Resident Rounds. Part III. CDS Protocol SAPHO Syndrome

November 2013 | Volume 12 | Issue 11 | Feature | 1294 | Copyright © 2013

Gunilla Carlsson Thorn MD, Shatil Amin MD, and Jonathan Cotliar MD

Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, IL

Abstract

No abstract available

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INTRODUCTION

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

Medications included insulin, metformin, and ranitidine.

Allergies

There were no known drug allergies.

Family History

Patient reported several family members with “bad skin”.

Social History

The patient reported a social history of two to three alcoholic drinks per week, 1 pack of cigarettes per week, distant history of heroin.

Physical Exam

The patient’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.

LABS/Imaging

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.

Histopathology

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.

Diagnosis

The diagnosis was SAPHO syndrome.

Treatment and Course

Our preferred treatment strategy is to start adalimumab; however, the patient’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 acetonide suspension.

DISCUSSION

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,

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