-
Journal of Cutaneous Pathology May 2024Cytophagic histiocytic panniculitis (CHP) is associated with a number of systemic conditions and is characterized by the presence of benign phagocytic histiocytes ("bean...
Cytophagic histiocytic panniculitis (CHP) is associated with a number of systemic conditions and is characterized by the presence of benign phagocytic histiocytes ("bean bag cells"), including phagocytosed erythrocytes, leukocytes, and platelets. We describe a case of a 72-year-old female who presented with a papular eruption that clinically mimicked pityriasis lichenoides et varioliformis acuta (PLEVA). Given that her skin biopsy had multiple features concerning PLEVA, this diagnosis was classified as a superficial pityriasis lichenoides-like variant of CHP. The histopathologic presence of cytophagic histiocytosis prompted workup for a systemic malignancy, leading to a diagnosis of underlying acute monocytic leukemia of myeloid lineage.
PubMed: 38769706
DOI: 10.1111/cup.14659 -
Pediatric Dermatology 2024Pityriasis lichenoides (PL) is an uncommon skin rash. PL has two main forms: Pityriasis lichenoides et varioliformis acuta (PLEVA): this "acute" (fast) form comes on...
Pityriasis lichenoides (PL) is an uncommon skin rash. PL has two main forms: Pityriasis lichenoides et varioliformis acuta (PLEVA): this "acute" (fast) form comes on quickly. Pityriasis lichenoides chronica (PLC): this "chronic" (long) form often develops slowly and lasts longer.
Topics: Child; Humans; Diagnosis, Differential; Pityriasis Lichenoides
PubMed: 38743416
DOI: 10.1111/pde.15645 -
Translational Pediatrics Apr 2024Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta, characterized by a rapid onset of...
BACKGROUND
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta, characterized by a rapid onset of painful, necrotic skin lesions and systemic symptoms. The diagnosis of FUMHD is complex, hinging on the clinical presentation, histopathological findings, and exclusion of other severe dermatoses. The key diagnostic criteria include sudden development of ulceronecrotic papules and plaques, fever, and evidence of systemic disease. Due to the rarity of FUMHD, there is no consensus on optimal treatment, reflecting a significant gap in the dermatological practice.
CASE DESCRIPTION
This report details a multimodal approach tailored to our 13-year-old patient, incorporating systemic corticosteroids, immunosuppressive therapy, and intensive supportive care. The strategy was designed to address the acute and aggressive nature of the disease while mitigating potential systemic complications. The report emphasizes on the intricate, multi-layered care required to manage FUMHD, illustrating the challenges and considerations in treating this complex condition. It underscored the necessity of a personalized, comprehensive care plan that extends beyond medical intervention to include psychological and social support. The outcome of our patient was encouraging, with a marked reduction in cutaneous manifestations and improvement in systemic symptoms.
CONCLUSIONS
It was found that prevention and care of skin injuries and complications, as well as the protection of patient's mental state during the development of the disease, are very important. Therefore, early diagnosis, prompt treatment, close monitoring of infection indicators, and specialized care are essential to improve the prognosis of patients with FUMHD.
PubMed: 38715678
DOI: 10.21037/tp-23-520 -
Journal of Cutaneous Pathology Apr 2024Psoriasis is an inflammatory skin disease driven by upregulation of cytokines in the Th17 pathway, including interleukin-36 (IL-36). Previous studies have highlighted...
BACKGROUND
Psoriasis is an inflammatory skin disease driven by upregulation of cytokines in the Th17 pathway, including interleukin-36 (IL-36). Previous studies have highlighted the utility of IL-36 immunostaining for psoriasis compared to spongiotic dermatitis and other psoriasiform dermatoses; however, no study has examined the role of IL-36 staining in distinguishing psoriasis from pityriasis rosea (PR) and pityriasis lichenoides (PL), known histologic mimickers of psoriasis.
METHODS
We compared the immunostaining pattern of IL-36 for 21 PR cases, 22 PL cases, and 10 psoriasis cases. We graded the immunostaining as 0, negative; 1, focal weak; 2, diffuse weak; 3, focal, strong; or 4, diffuse strong. We further categorized stains as negative (0-2 score) or positive (3-4 score) and utilized Fisher's exact test to compare the immunostaining pattern of these entities.
RESULTS
All psoriasis specimens were positive for IL-36, whereas all PR specimens were negative (p = 0.00000002). Twenty PL specimens were negative (p = 0.000001). Nine of 10 pityriasis lichenoides et varioliformis acuta cases were negative (p = 0.00012), and 11 of 12 cases of pityriasis lichenoides chronica were negative (p = 0.00003).
CONCLUSIONS
Our findings highlight the potential role of IL-36 immunostaining in distinguishing psoriasis from other psoriasiform dermatoses, including PR and PL.
PubMed: 38689501
DOI: 10.1111/cup.14633 -
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 -
Dermatology Reports Mar 2024In the wake of a global COVID-19 pandemic, where innovations in vaccination technology and the speed of development and distribution have been unprecedented, a wide...
In the wake of a global COVID-19 pandemic, where innovations in vaccination technology and the speed of development and distribution have been unprecedented, a wide variety of post-vaccination cutaneous reactions have surfaced. However, there has not been a systematic review that investigates pityriasis eruptions and the associated variants following COVID-19 inoculations. A PubMed search using was performed to find case reports from the earliest record through November 2022. Data including types of vaccination and pityriasis were extracted and a quality review was performed; 47 reports with 94 patients were found: 64.9% had pityriasis rosea (PR), 3.2% PR-like eruptions, 16.0% pityriasis rubra pilaris, 7.4% pityriasis lichenoides et varioliformis acuta, 3.2% pityriasis lichenoides chronica, and 5.3% had reactions described as . The top three COVID-19 vaccinations reported were Pfizer-BioNTech (47.9%), Oxford-AstraZeneca (11.7%), and Moderna (8.5%). Pityriasis reactivity was reported most frequently after the Pfizer-BioNTech vaccination, with pityriasis rosea being the most common variant. A large difference was additionally found between the ratio of post-vaccination pityriasis reactions following Pfizer and Moderna vaccinations (5.63), and the ratio of Pfizer's usage in the United States as of December 28, 2022 relative to that of Moderna (1.59). Further studies with adequate follow-up periods and diagnostic testing will thus need to be performed to elucidate the root of this discrepancy and better characterize the association between different pityriasis reactions and COVID-19 vaccinations.
PubMed: 38623364
DOI: 10.4081/dr.2023.9742 -
Photodermatology, Photoimmunology &... May 2024Pityriasis lichenoides chronica is the chronic end of the spectrum of pityriasis lichenoides which have several forms of papulosuamous conditions. Several treatments...
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.
Topics: Humans; Female; Male; Pityriasis Lichenoides; Phototherapy; Kinetics
PubMed: 38616405
DOI: 10.1111/phpp.12964 -
The British Journal of Dermatology Apr 2024Lymphomatoid Papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population is scarce.
BACKGROUND
Lymphomatoid Papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population is scarce.
OBJECTIVES
To characterize epidemiological, clinical, histopathological, and prognostic features of paediatric LyP.
METHODS
This was a retrospective, multicentre international cohort study including 87 cases of children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18 years old at disease onset were included. Diagnosis was made in each centre based on clinical-pathological correlation.
RESULTS
Eighty-seven patients from 12 centres were included. The mean age at onset was 7.0 years (range 3 months-18 years) with a male to female ratio of 2:1. The mean time between onset of first cutaneous lesions and diagnosis was 1.3 years (range 0-14 years). Initial misdiagnosis concerned 26.4% of patients. Initially, LyP was most often misdiagnosed as Pityriasis lichenoides et varioliformis acuta (PLEVA), insect bites, or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned for 20.7% of patients. The main histological subtype was type A in 55.1% of the cases. If analysed, monoclonal TCR rearrangement was found in 76.5% of the skin biopsies. The overall survival rate was 100% with follow up at 5 years available for 33 patients and at 15 years for 8 patients. A development of associated haematological malignancy (HM) occurred in 9.6% of the cases (7/73), including four mycosis fungoides (MF) cases, one primary cutaneous anaplastic large cell lymphoma (pc-ALCL), one systemic ALCL and one case of acute myeloid leukaemia. If we compare incidence rates of cancer with the world 0-19 years old population from 2001-2010, we estimate a significantly higher risk of associated malignancy in general, occurring before the age of 19 years old with incidence rate ratio of 87.49 (CI 86.01-88.99).
CONCLUSIONS
We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall the prognosis of the disease is good, with excellent survival rates for all patients. Due to increased risk of associated HM, a long-term follow-up should be recommended for LyP patients.
PubMed: 38595050
DOI: 10.1093/bjd/ljae150 -
JAAD Case Reports May 2024
PubMed: 38576898
DOI: 10.1016/j.jdcr.2024.02.021 -
Nagoya Journal of Medical Science Feb 2024Whole-exome and whole-genome sequencing have become widespread in approximately the last 15 years, and the predisposing factors and pathomechanisms of inflammatory... (Review)
Review
Whole-exome and whole-genome sequencing have become widespread in approximately the last 15 years, and the predisposing factors and pathomechanisms of inflammatory keratinization diseases, which have been unknown for a long time, have gradually been revealed. Hence, various inflammatory keratinization diseases are recognized to cause innate immunity hyperactivation. Therefore, we have been advocating for the clinical entity, "autoinflammatory keratinization diseases (AiKDs)" since 2017. AiKDs are inflammatory keratinization diseases caused by autoinflammatory-related pathomechanisms in the skin. The aberrant activation of innate immunity and the resultant autoinflammation in the epidermis and the superficial dermis in AiKDs cause hyperkeratosis in the epidermis. Our initially proposed concept of AiKDs included generalized pustular psoriasis and related conditions, pityriasis rubra pilaris type V, and familial keratosis lichenoides chronica. Since then, the number of diseases known to be AiKDs has increased as previously unknown disease-causing factors and pathogenetic mechanisms of inflammatory keratinization diseases have been clarified one by one. To date, porokeratosis, hidradenitis suppurative, keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK) syndrome, and AiKDs associated with epidermal growth factor receptor (EGFR) deficiency or with hepatitis and autism have been recognized as AiKDs. The concept of AiKDs is considered extremely useful in our precise understanding of the pathogeneses behind inflammatory keratinization diseases and our appropriate treatment method selection. The number of AiKDs is expected to grow with the clarification of the pathomechanisms of further inflammatory keratinization diseases.
Topics: Humans; Keratosis; Skin; Skin Neoplasms; Syndrome
PubMed: 38505726
DOI: 10.18999/nagjms.86.1.1