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Seminars in Diagnostic Pathology Jan 2017Primary cutaneous lymphomas represent a broad group of diseases with different clinical, histopathological, phenotypic, molecular, and prognostic features. All cutaneous... (Review)
Review
Primary cutaneous lymphomas represent a broad group of diseases with different clinical, histopathological, phenotypic, molecular, and prognostic features. All cutaneous lymphomas share the same tropism of neoplastic lymphocytes for the skin, but precise classification is paramount for proper management of the patients. Primary cutaneous lymphomas are classified according to the schemes proposed by the European Organization for Research and Treatment of Cancer (EORTC)-Cutaneous Lymphomas Task Force together with the World Health Organization (WHO) in 2005, and the WHO classification of 2008 with the 2016 update. The recognition of organ-based lymphomas, including cutaneous lymphomas, reflects a shift in the approach to lymphoproliferative disorders, and represents one of the major advances in the WHO classification of hematological tumors. Future studies should be aimed at shedding light on the many grey areas of cutaneous lymphomas (particularly the diagnosis and nomenclature of early mycosis fungoides and variants), and at gathering more data on the disorders that are still listed as provisional entities in the WHO classification.
Topics: Dermatology; History, 19th Century; History, 20th Century; History, 21st Century; Humans; Lymphoma; Medical Oncology; Skin Neoplasms
PubMed: 27979336
DOI: 10.1053/j.semdp.2016.11.001 -
American Journal of Clinical Dermatology Dec 2016Pityriasis lichenoides (PL) is a dermatologic disorder that manifests in either the acute (pityriasis lichenoides et varioliformis acuta) or the chronic form (pityriasis... (Review)
Review
BACKGROUND
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.
OBJECTIVE
The goal of this study was to review the use of phototherapy for treating PL in the pediatric population.
MATERIALS AND METHODS
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'.
RESULTS
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.
CONCLUSION
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.
Topics: Adrenal Cortex Hormones; Child; Humans; Pityriasis Lichenoides; Treatment Outcome; Ultraviolet Therapy
PubMed: 27502793
DOI: 10.1007/s40257-016-0216-2 -
Postepy Dermatologii I Alergologii Feb 2021Keratinization means cytodifferentiation of keratinocytes turning into corneocytes in the stratum corneum. Disorders of keratinization (hyperkeratosis, parakeratosis and... (Review)
Review
Keratinization means cytodifferentiation of keratinocytes turning into corneocytes in the stratum corneum. Disorders of keratinization (hyperkeratosis, parakeratosis and dyskeratosis) are causing many dermatological diseases, including various types of ichthyoses, pachyonychia congenita, pityriasis rubra pilaris, all subtypes of psoriasis, pityriasis lichenoides, dyskeratosis congenita, leukoplakia and keratosis follicularis, which apart from skin lesions may affect the eye's adnexae causing ectropion, entropion, blepharitis, madarosis, and trichiasis, the ocular surface causing keratitis, conjunctivitis, corneal ulceration and episcleritis, which in turn cause uveitis and various fundoscopic changes (proliferative retinopathy, retinal vasculopathy, macular oedema and birdshot chorioretinopathy). Knowledge of ocular symtoms associated with pathological keratinization is crucial, preventing sight-threatening complications such as corneal perforation, lagophthalmus, phthisis bulbi, retinal neovascularization, retinal vasculopathy and optic nerve atrophy. This review encourages dermatologists to monitor patients for ocular symptoms and encourage ophthalmologists to monitor patients for dermatological symptoms.
PubMed: 34408561
DOI: 10.5114/ada.2021.104272 -
Clinical and Experimental Dermatology Dec 2021The classification of pityriasis lichenoides (PL) into pityriasis lichenoides et varioliformis acuta (PLEVA), PL chronica (PLC) and febrile ulceronecrotic...
The classification of pityriasis lichenoides (PL) into pityriasis lichenoides et varioliformis acuta (PLEVA), PL chronica (PLC) and febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is based on both clinical and chronological features. In this retrospective monocentric study, we aimed to investigate the relevance of the classification in routine practice. We enrolled 49 patients (25 female, 24 male; median age 41 years). The lesions were papular in 76% of patients, necrotic in 12% and mixed in 12%. We found three histological patterns: 'classic' (65%), 'lymphomatoid' (13%) and 'mild' (22%). The 'lymphomatoid' pattern was associated with necrotic presentation and the 'mild' pattern with papular lesions (P = 0.01). Among the 27 patients with follow-up, 18% had relapses and 44% had chronic disease. One patient had mycosis fungoides. Neither clinical nor histological findings were correlated with disease progression, and are a reflection of the intensity of epidermal injury rather than of the disease course. The term 'pityriasis lichenoides' should be preferred to the classic PLEVA/PLC/FUMHD classification.
Topics: Adolescent; Adult; Aged; Child; Chronic Disease; Female; Follow-Up Studies; Humans; Male; Middle Aged; Necrosis; Pityriasis Lichenoides; Recurrence; Retrospective Studies; Severity of Illness Index; Young Adult
PubMed: 34170558
DOI: 10.1111/ced.14818 -
Jornal de Pediatria Apr 2024This study aims to evaluate the characteristics and treatment response of patients with pityriasis lichenoides seen in the last 43 years in a pediatric dermatology...
OBJECTIVES
This study aims to evaluate the characteristics and treatment response of patients with pityriasis lichenoides seen in the last 43 years in a pediatric dermatology service.
METHODS
This was a retrospective, analytical, longitudinal study of patients under 15 years of age. The medical records were reviewed and data were presented as frequencies, means and variances. Student's t-test, Mann-Whitney test, Fisher's exact test, Pearson/Yates chi-square test and multivariate logistic regression model were used, with p < 0.05 considered.
RESULTS
41 patients were included, 32 (78.0%) with pityriasis lichenoides chronica (PLC), five (12.2%) with pityriasis lichenoides et varioliformis acuta (PLEVA) and four (9.8%) with clinical PLC without biopsy. The age range of school children and adolescents was 19 (46.3%) and 13 (31.7%) respectively and 27 (65.8%) were male. Two peaks of the highest frequency were observed between 2004 and 2006 (10 patients - 24.4%) and another between 2019 and 2021 (6 patients - 14.7%). There was remission in 71.9% (n = 23), with 56.6% (n = 17) of those who used antibiotic therapy and 80% (n = 4) of those who had phototherapy. The chance of remission was 13 times greater in patients with disease onset after 5 years of age.
CONCLUSIONS
The clinical form most commonly found was PLC mainly in school children and adolescents. The frequency peaks coincided with infectious outbreaks. The remission rate was satisfactory with antibiotic therapy, but higher with phototherapy. Remission was greater in patients with disease onset after 5 years of age.
PubMed: 38677323
DOI: 10.1016/j.jped.2024.03.011 -
Pediatric Dermatology Mar 2017Phototherapy is a well-recognized treatment in adults and children. Previous articles have reported success in treating recalcitrant skin disorders such as atopic...
BACKGROUND
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.
METHODS
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.
RESULTS
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.
CONCLUSION
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.
Topics: Adolescent; Child; Child, Preschool; Dermatitis, Atopic; Female; Humans; Male; Phototherapy; Pityriasis Lichenoides; Psoriasis; Retrospective Studies; Treatment Outcome; Vitiligo
PubMed: 28133819
DOI: 10.1111/pde.13072 -
Der Hautarzt; Zeitschrift Fur... Apr 2016Erythematosquamous dermatoses in adolescents comprise a wide range of differential diagnoses. Age-typical variations of the clinical manifestation, the need to... (Review)
Review
Erythematosquamous dermatoses in adolescents comprise a wide range of differential diagnoses. Age-typical variations of the clinical manifestation, the need to differentiate common conditions from rare diseases as well as the tremendous psychosocial impact which the patients perceive especially in this vulnerable period of life can become major challenges for pediatric dermatologists. This article summarizes key features of common erythematosquamous dermatoses and less frequent skin diseases occurring during adolescence.
Topics: Adolescent; Adolescent Health; Dermatology; Diagnosis, Differential; Erythema; Female; Germany; Humans; Male; Psychology, Adolescent; Skin Diseases, Papulosquamous; Young Adult
PubMed: 26872905
DOI: 10.1007/s00105-016-3760-z -
Archives of Dermatological Research Apr 2023The exact aetiology of pityriasis lichenoides chronica (PLC) remains unknown. While phototherapy is the most investigated therapeutic modality, azithromycin has been... (Randomized Controlled Trial)
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.
Topics: Child; Humans; Child, Preschool; Azithromycin; Pityriasis Lichenoides; Ultraviolet Therapy; Streptococcal Infections; Exanthema; Antibodies; Treatment Outcome
PubMed: 36129521
DOI: 10.1007/s00403-022-02398-0 -
Dermatopathology (Basel, Switzerland) Aug 2021The term "pseudomalignancy" covers a large, heterogenous group of diseases characterized by a benign cellular proliferation, hyperplasia, or infiltrate that resembles a... (Review)
Review
The term "pseudomalignancy" covers a large, heterogenous group of diseases characterized by a benign cellular proliferation, hyperplasia, or infiltrate that resembles a true malignancy clinically or histologically. Here, we (i) provide a non-exhaustive review of several inflammatory skin diseases and benign skin proliferations that can mimic a malignant neoplasm in children, (ii) give pathologists some helpful clues to guide their diagnosis, and (iii) highlight pitfalls to be avoided. The observation of clinical-pathological correlations is often important in this situation and can sometimes be the only means (along with careful monitoring of the disease's clinical course) of reaching a firm diagnosis.
PubMed: 34449607
DOI: 10.3390/dermatopathology8030042 -
International Journal of Clinical and... 2021Pityriasis lichenoides-like drug reactions simulate pityriasis lichenoides clinically and histopathologically, though important differences exist. As a rule, pityriasis...
Pityriasis lichenoides-like drug reactions simulate pityriasis lichenoides clinically and histopathologically, though important differences exist. As a rule, pityriasis lichenoides has minimal to no eosinophils. However, this case illustrates that pityriasis lichenoides-like drug reaction can present with numerous eosinophils. This, in our experience is not rare, but contrasts with clinical reports in the literature that describe pityriasis lichenoides-like drug reactions with minimal to no eosinophils in the infiltrate. While similar, distinguishing these diseases is important given that pityriasis lichenoides is a lymphoproliferative disorder with a more protracted clinical course that is difficult to treat. We provide histopathological clues to aid in this important distinction.
PubMed: 34646420
DOI: No ID Found