14 year old boy with cystic acne, severe arthritis and hypertrophic scars

presented by

David Elpern MD

Williamstown, MA, USA

on March 30, 2005

Dermatologist, The Skin Clinic, Williamstown, MA, USA

Abstract Fourteen year old boy with severe cystic acne or possibly acne fulminans. The course of his acne was complicated by isotretinoin enhancement of muscle and joint pain and the development of hypertrophic scarring.
14 yo boy
8 months
Face and trunk

The patient is a 14 yo boy who presented in July of 2004 with severe cystic acne on the back, chest, shoulders and lower face. He had a history of hypertropic scarring after varicella.

His base line laboratory tests were normal and he was started on isotretinoin 20 mg a day for one week increasing to 40 mg a day after a week. By week three there was some improvement in his acne, but he had developed significant muscle and joint pain. Isotretinoin was cut down to 20 mg, but his pain increased and he was started on prednisone 20 mg b.i.d. and minocycline 100 mg b.i.d. was substituted for the isotretinoin. His joint pain did not abate and he needed a wheel chair to get around. A rheumatologist saw him, diagnosed "arthritis with spondyloarthropathy" and prescribed Vioxx. The minocycline was discontinued and amoxicillin was prescribed.

The patient's joint and muscle pain gradually improved and the acne went into remission over a period of six weeks. However, many of the acne cysts had become hypertrophic scars. These have persisted and are now the patients chief concern. Intralesional triamcinolone 40 mg per cc has not been helpful; neither has Cordran tape.

Physical Examination

Initially severe cystic acne most prominent on chest, back and shoulders. (no photos were taken)
At present he has scores of hypertrophic scars.


Laboratory Data

All studies including ESR and CPK have been normal.



Diagnosis Acne fulminans with flare secondary to isotretinoin
Possible SAPHO Syndrome
Hypertrophic Scarring
Reasons Presented

Discussion of Acne fulminans: It is clear in hindsight that prednisone should have been prescribed at the outset. I failed to appreciate that this was more than cystic acne.


Who has seen the SAPHO syndrome? Is this the correct diagnosis.? It may have been triggered by the isotretinoin but can occur de novo.

Does anyone have any novel suggestions for this patient's hypertrophic scarring?


Karolyi Z, Harhai I, Eros N. , [Dermatologic aspects of SAPHO-syndrome] [Article in Hungarian] Orv Hetil. 2001 Aug 19;142(33):1801-4.

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis) as a new disease entity was first described in 1987. The syndrome is characterized by the presence of pustular dermatoses together with aseptic osteoarticular lesions. The bone involvement includes hyperostosis, aseptic osteomyelitis or arthritis of the anterior chest wall, sacroiliac joints or long bones. Skin diseases include acne conglobata or acne fulminans, palmoplantar pustulosis and hidradenitis suppurativa. Authors describe the dermatological relationship of SAPHO syndrome reporting their 7 cases (3 acne fulminans, 4 palmoplantar pustulosis). Authors draw attention to the isotretinoin therapy as a possible provoking factor of the articular symptoms, and they emphasize the diagnostic role of bone scintigraphy.

Jemec GB, Rasmussen I. , Bone lesions of acne fulminans. Case report and review of the literature. J Am Acad Dermatol. 1989 Feb;20(2 Pt 2):353-7. Department of Dermatology, Rigshospitalet, University of Copenhagen, Denmark.

Acne fulminans, which is the most severe form of acne, affects mainly teenage men; its pathogenesis is unknown. In its most severe form bone lesions may occur, and although they appear to be transient, they cause considerable discomfort to the patient. Roentgenologically these lesions are not well defined, and often biopsies are performed to rule out malignancy or infection. The result of bone biopsies has, however, always been reported to be benign, as they are in this case. We report a case of acne fulminans with a distinct presentation of bone lesion in a technetium 99 scan and findings of high levels of C3 and C4 and low levels of serum estradiol. These changes have not been described before and are contrary to previously published findings.

Comments from Faculty and Members

Thomas Jayakar MD, PhD , Chennai, India on Apr 4, 2005

The use of oral isotretinoin is stongly associated with SAPHO syndrome. All the components have well been recognized in clinical practice, but compiling them into a syndrome was in 1987.
Presently there is one more feature that frequently appears with these features, namely depression. This symptom is very high in frequency as an isolated finding, although I do find it associated with the components of SAPHO. Frequent findings are fever, arthritis, acne, and depression, the so called FAAD syndrome, hitherto undescribed. Any takers for this? Expert comments are solicited.

Khalifa Shaquie MD, PhD, Professor of Dermatology, College of Medicine, University of Baghdad, Baghdad, Iraq on Apr 4, 2005

This is an interesting case of acne fulminans or conglobata. In countries where isotretinoin is either very expensive or not available like in Iraq, we usaully manage by giving oral cotrimoxazole together with antiandrogen like finesteride or spironolactone and 10mg of prednisolone. Patients respond usually to this regime. If the patient has arthritis ,we combine with non-steroidal anti-inflammatory agents.

Shahbaz Janjua MD, Lalamusa, Pakistan on Apr 13, 2005

SAPHO has no clear etiology but the skin manifestations common to all variants could act as trigger to cause the reactive inflammation of the synovium, osteitis, pustulosis and hyperostosis. This could only be a hypothesis but I still am not covinced that it was isotretinoin that triggered the SAPHO in this patient because isotretinoin is frequently prescribed for cystic acne. SAPHO could only be a coincidence. But I agree that in cases of acne fulminans, oral steroids are the mainstay of treatment.

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