Successful Treatment of Coexistent Acne Fulminans and Severe Hidradenitis Suppurativa with Adalimumab

Abstract

The coexistence of hidradenitis suppurativa (HS) and acne fulminans (AF) has only recently been reported in the literature. We present a case of a 17-year-old man who presented with a 2 years history of severe acne and HS. He was initially started on oral clindamycin and rifampicin for 3 months with no clinical improvement. Acne lesions became worse with the presence of nodules and necrotic ulcers, while weight loss, low-grade fever, back and knee pain, and psychological distress were noted. We prescribed adalimumab in its standard dosing regimen. Remission of AF was achieved in 3 months, whereas adalimumab has not been as effective in treating the HS lesions. Its dosage was increased to 80 mg weekly and more than 80% clinical improvement of HS lesions was obtained in 2 months. The patient maintained on this dosage till this day and efficacy is sustained. TNF-α inhibitors are considered an effective option in the treatment of HS, while it has been also suggested as a treatment option in AF. Our patient was successfully treated with adalimumab. Since the coexistence of HS and AF has a devastating emotional effect on the patient, there is an urgent need to implement therapeutic approaches.

Established Facts

• Hidradenitis suppurativa (HS) and acne fulminans (AF) are considered 2 skin conditions difficult to treat, with significant impact on patients' quality of life.

• TNF-α inhibitors have been established as an effective option in the treatment of HS, while they have been also suggested as a treatment option in AF.

Novel Insights

• The coexistence of hidradenitis suppurativa (HS) and acne fulminans (AF) could either represent a potential new syndrome or be related to PASS syndrome (pyoderma gangrenosum, acne, suppurative hidradenitis, and spondyloarthritis), as it is already known that HS can precede pyoderma gangrenosum onset.

• TNF-α inhibitors may be considered as an effective therapeutic approach in patients with coexistent HS and AF unresponsive to previous therapies

Introduction

Acne fulminans (AF) is a rare and severe form of acne. Its coexistence with hidradenitis suppurativa (HS) has recently been reported in the literature. The pathophysiology and the best therapeutic strategy of this condition are only partially known [1, 2]. We report the effectiveness of adalimumab in a patient with HS and AF who was refractory to previous therapies.

Case Report/Case Presentation

A 17-year-old man presented with cystic lesions, erosions, and crusts over his face and back (shown in Fig. 1). He reported acne lesions since adolescence and had been treated unsuccessfully with topical and systemic antibiotics. Examination revealed Hurley stage 3 HS lesions (draining sinuses and abscesses) in the axillae, inguinal, and perianal region (shown in Fig. 2). He reported coexistence of HS and severe acne for the last 2 years. The patient had no relevant family history. Treatment with oral clindamycin and rifampicin was initiated, based on the European guidelines for HS [1]. The therapeutic choice was directed toward HS since its burden was greater than the acne one. The patient did not present any improvement on the HS lesions, while acne lesions exacerbated with development of nodules and necrotic ulcers on a follow up visit after 3 months. He had lost 4 kg of weight and had a low-grade fever, driving us toward a diagnosis of AF. Furthermore, a back and knee pain of sudden onset has been developed. The patient also reported, visiting a psychiatrist who diagnosed anxiety disorder and depression symptoms but did not prescribe any pharmacological treatment. The patient's magnetic resonance imaging indicated a seronegative undifferentiated spondyloarthropathy. Based on previous reports of successful treatment of HS, acne conglobata, seronegative spondyloarthropathy, and SAPHO syndrome by TNF-α antagonist [3], we prescribed adalimumab, a fully human monoclonal IgG1 antibody directed toward membrane-bound TNF-α on the approved dosing regimen of 160 mg at week 0, 80 mg at week 2, followed by 40 mg weekly starting at week 4. Remission of AF was achieved in 3 months (shown in Fig. 3), while back and knee pain subsided. Patient's weight stabilized, and he reported an improved psychological health. Adalimumab has not been as effective in treating the HS lesions, with some of them healing slowly with extensive scarring and others being still active and draining. Therefore, we decided to increase the dosage of adalimumab to 80 mg every week, resulting in 80% clinical improvement of HS lesions after 2 months (shown in Fig. 4). The patient continued on this dosage, for a total of 10 months and efficacy was sustained.

Fig. 1.

Cystic lesions, erosions, and crusts over the patient's back on presentation.

Fig. 2.

Draining sinuses and abscesses in patient's left axillae on the first visit.

Fig. 3.

Remission of AF on the back achieved 3 months post-initiation of ada­limumab treatment. AF, acne fulminans.

Fig. 4.

More than 80% clinical improvement of HS lesions on left axillae obtained 2 months after increasing dosage of ada­limumab to 80 mg per week. HS, hidradenitis suppurativa.

Discussion/Conclusion

AF and HS are considered 2 skin conditions difficult to treat. Their impact on patients' quality of life is usually significant, as marked visible lesions have a negative impact on body image [4, 5]. Their coexistence together with the presence of seronegative spondyloarthropathy could either represent a potential new syndrome or be related to PASS syndrome (pyoderma gangrenosum, acne, suppurative hidradenitis, and spondyloarthritis), as it is already known that pyoderma gangrenosum can appear at any point after the development of HS lesions [6]. Antibiotics are commonly used to treat flares of HS, as secondary bacterial infections occur. In addition, they are considered to have immunomodulatory properties, helping to manage HS [7]. Surgery is often required to definitively treat the tunnels and scars. TNF-α is thought to be a primary driver of HS' inflammatory process. TNF-α concentration is significantly higher in the serum and skin of HS patients compared with healthy volunteers [8]. Adalimumab has the highest quality evidence supporting its use in HS. Its dose intensification in recalcitrant HS has been recently studied, revealing an enhanced level of effectiveness. Therefore, we decided to increase adalimumab's dose when our patient did not respond sufficiently to the registered adalimumab dose. Usually AF treatment consists of isotretinoin therapy combined with systemic steroids. However, in some patients isotretinoin has been considered a triggering factor of AF. TNF-alpha inhibitors have been also suggested as a treatment option in AF [9].

A recent publication described a similar case to ours achieving remission with oral antibiotics (ertapenem through a central catheter, followed by oral rifampin plus moxifloxacin plus metronidazole) [10]. Their efficacy could be explained by the similar microbiota composition in both lesional types in this specific case, identified by bacterial cultures and confirmed by metagenomics. Our patient was treated with adalimumab obtaining good clinical response on both AF and HS lesions and improving the patients' health related quality of life. In addition, we highlight the successful usage of high-dose adalimumab for this auto-inflammatory association when desired improvement has not been achieved with its standard doses. We avoided switching to another treatment option, taking into consideration that TNF-a antagonists (adalimumab and infliximab) are the most effective therapies in HS associated syndromes [11].

Statement of Ethics

The patient has given his written informed consent to publish his case (including publication of images).

Conflict of Interest Statement

The authors have no conflicts of interest to declare.

Funding Sources

The authors did not receive any funding.

Author Contributions

All authors contributed to the design of the work, acquisition, analysis, and interpretation of data, critically revised the manuscript for important intellectual content, and approved the final version.