Successful treatment of ulerythema ophryogenes with pulsed dye laser: a case report

Introduction

Ulerythema ophryogenes (UO) or keratosis pilaris rubra atrophicans faciei (KPAF) is a rare cutaneous disorder characterized by inflammatory keratotic papules that primarily affect the face. The inflammatory process in UO may eventually result in scars, atrophy, and alopecia (1).

UO occurs in childhood or early adulthood in association with various congenital syndromes. Hereditary UO is probably due to an irregular autosomal dominant inheritance and associations with Noonan syndrome have been described several times (1,2). It ends with complete atrichia of the eyebrow region. For the acquired ulerythema ophyrogenes, its occurrence during therapy with nilotinib has been demonstrated (3,4). The areas affected by the disease are the eyebrow area, cheeks, hairline, and beard area. It always appears symmetrically on both eyebrow regions, two-dimensional, initially limited to the lateral eyebrows. Symptoms manifest as redness and horny keratin plugs which are interspersed with erythema, producing a cosmetically disfiguring appearance. Prolonged persistence of this condition causes a gradual loss of the eyebrows, starting from the sides. Even the hair follicles eventually disappear completely (5).

Topical keratolytics containing lactic acid, salicylic acid or urea, vitamin D₃ analogues, antibiotics, and retinoid agents are used with only mild temporary improvement (6) and limited results (7).

Nevertheless, there is a dearth of data regarding systemic medication use in keratosis pilaris (KP) and its subtype (8). Laser therapy is a treatment option for KP patients who are refractory to topical medications (5). Particularly, the use of pulsed dye laser (PDL) and intense pulsed laser has been described, with encouraging results (7,9). The majority of research on lasers with wavelengths less than 600 nm emphasizes PDL application, which has been shown to be effective in treating KPAF (10-13).

However, the literature on this rare condition does not include many clinical cases. On this premise, the current article sheds light on the evaluation of the response of UO symptoms in a young female patient to PDL treatment in terms of erythema, alopecia and skin roughness improvements, as well as treatment tolerance. This clinical case may enrich and provide an interesting literature overview on the laser therapy for UO, which represents a rare skin illness difficult to treat. For this reason, the current study deserves to be reported as it reflects a promising treatment tool for UO for which few literature data are available. This case report is written following the CARE reporting checklist (available at https://acr.amegroups.com/article/view/10.21037/acr-24-215/rc).

Case presentation

We report a case of a 25-year-old female patient having an UO family history and who presented to our dermatology clinic for progressive erythema and gradual diffuse thinning of eyebrow hair that has been evident since childhood. Despite being misdiagnosed with alopecia areata (AA) by several physicians, she never received treatment for this disorder. The patient’s previous medical history is insignificant, and she had not been taking any medications. Cutaneous eyebrow examination revealed symmetrical well-defined confluent horny follicular erythematous papules with areas of alopecia around the lateral third of eyebrows without signs of pitted or atrophic scars. She was complaining of discomfort in the involved skin areas. Based on cutaneous examination and the clinical picture, we diagnosed the patient with UO.

The patient underwent a single session of dye laser (Synchro VasQ, DEKA M.E.L.A, Calenzano, Italy), with the following parameters: spot size 10 mm, fluence 10 J/cm², single pulse (0.5–3 ms). The timeline of clinical case history is represented in Figure 1.

Figure 1 Timeline of clinical case history. UO, ulerythema ophryogenes.

The use of anaesthetic was avoided. Only an external cooling system (Burian PRO, DEKA M.E.L.A, Calenzano, Italy) setted at 15 ℃ was used during the sessions. The Burian PRO cooling system can provide cooling between −4 and −30 ℃ and operates continuously for extended periods of time. Furthermore, this cooling system is equipped with a high-speed fan that increases the air flow for improved cooling effects, and with 10 cooling speed levels adaptable to various treatments.

As post-operative therapy, a healing and soothing cream with hyaluronic acid was applied to the treated areas after careful cleaning of the wounds with gauze and saline solution. The patient was advised to limit further photodamage by frequently applying sunscreen with a 50-sun protection factor and scheduled for regular follow-up visits.

All procedures performed in this study were in accordance the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

After only one therapy session of dye laser, the result was clinically optimal. A visible reduction in roughness and a partial hair regrowth in the treated area were observed at 2 months follow-up after treatment (as shown in Figure 2). The horn plugs were no longer evident, and the erythema had subsided. The hairs had become thicker, darker, and more numerous, covering most of the underlying dermatitis.

Figure 2 Clinical images of the female patient presenting erythema and areas of alopecia in the outer parts of her eyebrows. Pictures were acquired before [(A) frontal view; (C) left lateral view; (E) right lateral view] and at 2 months follow-up after the laser treatment session [(B) frontal view; (D) left lateral view; (F) right lateral view]. The images are published with the patient’s consent.

Following the laser procedure, the patient didn’t experience complications or severe adverse reactions.

Discussion

Our findings show that the investigated device represents a successful treatment for this condition, indicating that it might result in prolonged periods of improvement. After only one dye laser session, a visible reduction in roughness and partial hair regrowth in the treated area were observed at 2 months follow-up after treatment, without pain or discomfort perceived by the patient.

PDL has been widely used in vascular diseases (14-17). Furthermore, recent publications (18,19) demonstrated that the use of dye laser alone or combined with CO₂ laser has been proven to be a safe and effective treatment for multiple facial angiofibromas in young patients affected by tuberous sclerosis and for the successful management of Darier disease (DD).

Specifically, in the work of Sannino et al. (18), a clinical case of DD with extended and typical keratotic and eroded skin lesions in the axillary area led to optimal results following only two sessions with the dye laser system. The skin was almost fully recovered, and no severe side effects were observed.

The vascular elements of the lesions, as well as PDL’s immunomodulatory properties, may be responsible for the treatment’s effectiveness in an inflammatory disease like DD; it can elicit modest capillary injury and endothelial cell oedema in the dermal connective tissue, which can trigger the cutaneous immune response. Ultimately, this damage generates and releases growth factors and cytokines, which stimulate the production of new collagen (14,20,21).

Indeed, PDL’s effectiveness is assumed to be attributed to the induced apoptosis in vascular endothelial cells and the lowering of vascular endothelial growth factor levels (22). Ibrahim et al. (23) proposed the 810-nm diode laser as an efficient therapy for improving skin texture in KP; nevertheless, they noticed no reduction in baseline erythema.

Instead, similar results to those of our study were obtained on the improvement of erythema after only one dye laser session (10). Nevertheless, few recent literature data are available on the dye laser treatment efficacy to heal KP forms (7,10,11). Our positive clinical results, which involve a visible reduction of redness and a partial hair regrowth in the treated area, reflect the dye laser’s mechanism of action which is represented by the expulsion of keratin plugs from the follicular orifices; in this way, the erythema associated with this condition is attenuated or completely disappears.

This clinical case may enrich and provide an interesting literature overview on the laser therapy for UO, which represents a rare skin illness difficult to treat. The limitations of the current study are represented by the lack of analysis of more cases and the lack of long-term prognosis outcomes. Planning for a longer follow-up and recruiting more patients could represent an interesting approach to improve the study outcomes.

Conclusions

PDL treatment appears to be a safe and effective treatment for the erythema and alopecia symptoms associated with this refractory condition. Further studies with the analysis of more cases could be useful to improve the study findings. Furthermore, additional investigations with a longer follow-up will be required to verify the healing progression and to determine whether an early reduction in erythema in UO prevents or reduces the longer-term risk of atrophy and hair loss, as well as whether recurrence occurs over time.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-24-215/rc

Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-24-215/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-24-215/coif). I.F. and T.Z. report that they were employed by El.En. Group. The other authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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