Careers  |  Sign In  |  Register  |   Twitter

45-Year-Old Female with Papular-Pustular Rash on Face and Legs

Can you diagnose this case?

Signs and Symptoms

A 45-year-old woman was admitted to the hospital with a 5-day history of a papular-pustular rash on her face, trunk, and legs. Preceding the rash, she had had a low-grade fever, rhinorrhea, nasal congestion, nonproductive cough, and sinus tenderness.

Review of systems was positive for diffuse arthralgia, myalgia, and generalized malaise. The patient had a past medical history of Crohn disease, which had been well controlled with infliximab for more than 10 years.

At admission, her temperature was 38°C. Physical examination revealed the presence of nontender, erythematous pink papules with overlying pustules, ranging from 10 mm to 1 cm in size, diffusely scattered over her body and in varying stages of maturation (Figures). Some were newly arisen and appeared pustular. Others had erupted and were crusting over. Oral mucosal ulcers also were present.

Significant laboratory values included a white blood cell count of 17,200/µL, a C-reactive protein level of 191 mg/L, and an erythrocyte sedimentation rate of 66 mm/h. A chest radiograph showed no acute cardiopulmonary process.

What is causing this woman’s skin eruption and fever?

Choose one to reveal diagnosis and discussion

Sweet syndrome
Herpes simplex virus infection
Extraintestinal manifestation of Crohn disease
Varicella

Answer: Sweet syndrome

See the full case at Consultant360

After consultation with an infectious disease specialist, the woman was started on acyclovir empirically for presumed disseminated herpes simplex virus (HSV) infection and on levofloxacin for sinusitis. One of the pustules was unroofed, and fluid was sent for bacterial and viral cultures as well as polymerase chain-reaction (PCR) testing for HSV. The results of these studies returned negative, at which time acyclovir was discontinued. Test results for enterovirus, varicella, parvovirus, and HSV 1 and 2 were all negative.

Histology results of a punch skin biopsy from a lesion on the left anterior shin showed diffuse dermal infiltrate of neutrophils with leukocytoclasis, red cell extravasation, and marked subepidermal edema (Figures 2 and 3).

Figure 2. Histology results of a punch skin biopsy of a lesion on the left anterior shin showing subepidermal edema and a neutrophilic dermatosis involving the superficial and mid-dermis, consistent with Sweet syndrome. Hematoxylin and eosin staining, ×10 magnification.

Figure 3. Histology results of a punch skin biopsy from a lesion on the left anterior shin showing neutrophils and leukocytoclastic debris, consistent with Sweet syndrome. Hematoxylin and eosin staining, ×40 magnification.

Based on the symptoms and test results, the woman received a diagnosis of Sweet syndrome. She was started on 60 mg of prednisone daily and was discharged on a prednisone taper, with rheumatology clinic follow-up scheduled. She eventually had complete resolution of her symptoms.

Discussion

Sweet syndrome, also called acute febrile neutrophilic dermatosis, is an inflammatory disorder characterized by a rash, fever, and systemic symptoms. Sweet syndrome is categorized as drug-induced, malignancy-associated, and idiopathic.

The differential diagnosis for a pustular rash with fever includes cutaneous infection, Behçet disease, extraintestinal manifestations of Crohn disease (eg, erythema nodosum, early pyoderma gangrenosum), and cutaneous drug reaction.

The diagnosis of Sweet syndrome is based on the sudden onset of painful, erythematous plaques or nodules, and biopsy results that show a dense, neutrophilic infiltrate and subdermal edema. Minor diagnostic criteria include fever (temperature > 38°C), associated malignancy, inflammatory bowel disease, pregnancy, recent infection, rapid response to systemic corticosteroids, or 3 of the following laboratory abnormalities: elevated erythrocyte sedimentation rate or C-reactive protein level; leukocytosis (> 70% neutrophils). The diagnosis is met with both major and 2 of the minor criteria.

The trigger of Sweet syndrome in out patient likely was acute sinusitis. The treatment is systemic glucocorticoids (prednisone with a prolonged taper). Relapse occurs in approximately 30% of patients.

Katherine G. Garlo, MD, is a resident in the Department of Internal Medicine at Maine Medical Center/Tufts University School of Medicine in Portland, Maine.

Nicholas Perros, MD, is a resident in the Department of Internal Medicine at Maine Medical Center/Tufts University School of Medicine in Portland, Maine.

Robert Smith, MD, is a physician in the Department of Infectious Diseases at Maine Medical Center/Tufts University School of Medicine in Portland, Maine.

Katy Linskey, MD, is a physician in the Department of Pathology and Laboratory Medicine at Maine Medical Center/Tufts University School of Medicine in Portland, Maine.

References:

• Anzalone CL, Cohen P. Acute febrile neutrophilic dermatosis (Sweet’s syndrome). Curr Opin Hematol. 2013;20(1):26-35.

• Cohen P. Neutrophilic dermatoses: a review of current treatment options. Am J Clin Dermatol. 2009;10(5):301-312.

• Jose FA, Heyman MB. Extraintestinal manifestations of inflammatory bowel disease. J Pediatr Gastroenterol Nutr. 2008;46(2):124-133.

• Rappaport A, Shaked M, Landau M, Dolev E. Sweet’s syndrome in association with Crohn’s disease: report of a case and review of the literature. Dis Colon Rectum. 2001;44(10):1526-1529.

• Schadt CR, Callen JP. Management of neutrophilic dermatoses. Dermatol Ther. 2012;25(2):158-172.

More Cases
45-Year-Old Female with Asymptomatic Solitary Papule

Can you identify this skin ailment? Take this week's Derm Case Challenge to validate your diagnosis.

45-Year-Old Female with Asymptomatic Solitary Papule
38-Year-Old Male with Left Thumb Lesion

Derm Case Challenge

38-Year-Old Male with Left Thumb Lesion
75-Year-Old Female with Rash on Lower Extremities

Test your dermatology knowledge with this week's Derm Case Challenge.

75-Year-Old Female with Rash on Lower Extremities
40-Year-Old Male with Growing Lesion on Back

Can you identify this skin ailment? Take this week's Derm Case Challenge to validate your diagnosis.

40-Year-Old Male with Growing Lesion on Back
45-Year-Old Male with Photodistributed Rash

Derm Case Challenge

45-Year-Old Male with Photodistributed Rash
46-Year-Old Female with Knee and Lower Back Aches

Derm Case Challenge

46-Year-Old Female with Knee and Lower Back Aches
75-Year-Old Female with Rash on Lower Extremities

Test your dermatology knowledge with this week's Derm Case Challenge.

75-Year-Old Female with Rash on Lower Extremities
40-Year-Old Female with Inframammary Rash

Derm Case Challenge

40-Year-Old Female with Inframammary Rash
42-Year-Old Male with Abnormal-Looking Thumbnails

Test your dermatology knowledge with this week's Derm Case Challenge.

42-Year-Old Male with Abnormal-Looking Thumbnails
27-Year-Old Male with Bumps on Scalp and Neck

Derm Case Challenge

27-Year-Old Male with Bumps on Scalp and Neck