A Teen with Persistent Painful Papules and Plaques

March 1, 2015
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Clinical Vignette

Fearful frustrated parents deliver their ill appearing 17-year-old daughter to your office for evaluation of persistent painful papules and plaques associated with high fever, sore throat, and arthralgias (fig1, 2, 3). The eruption first appeared on the distal extremities 3 days ago and has now disseminated to the proximal extremities and trunk. She was sent home from the local emergency room yesterday with instructions to take plenty of fluids and ibuprofen.

Diagnosis and Clinical Presentation

Diagnosis – Sweet’s Syndrome Sweet’s syndrome also known as acute febrile neutrophilic dermatosis is reaction pattern in the skin characterized by a neutrophilic infiltrate which may present in several forms (1-5). 1. Classical Sweet’s Syndrome (CSS) is often preceded by a streptococcal or viral upper respiratory tract infection and occasionally associated with inflammatory bowel disease or pregnancy. 2. Malignancy Associated Sweet’s Syndrome (MASS) occurs in the context of patient with an occult solid tumor or a hematologic dyscrasia most commonly acute myeloid leukemia. 3. Drug Induced Sweet’s Syndrome is most commonly triggered by granulocyte colony stimulating factor (GCSF) and the antibiotic sulfamethoxazole-trimetoprim. Children with Sweet’s syndrome appear ill with acute onset of high fever, painful papules and nodules, arthralgias, and no apparent source of infection (1,3,5). The skin eruption, which most commonly develops on the face and extremities and may spread to the trunk, is characterized by edematous violaceous round to annular papules and plaques. Vesicles and pustules may appear at the periphery of the enlarging plaques which usually do not become frankly bullous or ulcerate. Healing occurs without scarring. Extracutaneous manifestations of Sweet’s syndrome may affect virtually every body system. Peripheral leukocytosis with neutrophilia, an elevated erythrocyte sedimentation rate and C-reactive protein are frequently seen. Two major and two minor criteria are needed for the diagnosis of Sweet’s syndrome: Major criteria: 1. abrupt onset of typical cutaneous lesions; 2. histopathology consistent with Sweet’s syndrome. Minor criteria: 1. syndrome is preceded by one of the associated infections, vaccinations, or accompanied by malignancy, inflammatory disorder or pregnancy; 2. presence of fever and constitutional signs and symptoms; 3. leukocytosis; 4. excellent response to systemic corticosteroids and no response to antibiotics.

Laboratory Findings

A skin biopsy from an violaceous nodule on the left arm of our patient revealed a dense dermal infiltrate containing numerous neutrophils with no signs of infection or vasculitis (4) (fig 4: H&E X20, fig. 5: H&E X40, fig 6: H&E X100). These changes were most consistent with a neutrophilic dermatosis. Neutrophilic Dermatoses as a group are characterized by the presence of mature neutrophilic leukocytes in the skin and acute onset of fever and non-specific constitutional symptoms without an obvious source of infection. Neutrophilic dermatoses include Sweet’s Syndrome (SS), DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) and Pyoderma Gangrenosum. Although these disorders can overlap clinically and histologically, specific diagnosis can usually be made with careful clinical-histologic correlation, history of exposure to potential triggering factors, and course of the eruption.

Differential Diagnosis

Erythema nodosum is another reactive erythema that may also be triggered by infections, drugs, inflammatory diseases, lymphomas, and pregnancy. Deep seated painful, erythematous to violaceous nodules develop typically on the anterior surface of both legs, and usually resolve in two to eight weeks without scarring. The eruption of Sweet’s syndrome can be clinically and histologically distinguished from erythema nodosum which is an inflammatory disorder involving fat. Disseminated gonococcemia begins with papules and vesicles which may occasionally evolve to pustules with a necrotic base. It may also be associated with fever and polyarthritis. A Gram stain obtained after unroofing a pustule reveals the organism. Rheumatoid neutrophilic dermatitis (RND) occurs most frequently in women with seropositive rheumatoid arthritis. The typical eruption consists of erythematous, urticarial-like papules, plaques or nodules that are less than 2 cm in diameter. Unlike Sweet’s syndrome, lesions in RND usually clear with aggressive treatment of the rheumatoid arthritis. Moreover, these patients do not present with fever of other constitutional symptoms.

Treatment

If left untreated, Sweet’s syndrome may persist for weeks to months. Systemic corticosteroids are the therapeutic mainstay usually beginning at 1mg/kg/day and tapering over 4 to 6 weeks (1-3, 5). Other reported treatment options include potassium iodide, colchicine, indomethacin, clofazimine, cyclosporine and dapsone. In some cases of classical Sweet’s syndrome, the symptoms resolve without any therapeutic intervention. Recurrence may occur in classical Sweet’s syndrome or malignancy associated disease and should prompt further evaluation for underlying systemic disease.

Our Patient

The punch biopsy established the diagnosis of acute neutrophilic dermatosis. Extensive medical evaluation failed to reveal an underlying infectious, immunologic or malignant process. She responded within 2 days to oral steroids administered in combination with oral potassium iodide and indomethicin. Her fever abated immediately and within a week all skin lesions had resolved leaving some patchy residual purpura.

Conclusion

Acute febrile neutrophilic dermatosis should be considered in children and adults with acute onset of persistent high fever, arthralgias, general malaise, and generally ill appearance, elevated white blood cell count, negative blood cultures, and the presence of a skin eruption comprised of red painful edematous papules, plaques, bullae, and pustules that typically start on the distal extremities and then disseminate.

References

  1. Herron MD, Coffin CM, Vanderhooft SL: Sweet Syndrome in Two Children. Pediatr Dermatol Volume 22, Issue 6, 2005; 525-529.
  2. Waltz KM, Long D, Marks JG Jr, Billingsley EM: Sweet's syndrome and erythema nodosum: the simultaneous occurrence of two reactive dermatoses. Arch Dermatol 1999; 135: 62 - 66.
  3. Herron MD, Coffin CM, Vanderhooft SL: Sweet Syndrome in Two Children. Pediatric Dermatology Volume 22, Issue 6, 2005; 525-529.
  4. Nischal KC, Khopkar U: An approach to the diagnosis of neutrophilic dermatoses: A histopathological perspective. Indian J Dermatol Venereol Leprol 2007; 73:222-30.
  5. Cohen PR: Sweet’s syndrome - a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J of Rare Diseases 2007; 2:34

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