Sweet ́ s syndrome and pancreatic neoplasm : an atypical association

Corresponding Author: Nelson Turra. E-mail: nelsonturra85@gmail.com, Telephone: +59891419695, Address: Avenida General Rivera 2620, Montevideo, Uruguay. Postal Code: 11300. 1Universidad de la República, Hospital de Clínicas Dr. Manuel Quintela, Cátedra de Dermatología, Montevideo – Uruguay 2Clínica Alemana, Valdivia – Chile 3Hospital Dr. Eduardo Schütz, Puerto Montt – Chile Sweet’s syndrome (SS) or acute febrile neutrophilic dermatosis, is a rare inflammatory condition characterized by fever, neutrophilia, and abrupt onset of painful erythematous nodules or plaques, 1,2,3 a dermal infiltrate of mature neutrophils on histology and a fast response to the steroidal treatment.4,5 It has an incidence of 3 cases per million persons-year6, and predominates in women (female-male ratio, 4:1).3,7 Malignancy is associated with SS in approximately 20% of cases,8 being the hematological tumors the most frequently associated (85%),7 and on the other hand, nearly two-third of the patients with an association with solid tumors have carcinoma of genitourinary organs.9 Intraductal papillary mucinous neoplasms (IPMN) is a cystic neoplasm from the epithelial cells lining the pancreatic ductal system, with an incidence of 1/281,000 patients per year,10 and it is the first case of SS associated to IPMN in medical literature. Case


S
weet's syndrome (SS) or acute febrile neutrophilic dermatosis, is a rare inflammatory condition characterized by fever, neutrophilia, and abrupt onset of painful erythematous nodules or plaques, 1,2,3 a dermal infiltrate of mature neutrophils on histology and a fast response to the steroidal treatment. 4,5It has an incidence of 3 cases per million persons-year 6 , and predominates in women (female-male ratio, 4:1). 3,7alignancy is associated with SS in approximately 20% of cases, 8 being the hematological tumors the most frequently associated (85%), 7 and on the other hand, nearly two-third of the patients with an association with solid tumors have carcinoma of genitourinary organs. 9Intraductal papillary mucinous neoplasms (IPMN) is a cystic neoplasm from the epithelial cells lining the pancreatic ductal system, with an incidence of 1/281,000 patients per year, 10 and it is the first case of SS associated to IPMN in medical literature.

Case
An 80-year-old man, with compensated diabetes and treated with metformin, was referred to our hospital with fever, polyarthralgia and multiple nodular erythematous and painful lesions with pseudovesicles on his face, neck and extremities of a four-days evolution (Figure 1).There were no digestive symptoms, weight loss, muscle weakness, or anorexia.A full blood examination showed leukocytosis (10.300/mm3) with neutrophilia (84%), erythrocyte sedimentation rate (30 mm/hour) and C-reactive protein (32,2 mg/dL).Histologically, a skin biopsy from a lesion on the neck demonstrated pustules and edema of the upper dermis, accompanied by perivascular neutrophilic infiltration (Figure 2).These findings were consistent with SS.The patient was admitted to our hospital and received prednisone 60 mg/day by oral administration, with a fast improvement of fever and skin lesions.Searching for indicators of malignancy, an abdominal magnetic resonance and a cholangioresonance showed two IPMN, located in the body and head of the pancreas (Figure 3).The location of the tumors determined that surgical treatment was not an option.A low-dose corticosteroid therapy was therefore considered to reduce the risk of recurrence.After 6 months, the patient didn´t present new exacerbations.

DisCussion
SS is an inflammatory skin condition characterized by tender, erythematous and edematous skin lesions and systemic corticosteroids are the cornerstone of management for this illness.According to the patient's age, tests to rule out malignancies were performed, and a pancreatic neoplasm was diagnosed.Additionally, we should consider that the SS could reappear because the pancreatic lesions were not removed, and a strict follow-up becomes indispensable, because a recurrence of SS has been reported in up to two-thirds of the cases, when the neoplasm has not been removed.4  There have been reports of SS arising in association with other malignancies; however, it is the first reported association of SS with a pancreatic neoplasm in medical literature.

ConClusion
It is imperative that the dermatologist doesn´t miss the sentinel nature of this clinical syndrome, because the SS could alert the physician to the diagnosis of cancer, especially in elderly people.

Figure 1
Figure 1 Sweet´s syndrome, clinical manifestation.Multiple nodular erythematous lesions on the face and the hands.