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1.
J Dermatolog Treat ; 32(4): 381-382, 2021 Jun.
Article in English | MEDLINE | ID: mdl-31464543

ABSTRACT

Granuloma annulare is frequently described in association with numerous systemic conditions and is often refractory to treatment. This retrospective review explored a large patient cohort to further strengthen associated comorbid conditions and assess response to different treatments in patients with granuloma annulare. This study found that patients with granuloma annulare frequently carried a diagnosis of hypothyroidism. Additionally, the highest rate of improvement in any treatment category was seen with pentoxifylline use. Thus, should be further explored as a first-line therapeutic option in the treatment of granuloma annulare.


Subject(s)
Granuloma Annulare/drug therapy , Pentoxifylline/therapeutic use , Adolescent , Adult , Aged , Aged, 80 and over , Female , Granuloma Annulare/complications , Humans , Male , Middle Aged , Retrospective Studies , Young Adult
2.
J Am Acad Dermatol ; 79(3): 487-494, 2018 Sep.
Article in English | MEDLINE | ID: mdl-29477734

ABSTRACT

BACKGROUND: Heterozygous mutations in caspase recruitment domain family member 14 gene (CARD14) have been shown to be associated with psoriasis and familial pityriasis rubra pilaris (PRP). Many subjects with CARD14 mutations display features of both disorders, which can result in diagnostic uncertainty. In addition, these eruptions are often recalcitrant to conventional psoriasis therapies such as methotrexate, oral retinoids, and tumor necrosis factor-α inhibitors. OBJECTIVE: We sought to describe the clinical characteristics, family history, and response to therapy in subjects with papulosquamous eruptions due to mutations in CARD14. METHODS: Subjects were referred for genetic testing as part of a registry of subjects with inherited disorders of keratinization. DNA was isolated from blood or saliva, and multiplex targeted sequencing or whole exome sequencing was performed. Clinical histories of subjects with CARD14 mutations were reviewed. RESULTS: We identified 15 kindreds with CARD14-associated papulosquamous eruption (CAPE). Characteristic features of CAPE include early age of onset; prominent involvement of the cheeks, chin, and ears; family history of psoriasis or PRP; minimal response to conventional topical and systemic psoriasis therapies; and improvement with ustekinumab. LIMITATIONS: Relatively small sample size. CONCLUSIONS: Many subjects with CARD14 mutations display characteristics of both psoriasis and PRP. We propose the term CARD14-associated papulosquamous eruption to describe this spectrum of disease. Subjects with clinical features suggestive of CAPE should undergo CARD14 sequencing and may benefit from treatment with ustekinumab.


Subject(s)
CARD Signaling Adaptor Proteins/genetics , Dermatologic Agents/therapeutic use , Facial Dermatoses/genetics , Guanylate Cyclase/genetics , Membrane Proteins/genetics , Skin Diseases, Papulosquamous/drug therapy , Skin Diseases, Papulosquamous/genetics , Ustekinumab/therapeutic use , Age of Onset , Child , Child, Preschool , Genetic Testing , Humans , Infant , Infant, Newborn , Phenotype , Pityriasis Rubra Pilaris/genetics , Psoriasis/genetics , Psoriasis/therapy , Retreatment
4.
J Am Acad Dermatol ; 74(6): 1073-1076.e2, 2016 Jun.
Article in English | MEDLINE | ID: mdl-27185423

ABSTRACT

BACKGROUND: Mucosal lichen planus (MLP) is a therapeutic challenge in need of a new treatment approach because of its debilitating effect on patient's quality of life. OBJECTIVE: We sought to evaluate a standardized treatment plan for patients with MLP. A second objective was to describe the effect of mycophenolate mofetil in this patient population. METHODS: The study retrospectively analyzed 53 patients with MLP treated using a standardized algorithm. The number of MLP lesions, disease activity, and pain at the last visit were compared with baseline scores determined at the initial visit. Results were analyzed using the paired samples t test and confirmed with the Wilcoxon matched pairs signed rank test. RESULTS: The average number of lesions was reduced from 3.77 to 1.67 (P < .001). The average disease activity was reduced from 2.73 to 0.90 (P < .001). Average pain reported decreased from 2.03 to 1.03 (P < .001). LIMITATIONS: This study was a retrospective analysis of a small patient population. There was no universal symptom severity scale used at the time of treatment for some patients. CONCLUSION: The standardized treatment plan reduced symptoms for patients with MLP. Mycophenolate mofetil appears to be a reasonable treatment option for these patients.


Subject(s)
Algorithms , Lichen Planus/diagnosis , Lichen Planus/drug therapy , Mycophenolic Acid/therapeutic use , Quality of Life , Adult , Aged , Aged, 80 and over , Cohort Studies , Dose-Response Relationship, Drug , Drug Administration Schedule , Female , Humans , Incidence , Lichen Planus/epidemiology , Lichen Planus, Oral/diagnosis , Lichen Planus, Oral/drug therapy , Lichen Planus, Oral/epidemiology , Male , Middle Aged , Mouth Mucosa/pathology , Mucous Membrane/pathology , Prognosis , Retrospective Studies , Risk Assessment , Severity of Illness Index , Statistics, Nonparametric , Treatment Outcome
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