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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 20  |  Issue : 2  |  Page : 175-177

A rare case of acne fulminans


Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India

Date of Submission28-Oct-2018
Date of Acceptance07-Dec-2018
Date of Web Publication10-Jan-2019

Correspondence Address:
Lt Col Preema Sinha
Department of Dermatology, Armed Forces Medical College, Pune - 411 040, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmms.jmms_68_18

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  Abstract 

Acne fulminans (AF) is a rare yet the most severe inflammatory form of acne. It predominantly affects prepubertal boys in the form of multiple exuberant pustular and ulcerative lesions over the face and trunk associated with systemic symptoms in the form of fever, polyarthralgia, and osteolytic bone lesions. In spite of multiple mentioned triggering agents, till date, no direct cause has been ascertained. Drugs like isotretinoin for acne and anabolic steroids are known to precipitate this condition. Due to systemic symptoms, the patient may sometimes require an aggressive inpatient treatment to prevent any systemic complications and grossly morbid scarring as a result of the disease. Herein, we report a rare case of AF in a 16-year-old female with predominantly facial involvement who was managed with oral corticosteroids and isotretinoin with excellent response.

Keywords: Acne fulminans, female, isotretinoin


How to cite this article:
Sinha P, Tripathy DM, Radhakrishnan S, Das P. A rare case of acne fulminans. J Mar Med Soc 2018;20:175-7

How to cite this URL:
Sinha P, Tripathy DM, Radhakrishnan S, Das P. A rare case of acne fulminans. J Mar Med Soc [serial online] 2018 [cited 2019 May 23];20:175-7. Available from: http://www.marinemedicalsociety.in/text.asp?2018/20/2/175/249768


  Introduction Top


Acne fulminans (AF), also called acne maligna, was initially described by Kelly and Burns in 1971, but as a distinct entity was coined by Plewig and Kligman.[1] Only around 200 odd cases have been reported in literature till date.[2] The condition has been mainly described in young adolescent males, with very few reports in females. The incidence of de novo occurring AF has gone down drastically and generally, most reported cases are following drugs such as anabolic steroids and isotretinoin.[3] Even the incidence of disease with systemic symptoms and a fulminant course is on a decline. Many associations in the form of multiple pyogenic granulomas, bone cysts, and Propionibacterium acnes colonization and a plethora of genetic and immunological factors have been implicated. Herein, we discuss this case in a female which was triggered by oral isotretinoin.


  Case Report Top


A 16-year-old girl, with no previous known comorbidities, presented with complaints of multiple red raised lesions over both cheeks and forehead of 2 months duration with recent exacerbation in the form of large nodulocystic lesions along with a fever of 5 days duration. She had a history of the appearance of multiple painful red raised as well as few pus-filled lesions over both cheeks and forehead for the last 2 months. She consulted a dermatologist who managed her as a case of acne vulgaris with both oral antibiotics (tablet azithromycin) and topical therapy in the form of benzoyl peroxide gel. She could not get much relief with the above treatment for which she was started on oral isotretinoin at the dose of 30 mg daily. Fifteen days later, her lesions not only increased in size and number, but also developed ulcerations. She also complained of fever, polyarthralgia and severe pain over her face and neck for 5 days. At this time, she reported to our center. There was no history of taking iodine-containing native treatments or any similar episodes in the past or any similar complaints in her family members.

On examination, the patient was averagely built and nourished. General examination revealed tachycardia with a temperature of 100.8°F.

Dermatological examination revealed the involvement of face in the form of multiple discrete-to-confluent, erythematous indurated plaques and cysts over the bilateral malar region, forehead, and bilateral temporal and mandibular regions, with the overlying skin studded with multiple pinhead-sized pustules at places [Figure 1]. Hemorrhagic crusted plaques were also seen at places. Rest of the dermatological examination including the scalp, palms, soles, hair, nails, and mucosa was normal. Musculoskeletal examination was normal with no joint swelling, effusion, or movement restriction.
Figure 1: Involvement of the face in the form of discrete confluent, erythematous indurated plaques and cysts over the bilateral malar region, forehead, and both temporal and mandibular regions studded with multiple pinhead-sized pustules

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Positive hematological and biochemical investigations revealed a raised total leukocyte count of 13,900/cumm with a differential leukocyte count of P80 L16E03M02 and erythrocyte sedimentation rate of 34 mm fall at the end of the 1st h. Pus swab from multiple sites over the face showed no growth after 48 h of incubation. Urine examination did not reveal any abnormalities. Imaging was not done as the patient did not have any specific clinical findings on joint examination.

The patient was managed as a case of AF with oral prednisolone at a dose of 0.5 mg/kg body weight along with tablet paracetamol 500 mg 6 hourly. Topical therapy was administered in the form of normal saline compresses and topical mupirocin ointment applications thrice daily. She responded well to the treatment with significant improvement of the existing lesions with no occurrence of fresh lesions. Her oral steroids were tapered off very slowly (5 mg every 2 weeks) over 4 months, and oral isotretinoin 20 mg daily was introduced after 6 weeks of oral steroids. Isotretinoin has been tapered off after 7 months of treatment. Presently, she is under follow-up with no relapse of skin lesions 6 months after stopping therapy [Figure 2].
Figure 2: Significant regression of lesions over the face after 9 months of therapy

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  Discussion Top


AF is an uncommon, poorly understood, severe variant of inflammatory acne with an acute clinical deterioration of lesions with the development of necrotic papules and ulcerations associated with systemic signs such as fever and polyarthralgia, leading to severe and disfiguring scars.[1] Causative factors include genetic predisposition, increased serum testosterone levels, and the role of drugs such as anabolic steroids and isotretinoin.[2],[3] Association with Marfan syndrome and late-onset congenital adrenal hyperplasia has been mentioned.[4] Other differential diagnoses include acne conglobata, Gram-negative folliculitis, and pyoderma faciale. Acne conglobata is generally seen in males with a chronic course presenting with comedones, nodules, abscesses, and draining sinus tracts affecting the face, neck, and trunk. Pyoderma faciale, also known as rosacea fulminans, generally affects females and is characterized by papules, pustules, cysts, and coalescing nodules on a background of red cyanotic facial erythema with absent comedones. Gram-negative folliculitis occurs in patients of acne or rosacea who have received long-term oral and topical antibiotics in the form of multiple superficial pustules and nodules. Our patient had involvement of the face with the presence of multiple nodular and crusted lesions along with fever and arthralgia, which was clinically akin to AF.

Few syndromes sharing the association of both joint disease and severe acne include SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis); PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne); and PAPASH (pyoderma gangrenosum, acne, and hidradenitis suppurativa).[1],[2]

Pathogenesis of such a fulminant course of this acne variant is yet to be fully elucidated. Many associations have been described in literature, among which the role of an infective agent and immunological switch could aptly explain the pathogenesis.

Suggested mechanisms include alterations in autoimmunity, innate immunity, and adaptive immunity and a role for autoinflammation, keeping the various auto-inflammatory syndromes in mind.[2]

P. acnes activates inflammasomes in the skin leading to interleukin-1 release.

Retinoids are known to increase skin fragility through a reduction in the number of tonofilaments and desmosomal attachments with secondary accumulation of epidermal amorphous material.

The induced skin fragility of the pilosebaceous duct epithelium allows enhanced interaction of P. acnes antigens and chemoattractants with the immune system. This results in cytokine and mediator release, especially neutrophil chemotaxis, which may be responsible for the early flares seen on treatment with isotretinoin.[5] Retinoids are also known to stimulate excessive granulation tissue, which can lead to pyogenic granuloma formation.[6]

The mainstay of treatment are the oral corticosteroids at a dose of 0.5–1 mg/kg body weight along with oral or parenteral broad-spectrum antibiotics and meticulous topical therapy in the form of saline compresses and topical antibiotics. Corticosteroids should be continued alone till the crusted lesions have healed, and then isotretinoin at a low dose should be added preferably after 4–6 weeks.[7] The overlap of steroid and isotretinoin should be maintained for 1–2 months, and then the steroids are gradually tapered off and isotretinoin is gradually increased. Generally, a 3 to 4-month course of oral steroids is required, like in our case. Indications for systemic steroids in acne patients include severe acne variants such as AF and acne conglobate. Patients of AF need to be administered isotretinoin for a longer duration than in normal acne patients. Other drugs used for this rare condition include ciclosporin and infliximab.[8],[9]


  Conclusion Top


We report this case for its rarity as the occurrence of the disease in a female triggered by oral isotretinoin is not commonly seen. Second, in spite of the dramatic course, the patient recovered well with minimal scarring posttherapy of 9 months.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Karvonen SL. Acne fulminans: Report of clinical findings and treatment of twenty-four patients. J Am Acad Dermatol 1993;28:572-9.  Back to cited text no. 1
    
2.
Greywal T, Zaenglein AL, Baldwin HE, Bhatia N, Chernoff KA, Del Rosso JQ, et al. Evidence-based recommendations for the management of acne fulminans and its variants. J Am Acad Dermatol 2017;77:109-17.  Back to cited text no. 2
    
3.
Kraus SL, Emmert S, Schön MP, Haenssle HA. The dark side of beauty: Acne fulminans induced by anabolic steroids in a male bodybuilder. Arch Dermatol 2012;148:1210-2.  Back to cited text no. 3
    
4.
Placzek M, Degitz K, Schmidt H, Plewig G. Acne fulminans in late-onset congenital adrenal hyperplasia. Lancet 1999;354:739-40.  Back to cited text no. 4
    
5.
Li AW, Antaya RJ. Isotretinoin-induced acne fulminans without systemic symptoms with concurrent exuberant granulation tissue. Pediatr Dermatol 2018;35:257-8.  Back to cited text no. 5
    
6.
Campbell JP, Grekin RC, Ellis CN, Matsuda-John SS, Swanson NA, Voorhees JJ, et al. Retinoid therapy is associated with excess granulation tissue responses. J Am Acad Dermatol 1983;9:708-13.  Back to cited text no. 6
    
7.
Dessinioti C, Katsambas A. Difficult and rare forms of acne. Clin Dermatol 2017;35:138-46.  Back to cited text no. 7
    
8.
Giavedoni P, Mascaró-Galy JM, Aguilera P, Estrach-Panella T. Acne fulminans successfully treated with cyclosporine and isotretinoin. J Am Acad Dermatol 2014;70:e38-9.  Back to cited text no. 8
    
9.
Iqbal M, Kolodney MS. Acne fulminans with synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome treated with infliximab. J Am Acad Dermatol 2005;52:S118-20.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2]



 

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