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Actas Dermo-sifiliograficas Jun 2021Autoinflammatory keratinization disease (AiKD) is a novel clinical concept encompassing diseases with a genetic background and mixed pathogenic mechanisms of... (Review)
Review
Autoinflammatory keratinization disease (AiKD) is a novel clinical concept encompassing diseases with a genetic background and mixed pathogenic mechanisms of autoinflammation and autoimmunity, leading to an aberrant keratinization of the skin. Recent advances in medical genetics have revealed genetic causes and/or predisposing factors for a number of AiKD's, such as mutations in IL36RN related with pustular psoriasis, acrodermatitis continua and hidradenitis suppurativa, in CARD14 in pityriasis rubra pilaris type V and some forms of pustular psoriasis, and in NLRP1 related with familial keratosis lichenoides chronica (KLC). It is suspected that AiKD pathophysiology would also be involved in non-monogenic disorders. The bidirectional relationship between inflammation and keratinization should be understood in order to outline optimal management, and new drug development should take both targets into account. We assume that new inflammatory keratinization diseases may be recognized as AiKDs in the coming years.
PubMed: 34118208
DOI: 10.1016/j.ad.2021.05.015 -
Dermatologic Clinics Oct 2019Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica are the 2 main subtypes of pityriasis lichenoides. They represent the acute and chronic... (Review)
Review
Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica are the 2 main subtypes of pityriasis lichenoides. They represent the acute and chronic forms of the disease; both may have clonal T cells. Several treatment modalities are used, but it has been difficult to determine efficacy because of the possibility of spontaneous remission. Cutaneous CD30+ lymphoproliferative disorders constitute many cutaneous T-cell lymphomas and comprise lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma (ALCL). Both have an excellent prognosis. Lymphomatoid papulosis often only requires observation or treatment of symptoms. First-line therapies for primary cutaneous ALCL are surgical excision or radiotherapy.
Topics: Adrenal Cortex Hormones; Anti-Bacterial Agents; Antibodies, Monoclonal; Antineoplastic Agents; Dermatologic Surgical Procedures; Humans; Immunosuppressive Agents; Keratolytic Agents; Ki-1 Antigen; Lymphoma, Primary Cutaneous Anaplastic Large Cell; Lymphomatoid Papulosis; Phototherapy; Pityriasis Lichenoides; Radiotherapy; Skin Neoplasms
PubMed: 31466587
DOI: 10.1016/j.det.2019.05.005 -
Journal Der Deutschen Dermatologischen... Jul 2020
PubMed: 32713141
DOI: 10.1111/ddg.14153_g -
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 -
Pediatric Dermatology Mar 2018Pityriasis lichenoides is an uncommon papulosquamous disorder of unknown etiology. The objective of this study was to review the clinical features and treatment...
BACKGROUND/OBJECTIVES
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.
METHODS
Seventy-five patients diagnosed with pityriasis lichenoides between 1997 and 2013 were reviewed, and 46 had long-term follow-up via telephone interviews.
RESULTS
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 ± 13 years (median 8 years). Disease duration was significantly shorter for patients with pityriasis lichenoides et varioliformis acuta (35 ± 35 months) than for those with pityriasis lichenoides chronica (at least 78 ± 48 months). 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 ± 20 months. The most effective treatments were phototherapy (47% response rate), heliotherapy (33%), topical corticosteroids (27%), and antibiotics (25%).
CONCLUSION
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.
Topics: Adolescent; Anti-Bacterial Agents; Child; Child, Preschool; Female; Follow-Up Studies; Glucocorticoids; Humans; Infant; Male; Phototherapy; Pityriasis Lichenoides; Prognosis; Treatment Outcome
PubMed: 29315771
DOI: 10.1111/pde.13396 -
The Journal of Allergy and Clinical... Dec 2017
Review
Topics: Humans; Inflammation; Keratins; Skin Diseases
PubMed: 28668225
DOI: 10.1016/j.jaci.2017.05.019 -
Pediatric Dermatology 2015Pityriasis lichenoides (PL) is a skin condition of unclear etiology that occurs not uncommonly in childhood. It is often classified into the acute form, pityriasis... (Review)
Review
Pityriasis lichenoides (PL) is a skin condition of unclear etiology that occurs not uncommonly in childhood. It is often classified into the acute form, pityriasis lichenoides et varioliformis acuta (PLEVA), and the chronic form, pityriasis lichenoides chronica (PLC). We performed a comprehensive review of the English-language literature using the PubMed database of all cases of childhood PL reported from 1962 to 2014 and summarized the epidemiology, clinical features, treatment options, and prognosis of this condition in children. The proposed etiologies are discussed, including its association with infectious agents, medications, and immunizations and evidence for PL as a lymphoproliferative disorder. We found an average age of PL onset of 6.5 years, with a slight (61%) male predominance. We also found that PLEVA and PLC tend to occur with equal frequency and that, in many cases, there is clinical and histopathologic overlap between the two phenotypes. When systemic therapy is indicated, we propose that oral erythromycin and narrowband ultraviolet B phototherapy should be first-line treatment options for children with PL since they have been shown to be effective and well tolerated. In most cases, PL follows a benign course with no greater risk of cutaneous T-cell lymphoma, although given the rare case reports of transformation, long-term follow-up of these patients is recommended.
Topics: Adolescent; Age Factors; Biopsy, Needle; Child; Clindamycin; Combined Modality Therapy; Female; Humans; Immunohistochemistry; Male; Pityriasis Lichenoides; Prognosis; Risk Assessment; Severity of Illness Index; Sex Factors; Treatment Outcome; Ultraviolet Therapy
PubMed: 25816855
DOI: 10.1111/pde.12581 -
Clinics in Dermatology 2016Phototherapy can be a safe and effective treatment for various skin diseases in children. Special considerations governing the use of this treatment modality in... (Review)
Review
Phototherapy can be a safe and effective treatment for various skin diseases in children. Special considerations governing the use of this treatment modality in pediatric populations include patient, family, and facility-based factors that are oriented around heightened concerns with regard to safety and tolerability of treatment. Although phototherapy has been found to be effective in a wide range of dermatologic conditions affecting pediatric populations, including psoriasis, atopic dermatitis, pityriasis lichenoides, cutaneous T-cell lymphoma, and vitiligo, there is need for additional research on other conditions in which phototherapy has shown promise.
Topics: Adolescent; Child; Child, Preschool; Dermatitis, Atopic; Humans; Infant; Infant, Newborn; Lymphoma, T-Cell, Cutaneous; Patient Selection; Pityriasis Lichenoides; Psoriasis; Skin Diseases; Ultraviolet Therapy; Vitiligo
PubMed: 27638444
DOI: 10.1016/j.clindermatol.2016.05.018 -
Dermatologic Therapy May 2020Pityriasis lichenoides (PL) is an uncommon cutaneous disorder. Oral erythromycin is proposed to be effective in treating the disease. Here, we reported 16 pediatric...
Pityriasis lichenoides (PL) is an uncommon cutaneous disorder. Oral erythromycin is proposed to be effective in treating the disease. Here, we reported 16 pediatric patients with PL and systematically reviewed published literatures on erythromycin treatment response in pediatric PL patients, to observe the different treatment response to erythromycin in the pityriasis lichenoides chronica (PLC) and the pityriasis lichenoides et varioliformis acuta (PLEVA) groups. Sixteen patients, 8 with PLC and 8 with PLEVA, were treated with erythromycin. In the PLC group, 25% (n = 2) patients responded to erythromycin, while in the PLEVA group, 87.5% (n = 7) patients responded to erythromycin. The response rate was higher in the PLEVA group than the PLC group (P =.05). No side effect was reported in the 16 patients. A total of 34 children including 16 from our studies were included for further descriptive analysis, in which 12 had PLC and 22 had PLEVA. In the PLC group, 41.7% (n = 5) of patients responded to erythromycin while in the PLEVA group, 90.9 % (n = 20) of patients responded. The response rate was higher in the PLEVA group than the PLC group (P = .004). In conclusion, erythromycin is effective and safe in the treatment of children with PL, and erythromycin was more effective in patients with PLEVA than PLC.
Topics: Child; Erythromycin; Humans; Pityriasis Lichenoides
PubMed: 32174014
DOI: 10.1111/dth.13311