Correspondence Address:
Hong Sang
Department Dermatology, Jinling Hosp, Nanjing Univ, Sch Med, Nanjing - 210002
Peoples R China

Full Text



Dear Editor,

A 44-year-old Chinese woman was presented with a 3-month history of a plaque on the left elbow, and then new plaques had gradually progressed to her trunk, legs, neck, and face in a symmetrical pattern within one month, with fever and severe pain. She was diagnosed with Type 2 diabetes and Myelodysplastic syndrome (MDS) one year ago.

On examination, she was febrile (39.4°C) and tachycardic but normotensive. Physical examination revealed sharp-edged red-brown papules and edematous erythema scattered over the head, face, neck, and two arms of different sizes and distributed asymmetrically. Her left arm was swollen and stiff. A canary yellow hyperplastic plaque of the size of about 5 cm × 6 cm lay on the left elbow joint, with old hemorrhage on the surface and false blisters scattered around [Figure 1]. Laboratory analysis revealed normal white blood cell count (5.2 × 109/L, 71.50% neutrophils, 20.3% lymphocytes, 6.8% monocytes), low hemoglobin (65 g/L) and platelet count (91 × 109/L), high erythrocyte sedimentation rate (140 mm/h), high C-reactive protein level (134.3 mg/L), and normal renal and hepatic function. Punch biopsy specimens were, respectively, obtained from the left elbow. Histopathological examination is characterized by epidermal papillomatous hyperplasia, dermal edema, and a dense dermal neutrophilic infiltrate [Figure 2]. These findings support the diagnosis of hyperplastic sweet syndrome (SS).{Figure 1}{Figure 2}

The key to supplementary diagnosis in this patient is the history of anemia. SS is always associated with elevated leukocyte and neutrophils, but no abnormality is seen in admission routine blood tests, except hemoglobin and platelet, which significantly decreased in this case, so we speculated that the change of leukocyte and neutrophils were covered up.

The patient has been treated with Methylprednisolone 40 mg intravenous for a week, then reduced dose for oral maintenance therapy. After medication, the body temperature rapidly recovered to normal, and the rashes completely disappeared except the plaque on the left elbow is shrank with the surface cracked. Because of the patients' poor compliance, the disease relapsed after self-medication withdrawal. She also refused MDS treatment, and the patient died one year later.

SS is characterized by fever and erythematous papules, plaques, or nodules with a predominantly neutrophilic dermal infiltrate on histopathologic examination. Although the pathogenesis of SS is not yet very clear, may hypersensitive to the underlying infection, inflammation, or malignancy. Malignancy was identified in 67% of SS patients, and 82% of them showed hematologic malignancies and 18% had solid malignancies. The most common malignancy was acute myelocytic leukemia (43% of patients), followed by MDS (in 18%).[1] SS with laboratory evidence of anemia[2] and chronic relapsing-remitting suggest the possibility of MDS.[3] Also, neutrophilic dermatosis such as SS is approved to be associated with a worse prognosis of the underlying hematologic tumor.

SS with atypical histological and clinical features prone to misdiagnosis. Histiocytoid SS is a rare histopathologic variant of SS. According to the recent research, histiocytoid SS infiltrate of immature cells of myeloid lineage in dermal, but not of histiocytes, and it is without a hint confirmed to be a hematologic malignancy.[4] The histopathological manifestation of necrotizing SS reveals polymorphonuclear including immature neutrophils infiltrates within the dermis or even deeper, also evident on fat necrosis and myonecrosis.[5] Bullous SS shows edematous plaques with bullae on extremities, the histopathology appears as severe papillary dermal edema.[6] Hyperplastic SS is rarely reported. A case of hyperplastic SS is suspected to be associated with pregnancy and low-molecular-weight heparin sodium.[7] Herein, we describe a case of hyperplastic SS, which is presented with hyperplastic plaques, with histopathology manifest as epidermal papillomatous hyperplasia. We speculate that the formation of proliferation is due to a delay in early treatment.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References