Role of streptococcal infection in the etiopathogenesis of pityriasis lichenoides chronica and the therapeutic efficacy of azithromycin: a randomized controlled trial.

The exact aetiology of pityriasis lichenoides chronica (PLC) remains unknown. While phototherapy is the most investigated therapeutic modality, azithromycin has been used scarcely. The aim of this study is to evaluate the therapeutic efficacy of azithromycin in the treatment of PLC compared to NB-UVB and evaluating the presence of streptococcal infection as a possible etiological factor in PLC patients. The study was designed as a randomised controlled trial. Twenty-four patients with PLC were randomly allocated into either azithromycin (n = 13, standard dose every 10 days) or NB-UVB (n = 11, thrice weekly) groups. End of study (EOS) was either complete clearance of lesions or a maximum of 8 weeks. Therapeutic efficacy was defined as percent reduction in lesions and was calculated for the rash as a whole, erythematous papules alone, and hypopigmented lesions alone and graded into complete, very-good, good, poor or no response. Anti-streptolysin O titre (ASOT), anti-deoxyribonuclease B titre (anti-DNaseB) and throat culture were evaluated at day 0. No significant difference existed between both groups as regards therapeutic efficacy. At EOS, NB-UVB achieved significantly more percent reduction in the extent of hypopigmented lesions and consequently in the rash as a whole (p = 0.001, p = 0.034, respectively). The extent of the rash as a whole was significantly less in the NB-UVB at EOS (p = 0.029, respectively). The effect of NB-UVB on hypopigmented lesions appeared early at week 4 of treatment. Only two patients, one from each group, relapsed during the 3 month follow-up. Evidence of recent streptococcal infection was present in 79% of the cases, mainly in the form of elevated ASOT (94.7%). It was significantly more encountered in young children (< 13 years) (p = 0.03) and was associated with more extent of erythematous papules and consequently with more extent of the rash as a whole (p = 0.05 and p = 0.01, respectively). It did not affect outcome of therapy at EOS. Azithromycin did not show more favorable response in patients with recent streptococcal infection. Therapeutic efficacy of azithromycin is comparable to NB-UVB in treatment of PLC; however, NB-UVB is superior in management of hypopigmented lesions. It is highly suggested that PLC could be a post streptococcal immune mediated disorder.Registration number: ClinicalTrials.gov, NCT03831269.

Lymphomatoid Papulosis and Other Lymphoma-Like Diseases.

Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica are the 2 main subtypes of pityriasis lichenoides. They represent the acute and chronic forms of the disease; both may have clonal T cells. Several treatment modalities are used, but it has been difficult to determine efficacy because of the possibility of spontaneous remission. Cutaneous CD30+ lymphoproliferative disorders constitute many cutaneous T-cell lymphomas and comprise lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma (ALCL). Both have an excellent prognosis. Lymphomatoid papulosis often only requires observation or treatment of symptoms. First-line therapies for primary cutaneous ALCL are surgical excision or radiotherapy.

PUVA-induced pityriasis lichenoides chronica-like papular lesions in patients with mycosis fungoides: a clinical, histopathological and immunohistochemical study.

Mycosis fungoides (MF) is the most common form of cutaneous T cell lymphoma (CTCL) with many clinical variants including papular and pityriasis lichenoides chronica (PLC)-like variants. During psoralen and ultraviolet A (PUVA) treatment of MF, PLC-like papular lesions were observed to appear. The exact nature of these lesions is not fully understood. This work aimed to study PLC-like papular lesions arising in MF patients receiving PUVA therapy clinically, histopathologically and immunohistochemically (using monoclonal antibodies against CD4 and CD8) and to compare them with lesions in classic PLC patients. Fifteen MF patients with PLC-like papular lesions arising during PUVA treatment were included and 15 patients with classic PLC served as controls. While the extent of these lesions significantly correlated with their duration (p < 0.05), it showed no significant correlation with the TNMB stage of MF, number of phototherapy sessions or cumulative UVA dose at which they started to appear. The response status of MF to PUVA did not affect their development. Compared to classic PLC, these lesions showed significantly more acute onset (p = 0.003). None of these lesions showed histopathological features essential to diagnose papular/PLC-like MF and no significant difference existed with regard to their histopathological and CD4/CD8 phenotypic features compared to classic PLC. Papular lesions mimicking PLC in MF patients receiving PUVA mostly represent an upgrading reaction with possible good prognostic implication.

An Atypical Presentation of PLEVA: Case Report and Review of the Literature

Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare, self-limited, cutaneous disorder of unknown etiology. Clinically, PLEVA is characterized by the sudden onset of scaly, erythematous macules and papules localized to the trunk and proximal extremities. We report the case of a patient who presented with multiple erythematous papules and plaques on the palms, forearms, and dorsal feet. J Drugs Dermatol. 2019;18(7):690-691.

A case of pityriasis lichenoides: Rapid resolution with azithromycin monotherapy in 3 weeks.

Pityriasis lichenoides (PL) is a spectrum of inflammatory skin diseases which include PL et varioliformis acuta (PLEVA) and PL chronica (PLC) as two ends of the disease and rarely both entities can coexist on the same patient. Treatment options are based on case series-reports, and anecdotes, and include topical corticosteroids, topical immunomodulators, systemic antibiotics (tetracycline, erythromycin), and phototherapy. Herein, we report a 13-year-old boy, exhibiting mixed manifestations of PLEVA and PLC lesions concurrently, with a rapid and dramatic response to azithromycin monotherapy.

Micosis fungoide. Experiencia en un hospital pediátrico / Mycosis Fungoides: Experience in a Pediatric Hospital

La micosis fungoide (MF) es el linfoma cutáneo primario de células T más frecuente. Su aparición en la infancia es excepcional. Objetivos: Describir las características epidemiológicas, clínicas, histopatológicas e inmunofenotípicas de los pacientes con MF. Describir los tratamientos utilizados y la evolución. Material y método: Se incluyeron todos los pacientes admitidos en el Hospital de Pediatría Dr. J. P. Garrahan (Argentina) en el período comprendido entre agosto de 1988 y julio de 2014 con diagnóstico clínico e histopatológico de MF. Resultados: Se diagnosticaron 14 pacientes con MF. La distribución por sexo fue M/F: 1:1,33. La edad media al diagnóstico fue de 11,23 años (rango: 8 a 15 años). El tiempo promedio de evolución hasta el momento del diagnóstico fue de 3 años y 6 meses (rango: 4 meses a 7 años). Todos los pacientes presentaron la forma clínica hipopigmentada y en el 42% se asoció la forma clásica. El 50% (n = 7) exhibió un inmunofenotipo CD8 positivo de forma exclusiva. El 78% presentó estadio IB. La fototerapia fue el tratamiento de elección. Cuatro pacientes tuvieron por lo menos una recaída y 3 demostraron progresión de su enfermedad a nivel cutáneo. La evolución fue favorable en todos los casos. Conclusiones: La MF es una entidad infrecuente en la infancia, siendo la forma hipopigmentada la más frecuente. Su diagnóstico es tardío debido a la similitud con otras enfermedades hipopigmentadas frecuentes en la niñez. A pesar de tener un buen pronóstico, presenta alta tasa de recidivas y requiere un seguimiento a largo plazo (AU)

Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. Objectives: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. Material and method: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. Results: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. Conclusions: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary (AU)

Successful treatment of pityriasis lichenoides chronica with narrow-band ultraviolet B therapy in a patient with Keratitis-Ichthyosis-Deafness syndrome: a case report.

Keratitis-ichthyosis-deafness (KID) syndrome is a rare genodermatosis causing ichthyosis-like skin lesions, keratitis, and deafness. Herein, we report a patient with this rare syndrome in association with pityriasis lichenoides chronica, which was succesfully treated with narrow-band ultraviolet B phototherapy despite our concerns regarding the increased risk of squamous cell carcinoma, hyperpyrexia, and keratitis.

Pityriasis lichenoides chronica presenting with bilateral palmoplantar involvement.

Pityriasis lichenoides is an uncommon, acquired, idiopathic, self-limiting skin disease that poses a challenge to patients and clinicians to diagnose and treat. Several variants exist including pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC), and febrile ulcer-onecrotic Mucha-Habermann disease. Precise classification can be difficult due to an overlap of clinical and histologic features. In this case report we describe a patient with a rare presentation of PLC exhibiting bilateral palmoplantar involvement and mimicking psoriasis. We review the literature and discuss the clinical course, pathogenesis, and current treatment modalities of PLC.

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

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.

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.

Pityriasis lichenoides in an Asian population.

BACKGROUND: There are few studies comparing pityriasis lichenoides (PL) in adults and children, with fewer involving Asians. We compared the clinical profile and treatment outcomes of 10 adults and five children diagnosed with PL. METHODS: We retrospectively reviewed a series of patients diagnosed with PL at our hospital from 2002 to 2008. RESULTS: We identified 10 adults and five children (nine adults and four children with pityriasis lichenoides chronica; one adult and one child with pityriasis lichenoides et varioliformis acuta). Mean age of onset of disease was 42.2 years in adults and 12.2 years in children. Median duration of disease before presentation was 5.5 months in adults and 6 months in children. Trunk and limbs were the most common sites of involvement. Itch was present in the majority. Almost all patients did not respond to topical corticosteroids. A good response was seen in three adults and two children treated with oral antibiotics. Three adults and two children responded to treatment with narrow-band ultraviolet B phototherapy. The median time to resolution was 8 months in adults and 21 months in children. Postinflammatory dyspigmentation was seen in six adults and four children. CONCLUSION: PL presents similarly in adults and children; however, it runs a longer course in children. In our series, postinflammatory hyperpigmentation was common, which may be explained by our patients' darker skin phototypes. We routinely prescribe oral antibiotics initially in patients without contraindications. Phototherapy may be useful in patients not responding to antibiotics.

Pityriasis lichenoides et varioliformis acuta: a disease spectrum.

Pityriasis lichenoides et varioliformis acuta (PLEVA), or Mucha-Habermann disease (MHD), is a cutaneous disorder evident with crops of erythematous macules and papules, usually on the trunk and flexural areas of the extremities. Its etiology remains unknown. PLEVA is speculated to be an inflammatory reaction triggered by certain infectious agents, an inflammatory response secondary to T-cell dyscrasia, or an immune complex-mediated hypersensitivity. Histologic examination of a skin biopsy specimen is the standard for the identification of PLEVA, but definitive diagnosis may be difficult. Apart from the febrile ulcerative variant, which may be fatal, PLEVA tends to be self-limited in its course. Treatment is targeted mainly at the symptomatic relief of pruritus.

Etanercept in therapy multiresistant overlapping pityriasis lichenoides.

INTRODUCTION: Pityriasis lichenoides (PL) exhibits a protean clinical presentation, particularly in its overlapping form (OPL) combining aspects of the acute and chronic types. Some patients are drug multiresistant and pose a therapeutic dilemma. The anti-tumor necrosis factor (TNF)-alpha agent etanercept, was recently introduced as an alternative treatment for psoriasis, rheumatoid arthritis, and psoriatic arthritis. CASE REPORT: A 65-year-old woman suffered from an overlapping form of pityriasis lichenoides (OPL) for 5 years. Several initial acute episodes were controlled by successive courses of oral antibiotics, topical corticosteroids, and/or psoralen ultraviolet light-A (PUVA) therapy. The disease progressively evolved to a more chronic form. Topical immune response modifiers and corticosteroids, as well as PUVA, ultraviolet light-B (UVB), methotrexate, dapsone, and cyclosporine were introduced, but all proved ineffective. Due to the therapy multiresistance, 2 weekly injections of etanercept were administered. After 2 months, a marked improvement was observed in regards to the patient's pruritus and inflammation. No treatment-related adverse effects were observed. Therapy was continued for 4 months without any new lesion development. However, 1 month after stopping treatment new OPL lesions recurred. CONCLUSION: At the time of publication, this is the first report of the effectiveness of etanercept in OPL. This drug might be considered as a therapeutic alternative for treatment multiresistant OPL.

Pityriasis lichenoides: the differences between children and adults.

BACKGROUND: Pityriasis lichenoides (PL) is a skin disease that affects both children and adults. Anecdotally, it is said to run a more benign course in children, with a frequent tendency to self-resolution. However, to our knowledge, there have been no published studies comparing PL in both age groups. OBJECTIVE: To evaluate the clinicopathological features, overall efficacy of treatments and disease outcomes in children and adults diagnosed with PL. METHODS: A retrospective review of records was undertaken on all patients diagnosed with PL at two regional centres during an 8-year period (from 1998 to 2006). For each individual, data were collected on age, sex, number of lesions, lesional morphology and distribution, symptoms, histopathological features, treatment modalities (and response), overall follow-up and length of remission. RESULTS: We recorded 25 children (median age 8 years, range 2-18) and 32 adults (median age 40 years, range 20-65) with PL. All the children and adults had more than 20 scaly, papular lesions. Children had greater lesional body involvement than adults. Lesions on the legs and trunk were present in 23 children (92%) compared with 19 adults (59%) (P < 0.01) and facial involvement was observed more commonly in children (n = 10, 40%) compared with adults (n = 8, 25%). Dyspigmentation was significantly more common in children (n = 18, 72%) compared with adults (n = 6, 19%) (P < 0.001). Topical corticosteroids were used by 16 children (64%) and 18 adults (56%) but only half in each group found these effective. Eight children (32%) were treated with erythromycin, with only two (25%) clearing, and one of these subsequently relapsing. In contrast, four adults (13%) received antibiotics, with three (75%) clearing and none relapsing thereafter. Ultraviolet B phototherapy was used in eight children (32%), with seven (88%) completely or almost clearing, but four (57%) subsequently relapsed. Fourteen adults (44%) received phototherapy; 10 (71%) completely cleared and only two of these (20%) subsequently relapsed. Strikingly, after a median disease duration of 30 months, only five children (20%) went into complete remission compared with 25 adults (78%) (P < 0.001). CONCLUSIONS: This is the first study to compare PL in children and adults. Our findings suggest that, compared with adults, PL in children is more likely to run an unremitting course, with greater lesional distribution, more dyspigmentation and a poorer response to conventional treatment modalities.

Role of bromelain in the treatment of patients with pityriasis lichenoides chronica.

OBJECTIVES: Pityriasis lichenoides chronica (PLC) is a skin disease of unknown etiology. Uncertainty about the etiopathogenesis of this skin disease is the reason for the unpredictable and non optimal efficacy of therapies available for its treatment. The aim of the present study was to evaluate the efficacy of bromelain, a crude aqueous extract of the stems and immature fruit of pineapple, in the treatment of PLC. MATERIALS AND METHODS: Eight patients (3 males and 5 females) with PLC were enrolled in the study and treated for three months with oral bromelain (40 mg 3 times a day for 1 month, 40 mg twice a day for 1 month and 40 mg/day for 1 month). RESULTS: All patients showed complete clinical recovery after treatment. In 12 months of follow up, two patients experienced relapse 5-6 months after suspension of therapy but responded to another brief cycle of therapy. No side effects were encountered during therapy. CONCLUSIONS: In conclusion bromelain can be considered an effective therapeutic option for PLC; its efficacy could be related to its anti-inflammatory, immunomodulatory and/or anti-viral properties.

Pitiriasis liquenoide localizada / Localized pitiryasis lichenoides

La pitiriasis liquenoide es una enfermedad papulodescamativa de etiología desconocida frecuente en la edad pediátrica. Las lesiones suelen distribuirse de manera difusa en el tronco y las extremidades, y sólo excepcionalmente se han descrito casos de afectación localizada en un área limitada del cuerpo. Presentamos el caso de un niño de 9 años con brotes recurrentes de pitiriasis liquenoide de localización exclusiva en la zona inferior del abdomen

Pitiryasis lichenoides is a papulosquamous disorder of unknown etiology frequently seen in the pediatric population. The lesions are usually widespread on the trunk and extremities, and only exceptional cases of localized forms have been reported. We report a 9-year-old patient with recurrent crops of pitiryasis lichenoides lesions exclusively involving the lower abdomen

Pityriasis lichenoides chronica.

A 19-year-old woman presented with a five-year history of guttate macules and yellow to skin-colored papules with collarette of fine scale on the trunk and the upper and lower extremities. Guttate pityriasis lichenoides chronica is an uncommon presentation of this T-cell-mediated disease. It is of unknown etiology; however, an infectious agent has been suspected. It is considered in a spectrum with pityriasis lichenoides et varioloformis acuta and rarely mycosis fungoides and CD30 lymphomas. Improvement has been shown after treatment with oral tetracyclines, ultraviolet B and UVA1 phototherapy, and PUVA photochemotherapy.

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.