Darier\'s disease (DD) is an autosomal dominant genodermatosis characterized by hyperkeratotic papules, often accompanied by scaling and crusting. Managing DD presents significant challenges due to the absence of an effective cure, with only symptom targeting treatments currently available. This study presents a case of refractory DD that showed poor response to established pharmacological treatments but demonstrated improvement with low-dose superficial X-ray radiotherapy (SRT). The radiation was delivered as a single 200 cGy treatment, which visibly improved the condition. Considering the different degrees of side effects, sequelae, and risk of developing radiation-induced cancer after exposure to moderate levels of radiation, it may be considered that we attempt to treat recalcitrant DD initially by applying a low dose of radiation in order to mitigate these undesired side effects. If larger doses or additional courses are necessary due to inadequate response, the risks and benefits must be carefully evaluated and discussed with patients.

OBJECTIVE: To assess the effectiveness and safety of treating erythematotelangiectatic rosacea using fractional radiofrequency (FRF).
METHODS: Twenty patients with a confirmed diagnosis of erythema capillaris rosacea were selected, and one side of each patient\'s face was randomly assigned to receive FRF treatments for three to six times, with an interval of 2 weeks between each treatment. VISIA, dermoscopy, and the Clinician\'s Erythema Evaluation Scale (CEA) were applied to evaluate the efficacy of the treatment before and after the treatment, to record the VAS scores and adverse reactions, and to conduct a patient satisfaction survey.
RESULTS: The characteristic counts and scores of red zone and porphyrin as assessed by VISIA test were significantly decreased, and the difference between the treated side and the pretreatment side was statistically significant (p < 0.05), and the efficacy of the treatment was statistically insignificant compared with the control side, except for the red zone and porphyrin which were statistically significant before and after the treatment (p > 0.05). By CEA score, the difference between the treated side after treatment and the control side was statistically significant (p < 0.05), and the difference between the treated side before and after treatment was statistically significant (p < 0.05); the difference between the control side before and after treatment was not statistically significant (p > 0.05). Dermatoscopic observation showed reduction in pore size, reduction of yellowish-white and black horn plugs within the pores, lightening of the red background and thinning and blurring of the capillary structure on the treated side of the skin compared to the control side, and the skin on the treated side showed the above mentioned changes before and after the treatment as well. The mean pain score of the subjects was obtained by VAS score 3.67 ± 0.90. Adverse effects included mild edema, erythema, and microscopic crusting; no long-term adverse effects were seen in all patients. The efficacy of FRF treatment was evaluated 1 month after the final treatment, and 85% of the subjects rated it as satisfactory, very satisfactory, and very satisfactory.
CONCLUSIONS: FRF for the treatment of erythematous capillary dilatation rosacea is effective, safe, and suitable for clinical promotion.

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BACKGROUND: Darier disease (DD) is a rare autosomal dominant disorder that primarily manifests as hyperkeratotic papules and itching. The underlying etiology of DD is pathogenic variation in the ATP2A2 gene. However, this disease has a high penetrance but variable expressivity, indicating that patients inheriting the genotype may have different manifestations due to exogenous factors. Meanwhile, a few reports have documented that COVID-19 may be implicated in the flare of DD.
METHODS: A 51-year-old man presented with keratotic papules and scaly erythematous rash on his trunk with pruritus after being infected with COVID-19. Laboratory test results were normal. Histological analysis revealed epidermal hyperkeratosis and intraepidermal lacunae containing dyskeratinized cells. Genetic analysis revealed a novel variant of ATP2A2 (c.815G>A, p.Trp272*), which was considered pathogenic in this case.
METHODS: The patient was diagnosed as having DD.
METHODS: Oral acitretin and topical corticosteroid hormone ointments were used.
RESULTS: The patient achieved complete resolution of symptoms during the 3-month follow-up period.
CONCLUSIONS: We revealed the first novel ATP2A2 variant (c.815G>A, p.Trp272*) in the flare of DD following COVID-19 infection. Additionally, this pathogenic variant enriches the ATP2A2 gene mutation spectrum.

Keratosis follicularis spinulosa decalvans (KFSD) is a rare X-linked hereditary disorder characterized by the triad of follicular hyperkeratosis-photophobia-alopecia. The clinical heterogeneity makes the diagnosis difficult. To investigate the clinicopathologic and trichoscopic features of KFSD and to further clarify the essential requisites for the diagnosis, we conducted a retrospective study of patients with KFSD. The clinical information, histologic features, and trichoscopic findings were evaluated. Eight patients were from seven separate families. Two females were mother and daughter from the same family and the other six patients were male and represented sporadic cases. The average age of onset of alopecia was 21.25 years. Involvement of the scalp hairs leading to progressive scarring alopecia on the midline of the scalp with variable degrees of inflammation was the pathognomonic feature. It typically began after puberty. Vellus hair-associated follicular hyperkeratosis affected all of the patients. However, photophobia was not a constant feature. Histopathologic examination revealed disorders of the hair follicle with an acute-chronic inflammatory response. Follicular changes including fused infundibulum, the protrusion of the outer root sheath into the follicular canal, and a dilatation of the follicles at the isthmus level caused by the occlusion of keratin were observed. The trichoscopic features included perifollicular scaling, tufted hairs, and loss of follicular openings. In conclusion, terminal hair involvement, either scalp hairs, eyebrows, or eyelashes, and the hyperkeratosis of the follicle of vellus hairs is the diagnostic basis of KFSD. We hypothesize that follicular changes in histopathology are the primary event that trigger variable inflammation and further follicular destruction.

Darier\'s disease is a rare, genetically determined dyskeratotic skin disorder. Although many conventional treatments have been reported, management of Darier\'s disease remains challenging. Most patients are at high risk of recurrence during long-term follow-up. Here, we present two patients who were successfully treated with ALA photodynamic therapy (PDT) and ablative 2940 nm Er:YAG fractional laser. Both patients exhibited significant improvements in the affected areas with moderate pain, transient erythema and edema. Remission durations of up to 2 years were observed in both patients after combination treatment. Our findings suggest that the combination of ALA-PDT and 2940 nm fractional Er:YAG laser may be an effective, safe and well-tolerated treatment option for Darier\'s disease.

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Objective To investigate the clinicopathological characteristics of established genital and extragenital lichen sclerosus(LS)and compare the differences between them. Method The clinicopathological data of 55 patients with established genital and extragenital LS diagnosed by pathological examination in the Department of Dermatology of Beijing Hospital were retrospectively analyzed. Results The 55 patients included 11 males and 44 females.Among them,38,15,and 2 patients had genital lesions,extragenital lesions,and both genital and extragenital lesions,respectively.Extragenital LS mainly involved the back(14.55%)and extremities(7.27%).Among the patients,28.30% were asymptomatic,and 73.58% and 24.53% felt itchy and painful,respectively.The asymptomatic patients had a higher proportion in extragenital cases(χ2=31.224,P=0.000),and the itchy symptom was more common in genital LS cases than in extragenital LS cases(χ2=39.073,P=0.000).Vulvar pain only appeared in the patients with genital LS.A total of 57 biopsy specimens from the 55 patients were reviewed,of which 40 were from genital lesions and 17 were from extragenital lesions.In addition to papillary dermal homogenization (100%),the common pathological changes include hyperkeratosis(98.25%),atrophy(50.88%),basal cell liquefaction degeneration (52.63%),dilated blood vessels(64.91%),a perivascular lymphocytic infiltrate(100%),histiocytic infiltrate(85.96%),and lichenoid inflammatory infiltrate(54.39%).Higher proportions of acanthosis (χ2=4.402,P=0.036),dilated blood vessels (χ2=9.330,P=0.002),and eosinophilic infiltrate (χ2=4.162,P=0.041) were observed in genital LS cases.A higher proportion of follicular plugging appeared in extragenital LS cases (χ2=5.076,P=0.024). Conclusion The established genital LS and extragenital LS showed some differences in clinical symptoms and pathological changes including acanthosis,dilated blood vessels,eosinophilic infiltrate,and follicular plugging.

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