Neutrophilic dermatosis of the hands: a localized, or localized and bullous variant

Updated on 23/08/2017.

Sometimes, Sweet’s syndrome can occur in an unusual form. This is called a disease variant.

What is neutrophilic dermatosis of the hands?

Neutrophilic dermatosis of the hands (NDH) is a localised variant of Sweet’s syndrome that was first reported in 1995 (Cravo et al, 2008; Kaur et al, 2015). Localised means that it is confined, or mainly confined, to one part of the body. NDH causes skin lesions to appear on the back of the hands (dorsal), thumb or fingers, but sometimes on the palms (palmar). Occasionally, additional lesions can appear on other parts of the body. A possible distributional variant affecting the arms has also been reported (Verma, 2012).

NDH is sometimes split into 2 sub-classifications, neutrophilic dermatosis of the dorsal hands (NDDH) and neutrophilic dermatosis of the palms (NDP) (Yang et al, 2017).

Does it affect both men and women?

Yes. NDH can affect both men and women, but mainly women. In fact, 69% of cases have been in women (Wolf and Tuzun, 2017).

What do the skin lesions caused by neutrophilic dermatosis of the hands look like?

In NDDH, the skin lesions are often described as bullous (bluish or grey abscess-like lumps and blisters), and sometimes they can ulcerate. Both bullous lesions and ulcers are more common in NDDH than in Sweet’s syndrome (Wolf and Tuzun, 2017). Swelling (oedema) may also be present. In NDP, the skin lesions are more likely to appear as reddened patches (Yang et al, 2017).

Will there be other signs or symptoms?

Patients with NDH can have a fever, raised white blood cell and neutrophil (type of white blood cell) count. They can also have a raised erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP). These are two blood tests that will show if there is inflammation in the body. However, these signs and symptoms are up to 33% less likely to occur in NDH than Sweet’s syndrome (Lozano-Masdemont et al, 2015; Wolf and Tuzun, 2017).

Find further information on symptoms here.

Can neutrophilic dermatosis of the hands be confused with other conditions?

Yes. NDH is often misdiagnosed as an infectious condition (Kaur et al, 2015; Yang et al, 2017). Also, when it is confined to the hands, it can be indistinguishable from another form of neutrophilic dermatosis called atypical pyoderma gangrenosum (Walling et al, 2006). Less commonly, it is confused with pustular drug reactions, rheumatoid neutrophilic dermatosis, bowel associated dermatosis arthritis syndrome, and erythema elevatum diutinum (Kaur et al, 2015).

What causes it?

NDH is most commonly seen in elderly patients, and can develop secondary to other conditions. NDDH but not NDP is often associated with cancer and blood disorders (21% of cases), especially myelodysplastic syndromes and leukaemia (Kaur et al, 2015; Wolf and Tuzun, 2017; Yang et al, 2017).

In late 2015, the first case of NDH was reported in a 67-year-old women who later went on to develop primary immune thrombocytopenia (Lozano-Masdemont et al, 2015). Prior to this, NDH had been reported to have developed in association with inflammatory bowel disease (19% of cases), rheumatoid arthritis, sarcoidosis, lymphoma, influenza vaccination*, fertilizer exposure, hepatitis C, and certain medications (Kaur et al, 2015; Wolf and Tuzun, 2017).

In August 2017, the first case of NDP was reported to have developed secondary to myelodysplastic syndromes in a 67-year-old woman who was undergoing cyclic chemotherapy with azacitidine (Yang et al, 2017).

In some patients with NDH, there is no underlying cause.

*Sweet’s syndrome triggered by vaccination is incredibly rare, and a definite connection between the Sweet’s syndrome and vaccination has not been established in all of the reported cases.

How is it diagnosed?

NDH is diagnosed in the same way as Sweet’s syndrome, but biopsy results are often different.

Find further information on diagnosis here.

How are the biopsy results different?

In both Sweet’s syndrome and NDH, biopsy of a lesion shows lots of neutrophils in the tissues. However, in Sweet’s syndrome and NDP, biopsy doesn’t tend to show vasculitis – inflammation of the small blood vessels (Yang et al, 2017). By contrast, vasculitis is commonly shown in NDDH, but there is debate over whether or not this is a true vasculitis or is caused by neutrophils releasing noxious substances and severe inflammation (Kaur et al, 2015).

How is it treated?

NDH is treated in the same way as Sweet’s syndrome, sometimes with the addition of very strong topical corticosteroids (steroid cream).

Find further information on treatment here.

Please note that NDH is often misdiagnosed as an infectious condition, and this can result in inappropriate treatment with antibiotics, and surgical wound debridement (surgical removal of infected or affected tissue) (Kaur et al, 2015). Antibiotics do not work in the treatment of Sweet’s syndrome (tetracyclines are sometimes an exception), and wound debridement must be avoided as it can lead to the development of new lesions. Read more here.

References.

Cravo, M., Cardoso, J., Tellechea, O., Cordeiro, M., Reis, J. and Figueiredo, A. (2008) Neutrophilic dermatosis of the dorsal hands associated with hypopharyngeal carcinoma. Dermatology Online Journal, Jul; 14 (7).

Kaur, S., Gupta, D., Garg, B. and Sood, N. (2015) Neutrophilic dermatosis of dorsal hands. Indian Dermatology Online Journal, Jan-Feb;6(1):42-5.

Lozano-Masdemont B., Molina-López I., Parra-Blanco V. and Suárez-Fernández R. (2015) Neutrophilic dermatosis of the hands (localized Sweet’s syndrome). Reumatologia Clinica, Dec 29th (online). Article available in Spanish and English. Use translate option.

Verma, S. (2012) Recurrent bilaterally symmetrical bullous sweet’s syndrome: a rare and confusing entity. Indian Journal of Dermatology; 57(6):483-5 (online).

Walling, H., Snipes, C., Gerami, P. and Piette, W. (2006) The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. Archives of Dermatology Jan;142(1):57-63 (PubMed).

Wolf. R. and Tuzun, Y. (2017) Acral manifestations of Sweet syndrome (neutrophilic dermatosis of the hands). Clinics in dermatology, Jan – Feb;35(1):81-84 (PubMed).

Yang, M., Kim, J., Kim, G., Song, M., Kim, H., Ko, H., Kim, M. and Kim, B (2017) Neutrophilic Dermatosis of the Palms in Association with Myelodysplastic Syndrome. Annals of Dermatology, Aug; 29(4): 495–497 (online).

Further information.

Oakley, A. (2015) Pyoderma gangrenosum. DermNet NZ (online). Originally published in 1998, and updated Sept. 2015.

© 2012-2017 Sweet’s Syndrome UK

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4 thoughts on “Neutrophilic dermatosis of the hands: a localized, or localized and bullous variant

  1. I had this variant of Sweets Syndrome this past January 1917 at seventy yrs. of age. I was six months getting better. I was hospitalized twice critical. Put on steroids. Terrible immune disease.

    Like

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