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Proceedings of the Royal Society of... 1921
PubMed: 19981655
DOI: No ID Found -
Clinical Reviews in Allergy & Immunology Dec 2023Recent advances in medical genetics elucidated the background of diseases characterized by superficial dermal and epidermal inflammation with resultant aberrant... (Review)
Review
Recent advances in medical genetics elucidated the background of diseases characterized by superficial dermal and epidermal inflammation with resultant aberrant keratosis. This led to introducing the term autoinflammatory keratinization diseases encompassing entities in which monogenic mutations cause spontaneous activation of the innate immunity and subsequent disruption of the keratinization process. Originally, autoinflammatory keratinization diseases were attributed to pathogenic variants of CARD14 (generalized pustular psoriasis with concomitant psoriasis vulgaris, palmoplantar pustulosis, type V pityriasis rubra pilaris), IL36RN (generalized pustular psoriasis without concomitant psoriasis vulgaris, impetigo herpetiformis, acrodermatitis continua of Hallopeau), NLRP1 (familial forms of keratosis lichenoides chronica), and genes of the mevalonate pathway, i.e., MVK, PMVK, MVD, and FDPS (porokeratosis). Since then, endotypes underlying novel entities matching the concept of autoinflammatory keratinization diseases have been discovered (mutations of JAK1, POMP, and EGFR). This review describes the concept and pathophysiology of autoinflammatory keratinization diseases and outlines the characteristic clinical features of the associated entities. Furthermore, a novel term for NLRP1-associated autoinflammatory disease with epithelial dyskeratosis (NADED) describing the spectrum of autoinflammatory keratinization diseases secondary to NLRP1 mutations is proposed.
Topics: Humans; Psoriasis; Inflammation; Mutation; Immunity, Innate; Keratosis; Guanylate Cyclase; Membrane Proteins; CARD Signaling Adaptor Proteins; Interleukins
PubMed: 38103162
DOI: 10.1007/s12016-023-08971-3 -
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 -
Dermatology Online Journal Nov 2004A 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...
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.
Topics: Adult; Female; Humans; Pityriasis Lichenoides
PubMed: 15748578
DOI: No ID Found -
Annals of Dermatology Oct 2016Pityriasis lichenoides (PL)-like skin lesions rarely appear as a specific manifestation of mycosis fungoides (MF).
BACKGROUND
Pityriasis lichenoides (PL)-like skin lesions rarely appear as a specific manifestation of mycosis fungoides (MF).
OBJECTIVE
We investigated the clinicopathological features, immunophenotypes, and treatments of PL-like MF.
METHODS
This study included 15 patients with PL-like lesions selected from a population of 316 patients diagnosed with MF at one institution.
RESULTS
The patients were between 4 and 59 years of age. Four patients were older than 20 years of age. All of the patients had early-stage MF. In all patients, the atypical lymphocytic infiltrate had a perivascular distribution with epidermotropism. The CD4/CD8 ratio was <1 in 12 patients. Thirteen patients were treated with either narrowband ultraviolet B (NBUVB) or psoralen+ultraviolet A (PUVA), and all of them had complete responses.
CONCLUSION
PL-like MF appears to have a favorable prognosis and occurrence of this variant in adults is uncommon. MF should be suspected in the case of a PL-like skin eruption. Therefore, biopsy is required to confirm the diagnosis of PL-like MF, and NBUVB is a clinically effective treatment.
PubMed: 27746631
DOI: 10.5021/ad.2016.28.5.540 -
Revista Chilena de Pediatria 2015Pityriasis lichenoides is a benign inflammatory disease of unknown etiology. There are two types of this condition: an acute form (PLEVA = pityriasis lichenoides et... (Review)
Review
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.
Topics: Adrenal Cortex Hormones; Aftercare; Anti-Bacterial Agents; Biopsy; Child; Chronic Disease; Humans; Male; Pityriasis Lichenoides; Treatment Outcome; Ultraviolet Therapy
PubMed: 26235693
DOI: 10.1016/j.rchipe.2015.04.024 -
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 -
Open Access Macedonian Journal of... Jan 2019Pityriasis lichenoides et varioliformis acuta (PLEVA) is an uncommon condition which presents acutely with papulo-vesicles that may develop necrotic, ulcerative, or...
BACKGROUND
Pityriasis lichenoides et varioliformis acuta (PLEVA) is an uncommon condition which presents acutely with papulo-vesicles that may develop necrotic, ulcerative, or hemorrhagic changes.
AIM
We studied clinical, and treatment characteristics of PLEVA hospitalised patients at our hospital from September 2009 to December 2014.
METHODS
The records of 15 PLEVA patients were retrospectively reviewed.
RESULTS
The median age of onset was 21.8 ± 18.81 (from 1 to 68), male to female ratio was 2/1. The common area of onset was trunk (60.0%) and extremities (33.3%). Clinical features were purpuric papules (100%), hemorrhagic crusted papules (46.7%), pustular purpuric papules (40%), and necrotic ulcerating lesions (13.3%).
CONCLUSION
All patients were received systemic antibiotics (macrolides: 53.3%, others: 46.7%), 2 patients were added immunosuppressive drugs. A 1-year-old patient died, others had a good response.
PubMed: 30745957
DOI: 10.3889/oamjms.2019.005 -
Proceedings of the Royal Society of... 1920
PubMed: 19980918
DOI: No ID Found