Acral manifestations of Sweet syndrome (neutrophilic dermatosis of the hands).

Neutrophilic dermatosis of the hand (NDH) is a rare localized variant of the syndrome, originally described two decades ago by Strutton et al. The lesions of NDH and Sweet syndrome are similar, as indicated in the first report of NDH. Both diagnoses are characterized by an acute onset of fever, leukocytosis, and tender, erythematous infiltrated plaques. There are also bullae and ulceration in NDH, in contrast to Sweet syndrome, in which bullae are quite uncommon, especially at the early stages. Similar to Sweet syndrome, the majority of NDH patients are women (69%). Patients with NDH present with fever, peripheral neutrophilia, leukocytosis, and/or an elevated erythrocyte sedimentation rate or C-reactive protein level, but at a significantly lower rate than those in Sweet syndrome (33%). Similar to Sweet syndrome, NDH has been associated with the following conditions: Malignancies (particularly hematological [21%], most common of which is acute myelogenous leukemia, but many other malignancies as well), inflammatory bowel disease (19%), medication and vaccination-related eruptions, bacterial and viral infections, rheumatologic diseases, and others. The clues to the diagnosis of NDH are the same as for Sweet syndrome. Awareness of this diagnosis is important not only to avoid unnecessary medical and surgical therapy and to expediently initiate the administration of steroids for this highly responsive dermatosis, but also to conduct an appropriate workup to exclude associated diseases, especially malignancies.

Wolf R ，Tüzün Y 《-》

[Neutrophilic dermatosis of the hands].

In 1964, based on eight cases, R. Sweet described a form of "acute febrile neutrophilic dermatosis" that was quickly renamed Sweet's syndrome. Over time, other entities (pyoderma gangrenosum, erythema elevatum diutinum, etc.) came to be included in the same nosological group, giving rise to the concept of neutrophilic dermatosis. In addition to types of passage between these different diseases, neutrophilic dermatoses may have a variety of atypical presentations such as that described as "neutrophilic dermatosis of the hands", of which we present a case herein. A 60-year-old woman with non-insulin-dependent diabetes and mild psoriasis was consulting for an eruption mainly on the hands. The lesions, of various shapes, comprised pustules and large bullous lesions on purple oedematous skin; these were painful and some were progressing to ulceration. They measured between 0.5 and 3 cm and were filled with an opaque liquid. These lesions were painful and some were progressing towards ulceration. The patient was also presenting inflammatory joint pain. The histopathology study revealed infiltration of the dermis, predominantly by neutrophils. A diagnosis of neutrophilic dermatosis was made without any clearly discernible distinction between Sweet's syndrome, palmoplantar pustulosis or pyoderma gangrenosum. Since the initial description of Sweet's syndrome in 1964, many unusual cases have been described in the literature (associated with neoplasias or systemic diseases, concomitant pustular or bullous lesions, etc.). In 1995, Strutton et al. described a new entity, pustular vasculitis of the hands, based on six cases, which is fairly similar to the present case. Understanding of this syndrome developed and Gallaria renamed it neutrophilic dermatosis of the dorsal hands. Walling and Duquia termed this form of dermatosis palmoplantar Sweet syndrome. This case highlights the difficulties in clearly distinguishing forms of neutrophilic dermatosis, adding to the notion of a continuum in neutrophilic disease.

Brajon D ，Cuny JF ，Barbaud A ，Schmutz JL ... -  《ANNALES DE DERMATOLOGIE ET DE VENEREOLOGIE》

[Neutrophilic dermatosis of the hands: acral sweet syndrome?].

Neutrophilic dermatosis may have various clinical presentations but share common histopathological manifestations with an aseptic infiltrate of polymorphonuclears neutrophils. Neutrophilic dermatosis of the hands is a recently described disorder of which we present a case. A 60-year-old woman, without significant past medical history, presented with an acute and painful eruption of both hands with fever. Clinical examination showed erythematous edematous lesions surmounted by pustules that covered the thenar eminences of both hands. The patient was also presenting inflammatory joint pain. Histological findings were predominantly neutrophilic infiltration in the dermis with leukocytoclastic debris. A diagnosis of neutrophilic dermatosis was made and all the lesions disappeared rapidly without relapse under oral prednisone (0.5mg/kg/j). The eruption observed in our patient was clinically and histologically suggestive of neutrophilic dermatosis of the hands, confirming the existence of a homogenous entity which is still debatable if it constitutes a separate entity or a localized variant of Sweet syndrome.

Boussofara L ，Gammoudi R ，Ghariani N ，Aounallah A ，Sriha B ，Denguezli M ，Belajouza C ，Nouira R ... -  《-》

Neutrophilic dermatosis of the dorsal hands: A review of 123 cases.

Neutrophilic dermatosis of the dorsal hands (NDDH) is an uncommon localized variant of Sweet syndrome first described in 1995. It is characterized by tender erythematous plaques, pustules, and bullae on the dorsa of the hands. A total of 123 cases of NDDH are included in this review. The mean patient age was 62.1 years, and there was a slight female preponderance. Overall, 78.0% of cases had bilateral involvement, and other sites were affected in almost a third of cases. Underlying disease was found in ∼40% of patients, with the most common associations being hematologic disorders (gammopathies, myelodysplasias, or malignancies), recent infection, solid organ tumors, and inflammatory bowel disease. Systemic or topical corticosteroids or both were employed in the treatment of 88.1% of cases, while dapsone, colchicine, and tetracyclines were the most common steroid-sparing agents used. Improvement was often rapid and complete resolution the norm. Although uncommon, NDDH is frequently misdiagnosed, and thus, its exact prevalence is probably underestimated. Misdiagnosis might have significant implications, including treatment delays or incorrect management. Moreover, recognition of NDDH is important, since a correct diagnosis should trigger a search for underlying diseases and proper treatment with corticosteroids, steroid-sparing agents, or both, which is almost invariably curative.

Micallef D ，Bonnici M ，Pisani D ，Boffa MJ ... -  《-》

The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum.

Walling HW ，Snipes CJ ，Gerami P ，Piette WW ... -  《ARCHIVES OF DERMATOLOGY》