively.@*Results@#Among the 81 patients with parapsoriasis, 44 were male and 37 were female, with age ranging from 6 to 77 years. Their disease course ranged from 7 days to 30 years, and the median disease course was 12 months. Moreover, 61 (75.3%) patients were aged less than 40 years, and 20 (24.7%) were aged 41 years and older. Of the 81 patients, 16 (19.8%) were diagnosed with small plaque parapsoriasis, 20 (24.7%) with large plaque parapsoriasis, 37 (45.7%) with pityriasis lichenoides chronica, and 8 (9.9%) with pityriasis lichenoides et varioliformis acuta. Additionally, distribution patterns of lesions included diffuse type in 65 cases (80.2%) , central type in 6 cases (7.4%) , and peripheral type in 10 cases (12.3%) . Histopathological examination of skin lesions revealed liquefaction degeneration of basal cells in 69 cases (85.2%) , migration of lymphocytes into the epidermis in 67 cases (82.7%) , focal parakeratosis in 42 cases (51.9%) , keratinocyte necrosis in 29 cases (35.8%) , extravasation of erythrocytes in 23 cases (28.4%) , epidermal spongiosis in 21 cases (25.9%) , and dermal perivascular focal infiltration in 61 cases (75.3%) .@*Conclusion@#Parapsoriasis has characteristic clinical and pathological manifestations, and a close combination of clinical manifestations with pathological features is necessary for its accurate diagnosis.

Detection of TCRγ gene rearrangements in skin lesions and peripheral blood of patients with parapsoriasis ;and their clinical significance / 中华皮肤科杂志

Objective To detect TCRγ gene rearrangements in skin lesions and peripheral blood of patients with parapsoriasis, and to study their clinical significance. Methods Totally, 20 patients with parapsoriasis were included in this study. BIOMED?2 multiplex PCR was performed to detect TCRγgene rearrangements in lesional skin (n=20)and peripheral blood(n=11)samples from the patients with parapsoriasis. Statistical analysis was performed to assess the relationship of TCRγ gene rearrangements with clinical types of parapsoriasis as well as general information and histopathological manifestations(including non?specific manifestations and atypical manifestations)of patients. Results TCRγ gene rearrangements were positive in lesional skin from 7 of the 20 patients, in peripheral blood from 3 of 11 patients, and in both lesional skin and peripheral blood from 2 patients. Positive TCRγ gene rearrangements in skin lesions were significantly correlated with mycosis fungoides(MF)?related atypical histopatho?logical manifestations(P0.05). During an average follow?up time of 44.85 ± 18.48 months, 1 case progressed into MF, and 2 were cured. Conclusions Positive TCRγgene rearrangements in skin lesions of patients with parapsoriasis may be correlated with MF?related atypical manifestations. The presence of TCRγgene rearrangements and atypical histopathological manifestations may suggest the possibility of progression from parapsoriasis into MF.

Pitiriasis liquenoide: presentación de un caso clínico y revisión de la literatura / Pityriasis Lichenoides: Case report and review of the literature

Introducción: La pitiriasis liquenoide es una enfermedad inflamatoria benigna de causa desconocida. Tiene dos formas de presentación: una forma aguda (PLEVA, pitiriasis liquenoide y varioliforme aguda) y otra crónica (PLC, pitiriasis liquenoide crónica). Ambas son más frecuentes en niños y adultos jóvenes. Objetivo: Describir un caso de PLC, comentar su presentación clínica, diagnóstico y tratamiento, y revisar la literatura. Caso clínico: Escolar de 7 años que presentó episodios recurrentes de lesiones tipo pápulas eritematocostrosas brillantes de distribución centrípeta, oligosintomáticas, que desaparecían dejando máculas hipopigmentadas. La biopsia de las lesiones confirmó una pitiriasis liquenoide crónica. Durante los 3 años de seguimiento se realizaron múltiples esquemas de tratamiento para atenuar las reagudizaciones, logrando una respuesta parcial. Conclusión: La PLC es una enfermedad infrecuente que representa un desafío diagnóstico y terapéutico para el médico. El diagnóstico de esta entidad se sospecha por la clínica y se confirma con la histología. No tiene tratamiento específico pero presenta buena respuesta a corticoides, antibióticos, inmunosupresores y fototerapia UVB de banda angosta (UVB-nb). Esta última es la que ha reportado los mejores resultados. Es importante el seguimiento de los pacientes por el riesgo de desarrollar enfermedades linfoproliferativas.

Introduction: Pityriasis lichenoides is a benign inflammatory disease of unknown etiology. There are two types of this condition: an acute form (PLEVA = pityriasis lichenoides et varioliformis acuta) and a chronic one (PLC = pityriasis lichenoid chronica). Both are more common in children and young adults. Objective: To describe a case of PLC, discuss its clinical presentation, diagnosis, treatment and present a review of the literature. Case Report: A seven-year-old child who presented with recurrent oligosymptomatic episodes of bright erythematous papular lesions in centripetal distribution, which subsided and left behind hypopigmented macules. The biopsy of the lesions confirmed chronic pityriasis lichenoid. During the 3 years of follow-up, multiple treatment regimens were used to reduce exacerbations, resulting in a partial response. Conclusion: PLC is a rare disease that represents a diagnostic and therapeutic challenge to the physician. The diagnosis of this condition is suspected clinically and confirmed by histology. It does not have specific treatment, but it responds well to corticosteroids, antibiotics, immunosuppressants and phototherapy with UVB narrowband (UVBnb). The latter has given the best results. It is important to monitor patients for risk of developing lymphoproliferative disorders.

The conundrum of parapsoriasis versus patch stage of mycosis fungoides.

Terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it difficult to diagnose mycosis fungoides in the early patch stage. Early diagnosis of mycosis fungoides (MF) is important for deciding on type of therapy, prognosis and for further follow-up. However, until recently, there has been no consensus on criteria that would help in diagnosing the disease early. Some believe that large plaque parapsoriasis (LPP) should be classified with early patch stage of MF and should be treated aggressively. However, there is no firm clinical or laboratory criteria to predict which LPP will progress to MF and we can only discuss about statistical probability. Moreover, long-term outcome analysis of even patch stage of MF is similar to that of control population. We therefore believe that LPP should be considered as a separate entity at least to prevent the patient from being given a frightening diagnosis. We also feel that patients need not be treated with aggressive therapy for LPP and will need only a close follow-up. This article emphasizes the criteria for diagnosing early MF and has highlighted the importance of considering LPP as a distinct benign entity.

Varón joven con lesiones eritemato-escamosas de larga evolución / Young male with long standing erythemato-squamous lesions

La parapsoriasis agrupa un número de estados patológicos caracterizados por lesiones eritemato-escamosas recalcitrantes en piel que no llenan los criterios de malignidad, y reflejan situaciones intermedias. Presentamos el caso de un varón joven con lesiones eritemato-escamosas de largo tiempo de evolución sin alteraciones del estado general y sin compromiso visceral. Hacemos una breve revisión clínica de la enfermedad y sus hallazgos histopatológicos.

Parapsoriasis brings together a number of pathological conditions characterized by erythemato-squamous skin lesions that do not meet the criteria for malignancy, and reflect intermediate situations. We present the case of a young male with erythemato-squamous lesions of long time of evolution, without alterations of the general statement and without visceral compromise. We make a brief review of the clinical disease and its pathological findings.

A Clinical and Histopathological Study of Parapsoriasis / 대한피부과학회지

BACKGROUND: Parapsoriasis described a group of cutaneous disease that can be characterized by scaly patches or slightly elevated plaques that have a resemblance to psoriasis, hence the nomenclature. Up to now, a uniformly accepted definition of parapsoriasis remains lacking. OBJECTIVE: The purpose of this study was to evaluate the clinical and histopathological features of parapsoriasis in Korea. METHODS: The clinical charts, photographs and skin biopsy slides of patients who had been clinically diagnosed as parapsoriasis at the initial visit were reviewed. Then the evaluation of age at onset, sex distribution, disease duration, anatomical distribution, clinical features of the skin lesions, associated symptoms, family history, co-existing disease, and histopathologic features were conducted. Six cases from small plaque parapsoriasis group and four cases from large plaque parapsoriasis group were investigated for the presence of TCR gene rearrangement using a PCR technique. RESULTS: The male to female ratio was 1: 1.2. The average ages of onset in large plaque type and small plaque type were 44.7 and 32.9 years respectively. 1) According to the clinical classification, 7 (29%) of the patients had large plaque type and 17 (71%) had small plaque type. 2) Many lesions of large plaque parapsoriasis group exhibited varying degrees of epidermal atrophy, fine wrinkles and telangiectasia. 3) Large plaque parapsoriasis showed more histologic changes consisting of epidermal atrophy, exocytosis and atypical lymphocytes. 4) Monoclonal T-cell receptor gene rearrangement was not detected in all of 10 patients with large and small plaque parapsoriasis. CONCLUSION: In our study, some of our findings are in agreement with those already in the literature, whereas others, particularly the male to female ratio and constitutional symptoms, differ. Our results also indicate that there are many clinical and pathological differences between large plaque and small plaque parapsoriasis.

A comparative clinicopathological study of pityriasis lichenoides chronica and small plaque parapsoriasis / 대한피부과학회지

A clinicopathological study of 12 cases of pityriasis lichenoides chronica and 14 cases of small plaque parapsoriasis was performed. Patients with pityriasis lichenoides chranica ranged in age from 15 to 62 years (mean, 30 years) with male predominance. They had erythematous or reddish scaly papules occuring mostly on both trunk and extremities(92%). The lesions were about the size of r ice grains or small peas. Patients with small plaque parapsoriasis ranged in age from 8 to 67 years(mean, 33 years) and presented yellowish or erythematous scaly maculies and patches comrnonly involving the trunk and extremities(64%). The size of the lesions were larger than those of pityriasis lichenoides chronica. Histopatbologically, the distinct feature of pityriasis lichenoides chronica was vacuolar alteration at the dermoepidermal junction characterizing interface dermatitis. The microscopic findings of small plaque parapsoriasis were, although not as distinct as in pityriasis lichenoides chronica, those af either spongiotic dermatitis or superfirial peri vascula dermatitis. We conclude from this study that these two conditions have different clinical and histologic features and we propose that the term guttate parapsoriasis shauld not be used as a synonym of either pityriasis lichenoides rhronica or small plaque parapsoriasis.