The Relationship Between Neutrophilic Dermatosis of the Dorsal Hands and Sweet Syndrome

A Case of Neutrophilic Dermatosis of the Dorsal Hands with Concomitant Involvement of the Lips

“…The clinical presentation of NDDH differs from that of classic SS. In classic SS, fever, leukocytosis and an increased ESR are observed in 80∼90% of the cases; inflammatory bowel disease (16%) and hematologic disease (54%) may be present as the accompanying diseases 4,7 . In NDDH, the lesions are clinically restricted to the hands; fever, leukocytosis and an increased ESR are observed in 33% of the cases and hematologic malignancy is present as an accompanying disease in 21% of the cases 7 .…”

“…Cellulitis or erysipelas is excluded according to the negative tissue cultures and stains and a lack of response to antibiotics 3 . Atypical pyoderma gangrenosum, also known as vesiculobullous PG, presents with chronic hemorrhagic bullous lesions that ulcerate superficially and are usually seen in patients with leukemia or polycythemia vera 7 .…”

“…However, pyoderma gangrenosum may be histologically indistinguishable from NDDH, although the first usually shows true vasculitis 7 . Thus, atypical PG and pustular vasculitis of the dorsal hands are arguably within the spectrum of a single disease entity, which is most appropriately termed NDDH 9 .…”

“…Systemic corticosteroids are the most common first-line therapy 10 . Walling et al 7 showed that treatment with systemic corticosteroids was successful in 71% of the cases. In summary, we have presented a case of recurrent NDDH with no associated systemic signs and symptoms and the patient was successfully managed with low dose systemic corticosteroids.…”

Dermatoses neutrofílicas: parte I

“…6 The "neutrophilic dermatosis of the dorsal hands" has been considered a localized variant of Sweet's syndrome. 12,13 The subcutaneous form of the disease presents with lesions that are usually painful, erythematous subcutaneous nodules, located on the extremities, similar to erythema nodosum. 6 Fever, usually above 38 O C, is the most common sign and may precede the lesions or occur simultaneously.…”