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 possible rare localised variant of Sweet’s syndrome that was first reported in 1995 (Cravo et al, 2008; Kaur et al, 2015). Localised means that it’s 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, and a possible distributional variant affecting the arms has also been reported (Verma, 2012).
NDH is sometimes split into 2 sub-types, 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 (69%) (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 can sometimes 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, and a raised erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP). The latter are two blood tests that will show increased levels of 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, and when it’s confined to the hands can be indistinguishable from another form of neutrophilic dermatosis called atypical pyoderma gangrenosum (Kaur et al, 2015; Walling et al, 2006; Yang et al, 2017). Less commonly, it’s 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 frequently associated with cancer and blood cancers (21% of cases), particularly myelodysplastic syndromes (MDS) (Kaur et al, 2015; Wolf and Tuzun, 2017; Yang et al, 2017). In August 2017, the first case of NDP developing secondary to MDS was reported in a 67-year-old woman who was undergoing cyclic chemotherapy with azacitidine (Yang et al, 2017).
Aside from MDS, NDH has also been reported to have developed in association with:
- Upper respiratory tract infection (Gammoudi et al, 2017).
- Inflammatory bowel disease (19% of cases), mainly ulcerative colitis (Wolf and Tuzun, 2017).
- Rheumatoid arthritis (Baliu-Pique et al, 2017; Kaur et al, 2015).
- Sarcoidosis (Kaur et al, 2015).
- Lymphoma, or an internal cancer (Kaur et al, 2015; Oakley, 2015).
- Leukaemia (Kaur et al, 2015).
- Primary immune thrombocytopenia (Lozano-Masdemont et al, 2015).
- Influenza vaccination (Kaur et al, 2015; Oakley, 2015).
- Fertilizer exposure (Kaur et al, 2015).
- Skin puncture while handling fish (pathergy) (Gammoudi et al, 2017).
- Hepatitis C (Kaur et al, 2015).
- Medications – lenalidomide, thalidomide, granulocyte colony stimulating factor (G-CSF), non-steroidal anti-inflammatory drugs, cotrimoxazole and others (Kaur et al, 2015; Oakley, 2015).
In some patients with NDH, there is no underlying cause.
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, i.e. inflammation of the small blood vessels (Yang et al, 2017). By contrast, vasculitis is commonly shown in NDDH. As a result of this difference, there’s debate over whether or not NDDH is a variant of Sweet’s syndrome or another form of neutrophilic dermatosis; the vasculitis is a true vasculitis or caused by neutrophils releasing noxious substances and severe inflammation (Gammoudi et al, 2017; 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.
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 don’t work in the treatment of Sweet’s syndrome (tetracyclines can be an exception), and wound debridement must be avoided as it can lead to the development of new lesions. Read more here.
Oakley, A. (2015) Pyoderma gangrenosum. DermNet NZ (online). Accessed 7/04/18.
Baliu-Piqué, C., Nogués-Antich, E., Cucurell-Palomas, M., and Grados, D. (2017) Neutrophilic Dermatosis of the Hands (Sweet Syndrome of the Hands) Associated With Rheumatoid Arthritis. Reumatologia Clinica, Dec (online).
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) (eScholarship).
Gammoudi, R., Boussofara, L., Ghariani, N., Sriha, B., Belajouza, C., Denguezli, M. and Nouira, R. (2017) Neutrophilic dermatosis of the hands: an acral variant of Sweet’s syndrome or a distinct entity? European Medical Journal, Nov; 5(1):62-63 (online).
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.
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).
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 (PMC).
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