Efficacy and safety of excimer light (308 nm) in the treatment of pityriasis lichenoides chronica.

INTRODUCTION: Pityriasis lichenoides chronica is the chronic end of the spectrum of pityriasis lichenoides which have several forms of papulosuamous conditions. Several treatments obtained complete clearance of the condition including phototherapy and specifically narrow band ultraviolet B. The Excimer light 308 is a monochromatic light that acts within the ultraviolet B wavelength and used as a targeted phototherapy in several skin conditions. METHODS: Thirty-four patients with histopathologically diagnosed pityriasis lichenoides chronica underwent treatment with biweekly sessions of excimer light 308 nm. Treatment continued until complete clearance was obtained or to a maximum of 48 sessions (24 weeks). RESULTS: Thirty-one patients obtained complete clearance with no recurrence till the end of the study period, two patients had partial response and only one patient showed poor response to treatment. CONCLUSION: Excimer light can be a safe and effective treatment of pityriasis lichinoides chronica in different ages and genders.

Febrile Ulceronecrotic Mucha-Habermann Disease Associated With Hemophagocytic Lymphohistiocytosis: A Case Report and Review of the Literature.

ABSTRACT: Mucha-Habermann disease (MHD) is an inflammatory skin disease characterized by polymorphous eruptions of erythematous, necrotic macules that have been reported for similarities to cutaneous T-cell lymphoma. Febrile ulceronecrotic MHD (FUMHD) represents a severe variant of MHD, marked by ulcers, hemorrhagic bullae, and systemic symptoms. Herein, we report a case of a severely atypical lymphomatoid expression of FUMHD associated with hemophagocytic lymphohistiocytosis (HLH). A previously healthy 21-year-old woman was admitted to the hospital with a rapidly progressive necrotic papular rash. Physical examination revealed right orbital swelling, bilateral hemorrhagic auricular bullae, and multiple ulcerative purpuric papulonodules on the trunk, face, and extremities. Biopsy indicated a dermal and subcutaneous infiltrate of atypical CD8 + lymphocytes with loss of CD5 and reduction in CD7 expression, along with features of lymphomatoid vasculitis. A diagnosis of a severely atypical lymphomatoid expression of FUMHD was made. The patient also met 7 of 9 HLH-2004 criteria, leading to a diagnosis of HLH. Positron emission tomography/computed tomography, flow cytometry, and rheumatologic workup were unremarkable. Treatment with an eight-week course of etoposide and dexamethasone for HLH led to rapid clinical improvement. Over time, her skin lesions regressed and eventually scabbed over to leave hyperpigmented scars, confirming the diagnosis of MHD. She has remained stable, off therapy for 4 years. Although potentially fatal, FUMHD often exhibits favorable outcomes and may resolve without recurrence, as in our patient. FUMHD should be considered in the differential diagnosis for patients presenting with cutaneous CD8 + necrotizing angiocentric lymphoproliferative disease complicated by HLH.

Dermoscopy as a diagnostic aid in pityriasis lichenoides et varioliformis acuta.

Diagnosis of pityriasis lichenoides et varioliformis acuta (PLEVA) is based on the characteristic pattern of lesions in different stages of development, ranging from erythematous maculopapules to papules with a crusted and/or necrotic centre. However, it may raise the differential diagnosis with other entities. It is therefore not uncommon to have to perform skin biopsies to reach a diagnosis, including in infants. In this study, we report the cases of three patients with PLEVA, highlighting the correlations between the clinical, dermoscopic and histological features. Observation of the dermatoscopic findings described, such as punctate or glomerular vessels and erythematous globules surrounding a homogeneous orange or crusty central area, may allow for a rapid diagnosis, avoiding the need for invasive techniques.

Narrowband ultraviolet B phototherapy for pityriasis lichenoides: A real-life experience.

INTRODUCTION: Pityriasis lichenoides (PL) is a papulosquamous disease affecting both children and adults, for which narrowband-UVB (NB-UVB) phototherapy is regarded as a commonly used treatment option. The aim of this study was to investigate the efficacy of NB-UVB phototherapy in the management of PL and to compare response rates in pediatric and adult age groups. MATERIALS AND METHODS: This observational, retrospective study included 20 PL patients (12 pityriasis lichenoides chronica; PLC, 8 pityriasis lichenoides et varioliformis acuta; PLEVA) who failed to respond to other treatment modalities. The data for this study were collected retrospectively from patient follow-up forms in the phototherapy unit. RESULTS: A complete response (CR) was obtained in all pediatric patients with PL, while 53.8% of adult patients had achieved CR. The mean cumulative dose required to achieve the CR was higher in pediatric patients than adult patients with PL (p < .05). The CR was achieved in 6 (75%) of 8 PLEVA patients, while 8 (66.7%) of 12 PLC patients had reached to CR. The mean number of exposures for patients with PLC to achieve a CR was higher than patients with PLEVA (p < .05). Erythema was the most common adverse effect during phototherapy particularly in 5 (35.7%) of the patients with PL who had achieved CR. CONCLUSIONS: NB-UVB is an effective and well-tolerated treatment option for PL especially in diffuse types. A higher response can be obtained in children with higher cumulative dose. Patients with PLC may require more exposures for CR than patients with PLEVA.

Acute lichenoid and varioliform pityriasis in a pediatric patient.

BACKGROUND: Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare dermatosis recognized as a benign condition of unknown etiopathogenesis. It is more common in pediatric patients and young adults and is characterized by multiple small or large erythematous plaques spread over the trunk and extremities. CASE REPORT: We describe the case of a 5-year-old male, previously healthy, with multiple erythematous lesions that disappeared leaving hypopigmented macules. The biopsy reported histological changes suggestive of mycosis fungoides. After a second revision of lamellae in this hospital, lymphocytic vasculitis (LV) with focal epidermal necrosis consistent with acute pityriasis lichenoides (PL) was identified. CONCLUSIONS: The existing knowledge about PLEVA lacks a consensus in specifying its classification, etiopathogenesis, diagnosis, and treatment, so this clinical condition represents a medical challenge. The diagnosis is made by clinical suspicion and confirmed by histology. The objective of this article was to report a case of PLEVA with an atypical presentation due to its histopathological findings, being the first report showing LV in children, as well as a review of the literature.

INTRODUCCIÓN: La pitiriasis liquenoide y varioliforme aguda (PLEVA) es una dermatosis poco frecuente, de etiopatogenia desconocida y evolución autolimitada. Es más común en pacientes pediátricos y adultos jóvenes, y está caracterizada por la presencia de múltiples placas eritematoescamosas pequeñas o grandes, diseminadas en el tronco y las extremidades. CASO CLÍNICO: Se describe el caso de un escolar de 5 años, de sexo masculino, previamente sano, que presentó múltiples cuadros de lesiones eritematosas que desaparecían dejando máculas hipopigmentadas. La biopsia reportó cambios histológicos sugestivos de micosis fungoide. Se realizó una segunda revisión de laminillas, identificando vasculitis linfocítica con necrosis epidérmica focal, consistente con pitiriasis liquenoide aguda. CONCLUSIONES: El conocimiento acerca de la PLEVA carece de un consenso que especifique su clasificación, etiopatogenia, diagnóstico y tratamiento, por lo que esta condición clínica representa un desafío médico. El diagnóstico se realiza por sospecha clínica y se confirma por histología. El objetivo de este artículo fue reportar un caso de PLEVA con presentación atípica por los hallazgos histopatológicos, siendo este el primer reporte de vasculitis linfocítica en niños, y además se realiza una revisión de la literatura.

Febrile ulceronecrotic Mucha-Habermann disease during pregnancy: A case report.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe form of 'pityriasis lichenoides et varioliformis acuta', with a progressive and potentially fatal course. To the best of our knowledge, there has been no reported case of FUMDH during pregnancy before. Due to life-threatening nature of the disease and the lack of evidence-based treatment, management of FUMHD in pregnancy is a therapeutic challenge. Additionally, some of the drugs that are effective in the treatment are contraindicated in pregnancy. Herein, we report a 27-year-old woman diagnosed with FUMHD in her 19th week of pregnancy and treated with ceftriaxone and erythromycin.

Febrile Ulceronecrotic Mucha-Habermann Disease: A Case Report and Review of Literature in the Paediatric Population.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta (PLEVA) that is characterized by a large ulceronecrotic appearance with high fever and a variety of systemic symptoms. We report here a case of FUMHD in a 17-year-old male Chinese patient who was treated successfully with a combination therapy of methotrexate, methylprednisolone, and intravenous immunoglobulin. In addition, a literature review was conducted to summarize the key characteristics of paediatric FUMHD cases.

The role of NLRP1 and NLRP3 inflammasomes in the etiopathogeneses of pityriasis lichenoides chronica and mycosis fungoides: an immunohistochemical study.

Mycosis fungoides (MF) is the most common subtype of primary cutaneous T cell lymphomas, whereas pityriasis lichenoides chronica (PLC) is a chronic inflammatory skin disorder. The inflammasome is a part of the natural immune system which has a multimeric structure consisting of the receptor, adaptor and effector protein that show specificity for various ligands or activators. After the activation of the inflammasome complex, caspase 1 becomes activated which subsequently triggers interleukin-18 (IL-18) and interleukin-1ß (IL-1ß) production. In our study we aimed to examine the roles of nucleotide-binding oligomerization domain-like receptor containing pyrin domain 1 (NLRP1) and nucleotide-binding oligomerization domain-like receptor containing pyrin domain (NLRP3) inflammasomes in the etiopathogeneses of PLC and MF. NLRP1, NLRP3, caspase 1, IL-18 and IL-1ß levels were examined and compared immunohistochemically in the skin biopsies belonging to 16 control patients; 16 PLC cases, 12 cases with stage 1 MF and 12 cases with other stages of MF (stage 2-4). In the paired comparisons of NLRP1, stage 2-4 MF group and PLC group were shown to have increased levels of NLRP1 expression compared to the control group. IL-1ß was also expressed at statistically significantly higher levels in each of the stage 1 MF, stage 2-4 MF and PLC groups compared to the control group. In the paired comparisons of caspase 1 and IL-18, it was found that stage 1 MF, stage 2-4 MF and PLC groups had increased levels of expression compared to the control group. Our findings suggest that the NLRP1 inflammasome pathway might play a role in the etiopathogenesis and progression of PLC and MF.

A case of febrile ulceronecrotic Mucha-Habermann disease responsive to systemic corticosteroids.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare, potentially fatal subtype of pityriasis lichenoides et varioliformis acuta (PLEVA). Herein, we present a rare case of a 14-year-old male without significant past medical history who was diagnosed with FUMHD without a clear inciting factor. He was effectively treated with systemic corticosteroids with complete resolution of symptoms.

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.

Type D lymphomatoid papulosis with pityriasis lichenoides et varioliformis acuta-like features in a child with parvovirus infection: a controversial diagnosis in the spectrum of lymphoid proliferations: case report and literature review.

BACKGROUND: Lymphomatoid papulosis (LyP) is a rare condition in pediatrics; LyP histological type D has been reported in only 7 children. The differential diagnosis of LyP in the spectrum of lymphoid proliferation remains controversial. CASE PRESENTATION: A 6-year-old boy presented to Emergency Department with a 3-week history of an erythematous papulo-vesicular itchy eruption over the submandibular regions, trunk and extremities. History, symptoms and laboratory tests were unremarkable. SARS-CoV-2 antigen was negative. The clinical suspicion of pityriasis lichenoides et varioliformis acuta (PLEVA) was posed, and topical steroids were introduced. One week after, he returned with an extensive painful scaly papulo-erythematous rash, with some ulcerated and necrotic lesions, and fever; therefore the child was hospitalized. Biochemical results were within reference limits, except for high level of C-reactive protein, aspartate aminotransferase, alanine transaminase and bilirubin. Due to a persistently high fever, systemic corticosteroid treatment was administered, with a good clinical response and an improvement of the skin lesions. Anti-PVB-19 Immunoglobulin M was detected. Elevated levels of IL-6, IL-10 and IFN-γ were also recorded. Five days post-admission, most of the lesions had cleared, and the child was discharged. Methotrexate was started, with a positive response. At skin biopsy a "PLEVA-like" pattern was apparent, with a dense, wedge shaped lymphoid infiltrate featuring epidermotropism and morphologically comprising pleomorphic and blastic cells. The pattern of infiltration was highlighted by immunohistochemical stains, which prove the process to feature a CD8+/CD30 + phenotype, the latter being intense on larger cells, with antigenic loss. Polymerase chain reaction for T-cell receptor gamma (TCRG) chain clonality assessment documented a monoclonal peak. A diagnosis of LyP type D was favored. CONCLUSION: The reported case encompasses most of the critical features of two separated entities-PLEVA and LyP-thus providing further support to the concept of them representing declinations within a sole spectrum of disease. Studying the role of infectious agents as trigger potential in lymphoproliferative cutaneous disorders and detecting novel markers of disease, such as cytokines, could have a crucial impact on pathogenic disease mechanisms and perspective therapies.

Childhood lymphomatoid papulosis Type D, a rare and challenging diagnosis.

A 10-year-old female with a several-year history of pityriasis lichenoides (PL) presented with a new, asymptomatic, large, and necrotic ulcer of her right upper arm. Skin biopsy was consistent with lymphomatoid papulosis (LyP) Type D, a recently recognized subtype of LyP that is distinguished histologically by marked epidermotropism and a perivascular infiltrate of medium-sized pleomorphic lymphocytes with a cytotoxic phenotype (CD3+, CD8+). This is only the sixth reported case of LyP Type D in a child, and while the prognosis in children appears favorable, with no reports of progression to lymphoma to date, more experience in children with longer-term follow-up is needed. Our case highlights both the challenging clinical diagnosis, since in our patient the longstanding clinical presentation was indistinguishable from PL, as well as histopathologic diagnosis, which required expert opinion and consensus.

Cutaneous manifestations following COVID-19 vaccination: A report of 25 cases.

Various adverse effects particularly cutaneous manifestations associated with different COVID-19 vaccines have been observed in practice. The aim of our study was to evaluate all patients who presented to our tertiary center with skin manifestations following COVID-19 vaccines injection from September to December 2021. All patients with skin manifestation within 30 days or less following COVID-19 vaccination were enrolled in our case-series. All cases included in our study were diagnosed based on clinical and/or histopathological evaluation and all other possible differential diagnoses were ruled out. Twenty-five individuals including 16 (64%) males and 9 (36%) females with the mean age of 47 ± 17.62 years (range 18-91) were enrolled in our study. Twenty-two (88%) patients developed lesions after Sinopharm vaccine injection and 3 (12%) cases manifested lesions after the AstraZeneca vaccine. Six (24%) patients developed new-onset lichen planus (LP) and 1 (4%) patient manifested LP flare-up. Two (8%) individuals developed psoriasis and 1 (4%) case showed psoriasis exacerbation. One (4%) patient developed new-onset pemphigus vulgaris (PV) and 1 (4%) case experienced a flare of PV lesions. One (4%) patient manifested pityriasis lichenoides et varioliformis acuta (PLEVA) flare-up. Other new-onset cases were as follows: toxic epidermal necrolysis (TEN) (n = 1, 4%), bullous pemphigoid (BP) (n = 2, 8%), alopecia areata (AA) (n = 2, 8%), pytriasis rosea (n = 1, 4%), herpes zoster (n = 1, 4%), cutaneous small vessel vasculitis (n = 1, 4%), erythema multiform (EM) and urticaria (n = 3, 12%), and morphea (n = 1, 4%). Physicians should be aware of the possible side effects especially cutaneous manifestations associated with COVID-19 vaccines.