Sweet syndrome with osseous and splenic involvement: A case report

Abstract

Sweet syndrome is an uncommon inflammatory skin condition. Here we present a case of Sweet syndrome in a young woman with rare extracutaneous manifestations, including bone and splenic fluid collections, with marked improvement following treatment with systemic corticosteroids. The patient was subsequently diagnosed with Crohn's disease which can be seen in the setting of Sweet syndrome. Sterile abscesses should be considered in patients with a clinical diagnosis of Sweet syndrome and focal symptomatology.

Keywords: AFB, acid fast bacilli; ANA, antinuclear antibodies; Abscess; Acute febrile neutrophilic dermatosis; CRMO, chronic recurrent multifocal osteomyelitis; CRP, c-reactive protein; Crohn's disease; EKG, electrocardiogram; ESR, erythrocyte sedimentation rate; GI, gastrointestinal; H&E, hematoxylin and eosin; Osteomyelitis; PICC, peripherally inserted central catheter; RF, rheumatoid factor; SS, Sweet syndrome; Sweet syndrome; WBC, white blood cell; p-ANCA, perinuclear-antineutrophil cytoplasmic antibodies.

Fig. 1

(A) Right lateral ankle unruptured fluid collection and surrounding erythema. (B) Right lateral ankle and foot swelling with spontaneous rupture of fluid collections.

Fig. 2

Left lateral ankle and foot swelling with spontaneous rupture of fluid collection over the left ankle.

Fig. 3

Right distal forearm erythematous plaque.

Fig. 4

Axial CT with IV contrast demonstrating multiple round fluid collections in the spleen (arrows).

Fig. 5

Subsequent chest radiograph demonstrated a moderate left pleural effusion. A right upper extremity PICC and multiple EKG leads are present.

Fig. 6

(A) Sagittal T2 fat sat image from baseline MRI of the left foot shows focal hyperintensity within the posterior calcaneus with surrounding ill-defined marrow edema pattern (orange arrow). This is not associated with the Achilles tendon or plantar fascia attachments. A focal fluid collection is seen in the dorsal soft tissues of the forefoot with traversing extensor tendons (white arrow). Edema is noted along Kager's fat pad (blue arrow). (B) Axial T2 fat sat image from baseline MRI of the left foot confirms focal marrow signal abnormality in the posterior calcaneus (orange arrow). Fluid collections are seen in the forefoot medially and laterally (white arrows). Subcutaneous edema is present laterally (blue arrow). (C) Axial T2 fat sat image from baseline MRI of the left foot demonstrates a focal fluid collection posterior to the lateral malleolus (arrow). Diffuse subcutaneous edema is noted (Color version of the figure is available online.)

Fig. 7

Sagittal T2 fat sat image from MRI of the right foot demonstrates edema-like marrow signal intensity in the hallux distal phalanx (orange arrow). Multiple fluid collections are noted in the forefoot (white arrows) (Color version of the figure is available online.)

Fig. 8

(A) Sagittal T2 fat sat image from follow up MRI of the left foot (3 months after the MRI shown in Fig. 6) demonstrates persistent focal marrow signal hyperintensity in the posterior calcaneus (arrow), although with interval resolution of surrounding marrow edema pattern and resolution of the previously noted soft tissue fluid collection. (B) Axial T2 fat sat image from follow up MRI of the left foot demonstrates complete resolution of the fluid collection that was previously seen posterior to the lateral malleolus.

Fig. 9

(A) Low power magnification of right foot punch biopsy showing epidermal edema (spongiosis) (arrow) and dense dermal perivascular and interstitial (arrowhead) inflammatory infiltrate with abundant neutrophils (H&E, 40x). (B) Higher power magnification of the dermal perivascular (arrow) and interstitial (arrowhead) inflammatory infiltrate with abundant neutrophils, nuclear debris, and no eosinophils (H&E, 200x).

Fig 10

(A) Low power magnification of right colon biopsy showing chronic changes including shortened forked glands (arrow) with drop out and irregular distribution. There is a heavy chronic inflammatory infiltrate in the lamina propria (H&E, 40x). (B) High power magnification of right colon biopsy showing active inflammation including gland cryptitis (arrow) and crypt abscesses (arrowhead) (H&E, 400x).