History of Present Illness
A 57 year-old African American man presented with a 30-
year history of painful skin lesions. The patient noted
that the lesions begin as tender knots, which drain purulent
fluid and become thick and crusted over time. The patient
also noted a distant history of severe scarring acne as well
as persistent bone pain, joint pain, and morning stiffness.
Past Medical History
Past medical history included diabetes mellitus, anemia, and
benign prostatic hypertrophy.
Medications included insulin, metformin, and ranitidine.
There were no known drug allergies.
Patient reported several family members with â€œbad skinâ€.
The patient reported a social history of two to three alcoholic
drinks per week, 1 pack of cigarettes per week, distant history
The patientâ€™s cutaneous exam was notable for several patches
of scarring alopecia on the scalp and deep cribiform scarring
over the bilateral cheeks, nose and forehead. On the right
temple, left proximal medial arm and left proximal medial
thigh were large fluctuant plaques with overlying cribiform
ulcerations, thick overlying crusting and purulent drainage.
The extremities had wide spread scarring and innumerable
hyperpigmented vegetative nodules. The patient endorsed
tenderness of the sternum to palpation.
Abnormal: Hgb 8.5, tissue culture grew multiple mixed microorganisms
but no predominating pathogens.
Bone Scintigraphy: Extensive periosteal reaction involving both
femurs throughout their length consistent with hyperostosis.
Negative/normal: BMP, LFTs, ANA, RF, HIV, hepatitis serologies,
RPR, quantiferon gold, tissue fungus culture & stain, tissue mycobacteria
culture & smear.
Right thigh: The sections reveal a markedly corrugated epidermis
with irregular proliferative epithelial hyperplastic and
reactive changes without evidence of nuclear atypia. There is
a deep dermal infiltrate composed of lymphocytes, plasma
cells and aggregates of neutrophils. The dermis also shows
variable fibroplasia with some edema and telangiectasia.
Acantholysis is not noted but there is some dyskeratosis associated
with exocytosis of neutrophils. Immunohistochemistry
for Treponema and special stains (DPAS, Gram and acid fast
bacilli) were negative for microorganisms. Direct immunofluorescence
was negative for immune deposits.
The diagnosis was SAPHO syndrome.
Treatment and Course
Our preferred treatment strategy is to start adalimumab;
however, the patientâ€™s insurance denied coverage. We are currently
attempting to get the medication at no cost to the patient
through a patient assistance program. In the meantime, symptomatic
lesions are treated with intralesional triamcinolone
The syndrome of synovitis, acne, pustulosis, hyperostosis,
and osteitis (SAPHO) represents a rare constellation of chronic
overlapping osteoarticular and cutaneous manifestations. The
clinical presentation is heterogeneous and frequently incomplete,
resulting in diagnostic difficulties.
The typical cutaneous manifestations include palmoplantar pustulosis
and severe acne, which can manifest as acne conglobata,