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Journal of the American Academy of... Jun 2022Data regarding Asian patients with mycosis fungoides (MF) are limited.
BACKGROUND
Data regarding Asian patients with mycosis fungoides (MF) are limited.
OBJECTIVE
We aimed to investigate the clinical profile and long-term outcomes of patients with MF in Korea.
METHODS
A retrospective review of 223 patients with MF who were followed up for more than 6 months or died of MF within 6 months of diagnosis was performed.
RESULTS
Approximately 96.4% and 3.6% of the patients had an early stage and advanced stage, respectively. The mean age at diagnosis was 44.8 years. The mean duration of symptoms before diagnosis was 47.0 months. Various subtypes were noted, including mycosis fungoides palmaris et plantaris (21.5%), folliculotropic (8.5%), pityriasis lichenoides-like (6.7%), ichthyosiform (4.0%), lichenoid purpura-like (2.7%), and hypopigmented (2.2%) MF. Juvenile patients accounted for 16.6%. The higher the skin T stage, the poorer the response to treatment. The 10-year overall survival was 96.8% in early-stage patients and 25.0% in advanced-stage patients. General prognosis was favorable, while recurrence and subtype switching were seen in 29.4% and 2.7% of patients, respectively.
LIMITATIONS
Our patients may not represent all Korean patients with MF.
CONCLUSION
MF in Korea has a high proportion of variants, a younger age at onset, and favorable prognosis. A high index of suspicion and skin biopsy are needed for early diagnosis.
Topics: Biopsy; Humans; Mycosis Fungoides; Pityriasis Lichenoides; Prognosis; Retrospective Studies; Skin; Skin Neoplasms
PubMed: 34197872
DOI: 10.1016/j.jaad.2021.06.860 -
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 -
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 -
Journal of Cutaneous Pathology Sep 2021Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare congenital malformation of the dermis and subcutaneous tissue. Usually, RMH occurs in the midline of the face and...
Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare congenital malformation of the dermis and subcutaneous tissue. Usually, RMH occurs in the midline of the face and neck region. We described a case of RMH presenting as telangiectasia in a 57-year-old man with a history of pityriasis lichenoides chronicus. Histopathological examination revealed a subepidermal haphazard proliferation of striated muscular tissue perpendicular to the epidermis. These bundles of striated muscular tissue were admixed with adnexal structures. The diagnosis was consistent with RMH. RMH is more common in the neonatal period or in young children, but we should consider it as part of a differential diagnosis in older adults as well.
Topics: Desmin; Diagnosis, Differential; Hamartoma; Humans; Male; Mesoderm; Middle Aged; Pityriasis Lichenoides; Rhabdomyoma; Soft Tissue Neoplasms; Telangiectasis; Watchful Waiting
PubMed: 33964023
DOI: 10.1111/cup.14044 -
International Journal of Dermatology Dec 2021
Topics: Humans; Lymphoma; Pityriasis Lichenoides; Waldenstrom Macroglobulinemia
PubMed: 33934331
DOI: 10.1111/ijd.15636 -
Annals of Dermatology Feb 2021
PubMed: 33911821
DOI: 10.5021/ad.2021.33.1.94 -
JAAD Case Reports Jun 2021
PubMed: 33898675
DOI: 10.1016/j.jdcr.2021.03.046 -
The American Journal of Dermatopathology Dec 2021A 15-year-old boy presented to the pediatric dermatology department with long-standing patch stage CD8+ mycosis fungoides and subsequent development of recurrent...
A 15-year-old boy presented to the pediatric dermatology department with long-standing patch stage CD8+ mycosis fungoides and subsequent development of recurrent pityriasis lichenoides et varioliformis acuta eruptions. There have been rare reports of patients with chronic, recalcitrant pityriasis lichenoides developing mycosis fungoides, but we believe this to be the second case of mycosis fungoides preceding a diagnosis of pityriasis lichenoides, and the first case reported in the pediatric population.
Topics: Adolescent; Humans; Male; Mycosis Fungoides; Pityriasis Lichenoides; Skin Neoplasms
PubMed: 33795555
DOI: 10.1097/DAD.0000000000001949 -
Case Reports in Dermatology 2021Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by...
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by intermittent pyrexia, acute onset of generalized ulceronecrotic lesions, and histopathology suggestive of PLEVA. Prompt diagnosis and treatment are necessary to halt the progression of this potentially fatal disease; however, the widely variable presentation of FUMHD in addition to its rarity poses a diagnostic challenge. We report the case of a previously healthy 43-year-old woman who presented to the emergency department with 1 month of generalized rash and intermittent fevers. Her only reported new exposure were elective intravenous vitamin infusions received at a medi-spa 1 week prior to onset of lesions. Initial evaluations were inconclusive, and confluent ulceronecrotic, hemorrhagic lesions appeared on approximately 90% of her body despite steroid, antibiotic, and cyclosporine therapy. Repeat histopathology was consistent with PLEVA, and in the context of her clinical presentation she was diagnosed with FUMHD. The patient rapidly attained remission with methotrexate therapy but sustained residual scarring.
PubMed: 33613228
DOI: 10.1159/000511537 -
The Journal of Dermatology Nov 2020
Topics: Dermatomyositis; Humans; Lung Diseases, Interstitial; Pityriasis Lichenoides
PubMed: 33464622
DOI: 10.1111/1346-8138.15556