Pityriasis Lichenoides Research Articles

Development of Pityriasis Lichenoides in a Pediatric Patient with Mycosis Fungoides

Development of Pityriasis Lichenoides in a Pediatric Patient with Mycosis Fungoides

Efficacy of phototherapy in pityriasis lichenoides chronica of dark skin: A retrospective study from a tertiary center in Mexico

Dermatologic TherapyVolume 31, Issue 6 e12734 Therapeutic Hotline: Letter Efficacy of phototherapy in pityriasis lichenoides chronica of dark skin: A retrospective study from a tertiary center in Mexico Adrian Cuellar-Barboza, Adrian Cuellar-Barboza orcid.org/0000-0003-2405-5201 Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorJesus Alberto Cardenas-De La Garza, Jesus Alberto Cardenas-De La Garza Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorCesar Daniel Villarreal-Villarreal, Cesar Daniel Villarreal-Villarreal Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorJorge Ocampo-Candiani, Jorge Ocampo-Candiani orcid.org/0000-0002-0213-0031 Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorOliverio Welsh, Oliverio Welsh Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorMaira E Herz-Ruelas, Maira E Herz-Ruelas Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorMinerva Gómez-Flores, Corresponding Author Minerva Gómez-Flores minervagomezmx@yahoo.com.mx Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, Mexico Correspondence Dr. Minerva Gómez-Flores, Servicio de Dermatología, Hospital Universitario “Dr. José Eleuterio González”, U.A.N.L., Av. Madero y Gonzalitos s/n, Col. Mitras Centro, Monterrey, NL, Mexico 64460, Email: minervagomezmx@yahoo.com.mxSearch for more papers by this author Adrian Cuellar-Barboza, Adrian Cuellar-Barboza orcid.org/0000-0003-2405-5201 Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorJesus Alberto Cardenas-De La Garza, Jesus Alberto Cardenas-De La Garza Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorCesar Daniel Villarreal-Villarreal, Cesar Daniel Villarreal-Villarreal Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorJorge Ocampo-Candiani, Jorge Ocampo-Candiani orcid.org/0000-0002-0213-0031 Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorOliverio Welsh, Oliverio Welsh Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorMaira E Herz-Ruelas, Maira E Herz-Ruelas Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, MexicoSearch for more papers by this authorMinerva Gómez-Flores, Corresponding Author Minerva Gómez-Flores minervagomezmx@yahoo.com.mx Department of Dermatology, Universidad Autónoma de Nuevo León, Hospital Universitario “Dr. José Eleuterio González” y Facultad de Medicina, Monterrey, Nuevo León, Mexico Correspondence Dr. Minerva Gómez-Flores, Servicio de Dermatología, Hospital Universitario “Dr. José Eleuterio González”, U.A.N.L., Av. Madero y Gonzalitos s/n, Col. Mitras Centro, Monterrey, NL, Mexico 64460, Email: minervagomezmx@yahoo.com.mxSearch for more papers by this author First published: 03 October 2018 https://doi.org/10.1111/dth.12734Citations: 2Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume31, Issue6November/December 2018e12734 RelatedInformation

Discrasia linfoide epiteliotrópica de células T. Tratamiento con radiación ultravioleta

T-cell lymphoid dyscrasia is a group of different, infrequent, epitheliotropic inflammatory dermatosis that precede cutaneous T-cell lymphomas. Treatment with ultraviolet radiation is safe and efficacious. To describe responses to treatment, secondary effects and complications. Eleven patients with different T-cell lymphoid dyscrasias were treated. Two received psoralens plus ultraviolet A radiation (PUVA) and nine narrow band ultraviolet B radiation (NB-UVB). Seven women and four men from 5 to 30 years-old and phototypes III and IV were included. They had hypopigmented epitheliotropic lymphoid dyscrasia, chronic pityriasis lichenoides, hypopigmented parapsoriasis and pigmented purpuric dermatoses. Two received PUVA and 9 NB-UVB. Eight achieved complete response and three partial response with 10 to 119 sessions. Any patient presented neither relapse nor progression to cutaneous T-cell lymphoma during treatment or in the follow up period after 34 to 247 sessions. Secondary effects were xerosis and mild pruritus. The majority of patients with T-cell lymphoid dyscrasias can be successfully and safely treated with minimal secondary effects after a variable number of sessions and periods of time with ultraviolet radiation.

Pityriasis Lichenoides, Atypical Pityriasis Lichenoides, and Related Conditions

Pityriasis lichenoides (PLs) is an uncommon skin disease of unknown etiology. In recent years, an atypical form of PL has been described, showing overlapping features with mycosis fungoides (MF) and lymphomatoid papulosis. We studied 66 patients with an initial histopathologic diagnosis of PL (M:F=34:32; median age, 25 y; range, 7 to 85 y). According to clinical and phenotypic features, cases were classified into 4 categories: (1) Conventional PL (characteristic clinical features of PL without phenotypic aberrations) (n=20; M:F=8:12; median age, 37 y; range, 9 to 74 y); (2) Atypical form of PL (characteristic clinical features of PL with phenotypic aberrations) (n=25; M:F=16:9; median age, 21 y; range, 7 to 72 y). Four of these patients subsequently developed MF; (3) Lymphomatoid papulosis (waxing and waning lesions and positivity for CD30) (n=10; M:F=4:6; median age, 41 y; range, 16 to 83 y); (4) MF (clinical features typical of MF) (n=11; M:F=6:5; median age, 17 y; range, 8 to 85 y). Molecular analyses of clonality of the infiltrate did not reveal relevant differences among these 4 groups. Our study suggests that patients with an initial histopathologic diagnosis of PL may belong to different groups, showing that clinicopathologic correlation and complete phenotypic analyses are paramount in order to achieve proper classification. Although the relationship between PL and MF is yet a matter of debate, at the present state of knowledge, patients with a clinicopathologic presentation consistent with PL but with aberrant phenotypic features should be monitored in order to detect a possible evolution into MF.

CASE REPORT OF MUCHA-HABERMANN DISEASE

Pityriasis lichenoides et varioliformis acuta (PLEVA), also known as a Mucha-Habermann disease, is an uncommon, idiopathic, and acquired dermatosis. The disease is characterized by erythematous, scaly, papules, and polymorphic lesions which often progresses to hemorrhagic necrosis and heals with varioliform scarring. A febrile ulceronecrotic variant of PLEVA, also termed pityriasis lichenoides (PL) with ulceronecrosis and hyperthermia (PLUH) or febrile ulceronecrotic Mucha-Habermann disease (FUMHD), is a severe variant of PLEVA. The disease is characterized by the acute onset of large, more destructive, coalescent papules, leading to ulceronecrotic skin lesions associated with high fever and other systemic symptoms. In spite of the presence of multiple treatment modalities with variable success rate, the disease has poor prognosis. Here, we report the case of a 17-year-old male patient who presented with typical features of FUMHD and responded well to systemic administration of corticosteroid therapy.

Relationship Between Pityriasis Lichenoides and Mycosis Fungoides: A Clinicopathological, Immunohistochemical, and Molecular Study

Several cases of pityriasis lichenoides (PL) have been reported to evolve into mycosis fungoides (MF). To elucidate clues to this progression. Fifty-eight patients with PL between 2000 and 2013 (follow-up: 3-16 years, average: 8.3). A total of 3 (5.2%) of the 58 patients with PL developed MF after 3-11 years of prolonged clinical course. Papules and small plaques characterized PLs, and patches and larger plaques subsequent MFs. A total of 35 of 41 (85%) followed up non-MF associated patients with PL reported lasting complete remissions. Histopathologically, apoptotic keratinocytes disappeared mostly or completely in subsequent MFs. The presence of epidermotropism, folliculotropism, and epidermal lymphocytic nuclear atypia in PLs was not predictive of MF. CD8 cells were the dominant intraepidermal lymphocytes in the 3 PLs but remained so in only 1 subsequent MF. CD7 lymphocytes decreased substantially in 2 MFs, and lymphocytic nuclear atypia increased markedly in 1. T-cell receptor gene rearrangement studies demonstrated clonal populations in 1 of 2 studied PLs and in all 3 subsequent MFs. A few PLs may evolve into MF. Prolonged clinical course, appearance of patches and larger plaques, markedly increased lymphocytic nuclear atypia, marked diminution of apoptotic keratinocytes and CD7 and CD8 lymphocytes, and clonal T-cell receptor gene rearrangement may serve as clues.

Possible role of plasmacytoid dendritic cells in pityriasis lichenoides.

Plasmacytoid dendritic cells (pDCs) and their product, type I interferons (IFNs), have been implicated in the pathogenesis of several skin disorders characterized by an interface dermatitis (ID) pattern, such as lichen planus (LP). A type I IFN signature has previously been documented in pityriasis lichenoides (PL). Although pDCs are known to be the main source and most potent producers of local type I IFNs, their role in PL has not been investigated. To investigate the role of pDCs in PL. In total, 20 cases of PL and 20 comparable cases of LP were immunohistochemically tested for pDC occurrence and type I IFN production using anti-blood-derived dendritic cell antigen-2 (BDCA2; a specific pDC marker) and anti-myxovirus protein A (anti-MxA) antibodies (indirect marker of pDC activity), respectively. MxA is a well-established surrogate marker for local type 1 IFN production. A semiquantitative scoring system was used. pDCs were present in all 40 cases with no statistically significant difference between the two groups. MxA expression was intense and diffuse in the majority of PL and LP cases. pDCs constitute a central component of the inflammatory infiltrate in PL, suggesting that PL shares with the other entities that exhibit an ID a common pDC-driven process through type I IFN production, which ultimately leads to the cytotoxic attack.

Pityriasis lichenoides: Long-term follow-up study.

Pityriasis lichenoides is an uncommon papulosquamous disorder of unknown etiology. The objective of this study was to review the clinical features and treatment responses of individuals with pityriasis lichenoides seen at a tertiary referral center. Seventy-five patients diagnosed with pityriasis lichenoides between 1997 and 2013 were reviewed, and 46 had long-term follow-up via telephone interviews. Fifty (67%) patients were diagnosed with pityriasis lichenoides chronica, 22 (29%) with pityriasis lichenoides et varioliformis acuta, and 3 (4%) with mixed pityriasis lichenoides chronica and pityriasis lichenoides et varioliformis acuta features. Mean±standard deviation age at onset was 12±13years (median 8years). Disease duration was significantly shorter for patients with pityriasis lichenoides et varioliformis acuta (35±35months) than for those with pityriasis lichenoides chronica (at least 78±48months). At long-term follow-up, 23 of 28 (82%) patients with pityriasis lichenoides chronica and 3 of 16 (19%) with pityriasis lichenoides et varioliformis acuta had active disease. None progressed to lymphomatoid papulosis or cutaneous T-cell lymphoma. Ten of 23 active pityriasis lichenoides chronica cases had residual pigmentary change independent of race and lasted at least 35±20months. The most effective treatments were phototherapy (47% response rate), heliotherapy (33%), topical corticosteroids (27%), and antibiotics (25%). Pityriasis lichenoides is a predominantly pediatric disorder. The time course of pityriasis lichenoides chronica is significantly longer than that of pityriasis lichenoides et varioliformis acuta. Pityriasis lichenoides chronica may persist with pigmentary alterations in the absence of other signs of active inflammation. Treatment response is often limited, particularly for patients with pityriasis lichenoides chronica.

Clinicopathological correlation of noninfectious erythematous papulosquamous cutaneous lesions in a tertiary care hospital

Aims and Objectives: Papulosquamous lesions, the largest conglomerate of skin diseases, are characterized by scaling papules and plaques which amount to lots of confusion, and hence, a definitive histopathological diagnosis has a significant role. Our study was thus aimed at evaluation of correlation between clinical diagnosis with histopathological diagnosis of different noninfectious erythematous papulosquamous skin lesions encountered in a tertiary care center. Materials and Methods: A total of 50 cases of noninfectious erythematous, papulosquamous lesions prediagnosed by dermatologists of the same institute were included over a period of 1 year. Diagnosis was confirmed by histopathological examination using hematoxylin and eosin stain. Cases were tabulated according to the distribution of age, gender, localization of lesions, clinical, and histopathological diagnosis. Results: Majority of the patients were in the 20–40 years age group (66%) with slight female preponderance (52%). The limbs (42%) were most frequently involved site. Histopathologically, lichen planus was the most common (52%) followed by psoriatic lesions (20%), pityriasis rosea (4%), pityriasis rubra pilaris (4%), subacute cutaneous lupus erythmatosus (4%), prurigo simplex (4%), pityriasis lichenoides chronica (4%), urticaria (2%) and ashy dermatosis (2%). Correlation of clinical with histopathological diagnosis was positive in 92% cases and negative in 8% cases. Conclusion: The contribution of histopathology to the final diagnosis was significant. Skin biopsy is thus valuable in daily dermatology practice and appropriate clinicopathological correlation is very important for the effective diagnosis and treatment of patients.

Dermoscopy of Inflammatory Conditions: The Journey So Far

The use of dermoscopy in general dermatological practice has recently increased. Its non-invasive nature means it is being practiced frequently by dermatologists to diagnose various skin conditions. Dermoscopy, also known as dermatoscopy, allows dermatologists to quickly visualise skin structures up to the papillary dermis level. The skin patterns seen under dermoscopy are usually due to pigment and vascular structures; melanin and haemoglobin play major roles and give different patterns depending on the skin condition and pathological changes. Many inflammatory diseases are encountered by clinicians in daily practice; at times they are indistinguishable to the naked eye and a biopsy is required to confirm the diagnosis. Dermoscopy is a useful tool in the diagnosis and differentiation of inflammatory skin conditions and is aptly termed inflammoscopy when used in these situations. Inflammoscopy demonstrates the distinct characteristic patterns of many conditions and aids accurate diagnoses. In this article, the importance of dermoscopy in the diagnosis of relatively common inflammatory conditions, such as eczema, psoriasis, lichen planus, pityriasis rosea, pityriasis lichenoides et varioliformis acuta, pityriasis lichenoides chronica, and discoid lupus erythematosus, is highlighted. Here, an overview of dermoscopic patterns in each of these conditions is emphasised.

Significance of CD30 Expression by Epidermotropic T Cells in Lymphomatoid Papulosis and Lymphomatoid Pityriasis Lichenoides

CD30, a lymphocyte activation antigen that is expressed by neoplastic T cells in a subset of mycosis fungoides and primary cutaneous CD30+ lymphoproliferative diseases, is widely used to differentiate lymphomatoid papulosis (LyP) from pityriasis lichenoides.

Paraviral exanthems

Paraviral exanthems are distinct skin diseases due to infections with different viruses. Although no virus has been identified so far in some exanthems, the main age of manifestation, the clinical course of the exanthem, and the extracutaneous symptoms are suggestive for aviral genesis. While many viral infections are adirect result of the infection, paraviral exanthems reflect the response of the immune system to the infectious pathogens. Viruses cannot be identified in the skin. Typical paraviral exanthems include Gianotti-Crosti syndrome, pityriasis rosea, pityriasis lichenoides, papular-purpuric gloves and sock syndrome, and asymmetrical periflexural exanthema. Unilateral mediothoracic exanthem, eruptive pseudoangiomatosis are rare and eruptive hypomelanosis has been described recently.

Pityriasis lichenoides et varioliformis acuta in skin of color: new observations by dermoscopy.

BackgroundPityriasis lichenoides is an uncommon skin disease that presents in three different forms: pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC) and febrile ulceronecrotic-Mucha-Habermann disease. These represent a spectrum of a disease. PLEVA presents as skin eruption of multiple, small, red papules that develop into polymorphic lesions with periods of varying remissions, as well as possible sequels of hyper/hypopigmentation and varicella-like scars. Diagnosis of this condition is mainly clinical, and sometimes clinical differentiation from other conditions may be a difficult task that often requires histological analysis. In this study, PLEVA lesions were examined by dermoscopy, and the significance of specific dermoscopic findings was investigated in order to facilitate their differentiation from other inflammatory conditions.ObjectivesTo evaluate dermoscopic patterns in PLEVA and to correlate these patterns with histopathology.Materials and methodsThe study was conducted at S. Nijalingappa Medical College, Bagalkot. It was an observational case series study and patients were selected randomly. Ethical clearance and informed consent were obtained. PLEVA lesions in early and late phases were evaluated. A manual DermLite 3 (3Gen, San Juan Capistrano, CA) dermoscope attached to a Sony (Cyber Shot DSC-W800, Sony Electronics Inc., San Diego, California, USA, digital, 14 mega pixels) camera was employed. Histopathology was done to confirm the diagnosis. Data was collected and analyzed. Results were statistically described in terms of frequencies and types of dermoscopic patterns.ResultsThere was a total of 14 patients; 8 males and 6 females. Mean age of patients was 19 years. Mean duration of disease was 7 months. Dermoscopy in early-phase lesions revealed amorphous brownish areas around the hair follicles, dotted vessels, and scaling. Dermoscopy in late-phase lesions showed whitish-structureless areas and central white crust within whitish-structureless rim with scale, focal bluish-grayish areas or centrifugal strands irregularly distributed along the periphery and yellow structures. Red dots and hemorrhage were seen at the center and glomerular vessels at the periphery.ConclusionPLEVA demonstrates specific dermoscopic patterns that correlate well with histologic changes. New dermoscopic findings are described. Thus, dermoscopy is a good diagnostic tool in the clinical diagnosis of PLEVA.

Use of Phototherapy in Children

Phototherapy is a well-recognized treatment in adults and children. Previous articles have reported success in treating recalcitrant skin disorders such as atopic dermatitis (AD), psoriasis, pityriasis lichenoides chronica, and vitiligo in children. This was a retrospective review over an 18-month period from June 2012 to December 2013 of all children receiving phototherapy in a tertiary pediatric dermatology center. Seventy-five patients 3 to 17 years of age (mean 10.6 years; 35 male, 40 female) were included. Forty-eight (64%) patients had AD and 21 (28%) had psoriasis. Seventy received narrowband ultraviolet B (NBUVB) treatment and five received hand and foot psoralen and ultraviolet A (PUVA) treatment. All patients with AD were treated with NBUVB and four (8.3%) were also treated with hand PUVA. After phototherapy, 76% had documented clear to almost clear skin. At the 12-month follow-up, 52% of the patients with AD remained clear. All 21 patients with psoriasis underwent NBUVB phototherapy. The clearance rate after phototherapy was 86%. At the 12-month follow-up, 43% of the patients with psoriasis remained clear. Phototherapy can reduce disease burden in individuals with severe AD and psoriasis and should be considered as a second-line therapy if standard topical regimens are unsuccessful.

Treatment of adult diffuse pityriasis lichenoides chronica with narrowband ultraviolet B: experience and literature review.

Pityriasis lichenoides chronica (PLC) is an infrequent dermatosis of unknown aetiology, wholse evolution and response to treatment differs between children and adults. When PLC is recalcitrant or unresponsive to topical treatment, phototherapy is one of the main treatments used. We carried out a prospective study of adult diffuse PLC treated with narrowband ultraviolet B (NB-UVB). We treated eight patients whose disease showed no response to topical therapy, and obtained a complete response rate of 88% in a mean of 23 sessions (cumulative dose 16.99 J/cm2 ). However, the relapse rate was 43% in the first 6 months. Our results are similar to those of other published studies but there is much variability between them in the doses applied and the number of sessions needed. Further studies are necessary to devise a protocol for NB-UVB treatment of PLC.

Pityriasis lichenoides-like drug reaction: A clinical histopathologic study of 10 cases

Lymphomatoid drug reactions can mimic endogenous T and B cell lymphoproliferative diseases. We present a novel form of cutaneous drug reaction with features of pityriasis lichenoides (PL), a recognized form of T cell dyscrasia. Ten cases were studied where a cutaneous eruption exhibiting semblance to PL within a few weeks to months after starting a particular drug. The patient cohort comprised 7 females and 3 males with the mean age of 60 years. Widely distributederythematous cutaneous lesions were present in 6 cases whereas a more localized distribution was seen in three cases. The most frequently implicated drugsassociated with the eruption were antidepressants and statins. Histologic examination showed a morphologic picture identical to PL including marked epitheliotropism of mildly atypical lymphocytes, psoriasiform epidermal hyperplasia, dyskeratosis, hemorrhage, and a thick parakeratotic scale. Therewas a significant reduction in the expression of CD7 and CD62L amid the T cells. Regression of the eruption occurred in all cases excluding one. Thefindings conform the categorization of this process as a form of T-cell dyscrasia albeit one that is reversible, dependent on the drug withdrawal. The limitationof our study includes the retrospective design of the study.

A Fatal Case of Febrile Ulceronecrotic Mucha-Habermann Disease which Presenting as Toxic Epidermal Necrolysis.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a severe form of pityriasis lichenoides et varioliformis acuta, is an inflammatory dermatosis of unknown etiology manifested by ulcerative and necrotic lesions accompanied by systemic manifestations. The mortality rate of FUMHD is about 15%. It is reported here a case of FUMHD presenting as toxic epidermal necrolysis that resulted in multiple organ failure and death.

A Single-Arm PHASE 2A Study of NM-IL-12 (rHu-IL12) in Patients with Mycosis Fungoides-Type CTCL (MF) Undergoing Low-Dose TOTAL Skin Electron BEAM Therapy (LD-TSEBT)

Multiple treatment modalities are available for MF, but most result in inevitable relapse. Therefore, new treatment strategies that improve response rate and prolong response duration are greatly needed. TSEBT is a highly effective therapy in MF. LD-TSEBT (12 Gy) is much more tolerable than the conventional 36+ Gy dose, thereby allowing for re-treatment; however, LD-TSEBT has a less favorable complete response rate and response duration.Combining LD-TSEBT with immunostimulatory modalities in MF has a strong biological rationale, since radiation-induced exposure of cancer-specific antigens should be synergistic with concomitant stimulation of anti-cancer immune responses. Interleukin-12 is a robust candidate for radioimmunotherapy, as IL-12 has significant anti-MF activity as monotherapy, is very well tolerated without overlapping toxicity with TSEBT, and is a potent stimulator of innate and adaptive immunity.We report on a single-arm open-label phase 2a trial of combination of LD-TSEBT and NM-IL-12. Ten patients are planned for enrollment. Eligibility includes MF-type CTCL stages IB-IIIB and patients must be eligible for LD-TSEBT. TSEBT is started on study day 1 (fractionated 4 Gy/week, up to 12 Gy). NM-IL-12 is administered subcutaneously at 150 ng/kg on days 2 and 15, followed by 6 maintenance doses q4w at 100 ng/kg. The primary endpoint is safety with secondary endpoints being response rate and PFS.Currently, 6 patients are enrolled, 5 evaluable for response; 4 male; median age 55; three have stage IB, one IIB and one IIIB. Median number of previous therapies is 2 (0-6). The treatment was well-tolerated with only grade 1 or 2 AEs; most common AEs include grade 1 headache and chills.One patient achieved CR, 2 PR, and 2 SD. Median follow-up is so far 15 weeks and 5 patients remain on study. One patient has been withdrawn from the study due to development of a suspected PLC (pityriasis lichenoides chronica)-like skin reaction requiring topical steroid therapy. PK and PD analysis was completed in the first 4 patients. It demonstrated measurable drug levels in all patients studied, Cmax being 10.8-56.1 pg/ml achieved at 5-24 hours post injection. Interferon-γ and IP-10 (hallmark PD markers of IL-12 activity) were measurable after the first and the second injections in all patients. Levels of the inhibitory cytokine IL-10 were generally measurable after NM-IL-12 injections, but were very low (<1 pg/ml up to 12 pg/ml) - much lower than previously reported in IL-12 studies, thus unlikely to be associated with negative immunosuppressive feedback. There was no clear correlation between PK and PD measurements and either efficacy or toxicity, but the number of patients fully evaluated is small.Total RNA was extracted from patient blood samples and subjected to qRT-PCR. Gene specific primers were used to analyse intracellular expression of the IL-12 receptor and immune cell markers. Ongoing work investigates an activated cellular phenotype at week 15 of LD-TSEBT and NM-IL-12 treatment compared with the respective baseline samples.Overall these early results demonstrate that NM-IL-12 can be safely administered together with LD-TSEBT in CTCL patients. Encouraging clinical activity is observed including a CR. Enrollment is currently ongoing and planned PD and correlative biomarker studies are in progress.A phase 2B randomized trial of NM-IL-12 and low dose TSEBT compared against low-dose TSEBT alone in patients with MF is being developed based on the apparent benign AE profile as compared to approved systemic therapies. DisclosuresKim:Takeda: Membership on an entity’s Board of Directors or advisory committees, Research Funding; Eisai: Membership on an entity’s Board of Directors or advisory committees, Research Funding; Tetralogic: Research Funding; Neumedicine: Research Funding; Merck: Research Funding; Kyowa Kirin Pharma: Membership on an entity’s Board of Directors or advisory committees, Research Funding; Portola: Consultancy; miRagen: Research Funding; Innate: Research Funding; Horizon Pharma: Consultancy, Membership on an entity’s Board of Directors or advisory committees; Forty Seven Inc: Membership on an entity’s Board of Directors or advisory committees; Seattle Genetics: Membership on an entity’s Board of Directors or advisory committees, Research Funding; Soligenix: Research Funding. Ma:Neumedicines: Employment. Kha:Neumedicines: Employment. Lawrence:Neumedicines: Employment, Patents & Royalties. Vainstein:Neumedicines: Employment. Basile:Neumedicines: Employment, Equity Ownership, Membership on an entity’s Board of Directors or advisory committees, Patents & Royalties.

A case of pediatric pityriasis lichenoides et varioliformis acuta induced by coxsackie virus infection

A case of pediatric pityriasis lichenoides et varioliformis acuta induced by coxsackie virus infection

Phototherapy for Pityriasis Lichenoides in the Pediatric Population: A Review of the Published Literature.

Pityriasis lichenoides (PL) is a dermatologic disorder that manifests in either the acute (pityriasis lichenoides et varioliformis acuta) or the chronic form (pityriasis lichenoides chronica, also known as parapsoriasis chronica). Traditional first-line therapy consists of corticosteroids or antibiotics; however, these treatments are often accompanied with multiple side effects and may be ineffective. The goal of this study was to review the use of phototherapy for treating PL in the pediatric population. We performed a systematic review of the literature in the National Library of Medicine's PubMed database and the SCOPUS database discussing phototherapy for treatment of PL in the pediatric population. The following search terms were used: 'pityriasis lichenoides', 'pityriasis lichenoides chronica', 'pityriasis lichenoides et varioliformis acuta', and 'febrile ulceronecrotic Mucha-Habermann disease'. The systematic search and screening of articles resulted in 14 articles including a total of 64 patients with PL treated with phototherapy. Three different modalities were utilized, with five studies using broadband ultraviolet B (BB-UVB) radiation, nine studies utilizing narrowband UVB (NB-UVB), and two studies employing psoralen with ultraviolet A (PUVA) therapy. Overall, the use of BB-UVB had an initial clearance rate of 89.6% with 23.1% recurrence, whereas NB-UVB cleared 73% of the lesions with no recurrence, and PUVA therapy initially cleared 83% of the lesions with 60% recurrence. The side-effect profiles were similar and revealed limited toxicity. Phototherapy shows promising results and a favorable side-effect profile in the treatment of PL. Ultimately, large randomized controlled trials are needed to determine optimal treatments.