Case Presentation-Dermatology Kevin T. Belasco, MSIV Touro Univ. College of Osteopathic Medicine May 24, 2004

Case Presentation 32 y/o Caucasian female presents to community clinic with a chief complaint of fever, rash, malaise, and arthralgia with sudden onset beginning 4 days ago HPI: Patient returned 6 days ago from a three month-long church mission to Cambodia, where she taught English. She states her diet was composed largely of chicken and rice, with coconut milk and purified bottled water.

Case Presentation (Cont.) HPI (Cont.)Patient states she developed a cough with yellow sputum 3 weeks ago and was treated with a tetracycline antibiotic. She denies any headache, dizzines, chills, night sweats, N/V, diarrhea, or hematuria. She also denies any sick contacts PMHx/PSHx: Tonsillectomy 1982, Cholecystectomy PMHx otherwise non-contributory Allergies: NKDA Meds: MVI, Tylenol prn muscle aches SocHx: Denies smoking, EtOH, or illicit drug use. Lives with boyfriend in Los Angeles; schoolteacher

Case Presentation (Cont.) ROS: as per HPI; fever, rash, arthralgia, and malaise VS: T 100.9, P 95 R 22 BP 133/84 PE: Systems normal except described below: Gen: WD/WN, pleasant female, NAD, AAO x 3 HEENT: NC/AT, EOMI, no conjunctival injection, no pharyngeal erythema or exudate, no oral lesions Skin: sharply demarcated, painful plaques with erythema and pustules on the upper trunk, neck, and face Musculoskeletal: Mild erythema and swelling with tenderness to palpation over left elbow joint

Case Presentation (Cont.) Laboratory values: CBC: 16/14.0/40/343 N74 L20 M3 E1 B0 BMP: 136/4.1/105/28/16/1.1/112 ESR: 40 PPD: negative

Differential Diagnosis Erythema multiforme Drug Eruption, Fixed Cellulitis Pyoderma gangrenosum Granuloma annulare/ faciale Erythema nodosum Behçet's disease

Differential Diagnosis Sweet’s Syndrome Sjögren’s Syndrome Rheumatoid Arthritis (Cutaneous nodules) Systemic Lupus Erythematosus Cutaneous Tuberculosis (Lupus vulgaris) Erythema induratum Subcorneal pustular dermatosis Erythema elevatum et diutinum Leukocytoclastic vasculitis

Summary of Findings Clinical: Painful plaques with pustules with abrupt onset fever and arthralgia; history of recent international travel; recent upper respiratory tract infection Labs: Leukocytosis with neutrophilia and lymphopenia Elevated ESR Negative Gram stain

Sweet’s syndrome Acute febrile neutrophilic dermatosis Initially described in 1964 by Robert Sweet Three types: 1. Classic (Strep., Yersinia, BCG/Pneumococcal vaccine, IBD, pregnancy, idiopathic) 2. Malignancy-associated (AML) 3. Drug- induced (G-CSF, all-trans retinoic acid, minocycline, Bactrim, OCPs, carbamazepine, hydralazine) female-to-male ratio of 2-3:1 several hundred cases have been reported in the literature

Sweet’s syndrome Typically, skin lesions are preceded by URI or GI infection, and only occur after a 1-3 week asymptomatic period Most common in women over 30 Up to 20% cases associated with malignancy

Sweet’s syndrome Tender, well-demarcated erythematous plaques George Wash. Univ. Dermatology Dept

Sweet’s syndrome Pseudovesiculation with pustules and soft elevation in the center- mamillated George Wash. Univ. Dermatology Dept

Sweet’s syndrome Painful erythematous plaques with pustulation

Sweet’s syndrome: Histopathology

Sweet’s syndrome: Histopathology Skin biopsy reveals dermal neutrophilic infiltrate in reticular dermis with leukocytoclasia (fragmentation of neutrophilic nuclei); epidermis is usually spared Associated clinical phenomena: Koebnerization Pathergy (skin lesions at site of trauma)

Sweet’s syndrome: Treatment Prednisone is rapidly effective, in doses ranging from mg/day (initial dose of mg per kg per day) Topical and intralesional corticosteroids have frequently been used as adjunctive treatment along with systemic modalities Indomethacin, cyclosporine, dapsone, colchicine, KI, doxycycline, and clofazamine have been reported in the literature as effective alternative treatment modalities

References Habif: Clinical Dermatology, 4 th ed., Mosby 2004 Odom, et al: Andrew’s Diseases of the Skin, 9 th ed., Elsevier 2000 Fitzpatrick, JE: Dermatology Secrets in Color. Hanley & Belfus (Kimball AB review article) dermatology.cdlib.org (Dermatology Online Journal)