Clinical Challenges

What is your diagnosis? - December 2018

Publish date: November 30, 2018
By
James H. Tabibian, MD, PhD
Carilyn N. Wieland, MD
Seth Sweetser, MD

AGA Institute

Figure A

A 65-year-old man presented to the dermatology clinic with a diffuse, multifocal, erythematous rash consisting of nonpruritic papules and plaques on the chest (Figure A) and back (Figure A, inset), which became raised and involved the extremities.
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Figure B

He had no significant past medical history, and physical examination was otherwise unremarkable. Laboratory tests were notable for mild anemia and leukocytosis (13 × 109/L). A punch biopsy demonstrated diffuse, dense dermal inflammatory infiltrate composed predominantly of neutrophils, with characteristic dermal edema (Figure B) and bilobed nuclei (Figure B, inset); microbial studies were negative, as were immunostains for lymphoma cutis.
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Figure C

Extensive evaluation for potential systemic or other associated conditions was unrevealing, including upper endoscopy and computed tomography enterography; colonoscopy performed 2 years earlier was reportedly unremarkable but with only fair bowel preparation. The patient was prescribed clobetasol ointment and subsequently oral prednisone, both of which yielded prompt improvement; nevertheless, the rash recurred within 1 week of corticosteroid discontinuation. In addition, over the course of the following 6 months, progressive weight loss, periodic fevers, and abdominal distention were noted. Computed tomography of the abdomen was repeated, revealing a new critical finding (Figure C).

What is the diagnosis?


 

Cecal carcinoma–associated paraneoplastic Sweet’s syndrome

Based on the tomographic appearance of an “apple core”–like lesion in the right lower quadrant, the patient was referred for colonoscopy, which revealed a malignant-appearing cecal mass (Figure D), with biopsies confirming adenocarcinoma; despite these findings, no bowel-related symptoms were reported. The patient underwent laparoscopic right hemicolectomy, after which the skin lesions began to resolve, and corticosteroids were successfully tapered. The overall presentation was consistent with Sweet’s syndrome, with a paraneoplastic etiology being favored given the clinical scenario, including absence of alternative etiologies and dependence on corticosteroids for control of skin disease until resection of the underlying malignancy was performed.

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Figure D

Sweet’s syndrome was first described in a case series of eight patients published in 1964 by the English dermatologist Dr. Robert Douglas Sweet.1,2 Sweet’s syndrome is characterized by fever, neutrophilia, and sterile erythematous plaques or nodules, which most commonly involve the upper extremities and face and respond to corticosteroid therapy. It may be malignancy associated, drug induced, autoimmune disease related, or idiopathic.2,3 The pathogenesis of Sweet’s syndrome is unclear, but T-lymphocyte, neutrophil chemotaxis, and cytokine (e.g., interleukin-6 and granulocyte colony–stimulating factor) abnormalities have been suggested.2 Diagnosis is based on the clinical presentation and context together with typical dermatopathologic findings, including a dense neutrophilic infiltrate. Skin lesions may be phasic, but persist typically until appropriate therapy (e.g., corticosteroids, chemotherapy) is administered or the offending drug removed. Malignancy-associated (i.e., paraneoplastic) Sweet’s syndrome accounts for approximately 20% of all cases; these primarily involve hematologic malignancies, most commonly leukemia, but adenocarcinomata have also been implicated.3 Recurrence of Sweet’s syndrome can occur and often heralds relapse of the underlying disease.

References

1. Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol. 1964;76:349-56.

2. Von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol. 1994;31:535-56.

3. Cohen PR. Sweet’s syndrome–a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007;2:34.

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What is Your Diagnosis? - November 2018