Neutrophilic dermatosis of the hands: a localized, bullous variant

Updated on 04/02/2017.

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).

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?

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 NDH than in Sweet’s syndrome (Wolf and Tuzun, 2017). Swelling (oedema) may also be present.

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). 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. It is often associated with cancer and blood disorders (21% of cases), especially myelodysplastic syndromes and leukaemia (Kaur et al, 2015; Wolf and Tuzun, 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 already 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 some patients, there is no underlying cause.

*Sweet’s syndrome and variants caused by vaccination is so rare that a definite connection has not been established in all 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, biopsy doesn’t tend to show vasculitis – inflammation of the small blood vessels. By contrast, vasculitis is commonly shown in NDH, 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).

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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