Anti-neutrophil cytoplasmic antibody-positive neutrophilicdermatosis of the dorsal hands

M. Malik, W. Perkins and I. Leach

Department of Dermatology, Queens Medical Centre, Nottingham University Hospital, Nottingham, UK

doi:10.1111/j.1365-2230.2012.04359.x

Summary Neutrophilic dermatosis of the hands is a localized variant of Sweet syndrome (SS). It

was first reported in 1995, and is an uncommon condition, with < 100 cases reported

to date. The female preponderance, morphological and histological features, and

response to treatment are similar to SS, but it differs in its distribution on the body.

There may also be a lack of systemic features and inconsistent laboratory findings.

Significantly, about half of all cases are associated with haematological problems, i.e.

myelodysplasia and leukaemia. Other cases may be associated with ulcerative colitis or

solid tumours. We describe a case of a 71-year-old man with neutrophilic dermatoses

of the hands, who also had involvement of the lips. There was an associated rise in his

anti-neutrophil cytoplasmic antibody level, which corresponded with the activity of the

disease.

Sweet syndrome (SS) is the prototype of neutrophilic

dermatoses. Its hallmarks include fever, peripheral

leucocytoses, painful erythematous plaques or nodules,

and a neutrophilic infiltrate in the dermis. The rash is

distributed mainly on the face and upper part of the

body. An uncommon variant affecting the dorsa of the

hands has been described in literature as ‘neutrophilic

dermatosis of the dorsal hands’. We report a case of

neutrophilic dermatosis with a positive anti-neutrophil

cytoplasmic antibody (ANCA) titre.

A 71-year-old man presented with a 4-day history of

a painful rash on the hands associated with mild

systemic upset. His medical history included hypothy-

roidism and hypertension.

On physical examination, symmetrically distributed

haemorrhagic and ulcerated plaques with distinct

pustules were seen on the dorsa of both hands

(Fig. 1a). The lower lip was swollen and covered with

haemorrhagic crusts (Fig. 1b).

On histological examination of a diagnostic biopsy

taken from a finger, neutrophils were seen in the upper

dermis, along with some features of vasculitis.

Laboratory testing showed that the patient had a

normal full blood count with absence of leucocytoses.

Renal and liver function was normal, but the level of

inflammatory markers (erythrocyte sedimentation rate

and C-reactive protein) was raised. The level of peri-

nuclear (p)-ANCA was also raised, and there was a

polyclonal increase in immunoglobulins. Autoantibody

screen and HIV testing were negative.

A diagnoses of neutrophilic dermatoses of the dorsal

hands was made, and the patient was started on oral

prednisolone, which caused a dramatic improvement

with resolution of the rash over the course of a few

weeks. Interestingly, repeat testing of ANCA levels

showed a steady decrease, corresponding to the clinical

activity of the dermatosis.

The condition ‘neutrophilic dermatosis of the dorsal

hands’ is a relatively new diagnosis, and the condition

has been considered to be a variant of several condi-

tions, including neutrophilic dermatosis (i.e. SS),1,2

erythema elevatum diutinum,3 atypical pyoderma gan-

grenosum (PG)4 or vasculitis (i.e. pustular vasculitis).5

The morphological and histological characteristics and

response to treatment are similar to those of SS. The

Correspondence: Dr Moona Malik, Department of Dermatology, Queens

Medical Centre, Nottingham University Hospital, Nottingham, NG7 2UH, UK

E-mail: moona.malik@nuh.nhs.uk

Conflict of interest: none declared.

Accepted for publication 22 December 2011

Clinical dermatology • Concise report CEDClinical and Experimental Dermatology

� The Author(s)

CED � 2012 British Association of Dermatologists • Clinical and Experimental Dermatology 1

main differences lie in the distribution, lack of systemic

symptoms and the inconsistency of laboratory findings.

A number of important associations have been found,

including myelodysplasia, leukaemia, inflammatory

bowel disease, seropositive arthritis, lymphoma and

the anti-thyroid drug lanalidomide.

Interestingly, our patient had a raised p-ANCA level,

and repeat testing showed that it had a direct correla-

tion with the disease activity. Laboratory data that has

to date been published in case series or reports of

neutrophilic dermatosis of the hands have not included

evaluation of ANCA levels, but it has been described in

other neutrophilic dermatoses. Ayoub et al. evaluated

ANCA in neutrophilic dermatoses, and showed a clear

preponderance in patients with erythema elevatum

diutinum compared with SS or PG.5 The role of ANCA

in activating neutrophils has been reported in the

literature, and highlights its role in diseases other the

typical vasculitides.

In conclusion, we report a case of ANCA-positive

neutrophilic dermatosis of the dorsal hands, the first

such case reported, to our knowledge. There were no

systemic associations and a dramatic response to

steroids was obtained. We recommend testing for ANCA

in all patients with similar dermatoses, as this may have

important prognostic significance.

References

1 Walling HW, Snipes CJ, Gerami P, Piette WW. The

relationship between neutrophilic dermatoses of the dorsal

hands and Sweet’s syndrome: report of 9 cases and com-

parison to atypical pyoderma gangrenosum. Arch Dermatol

2006; 142: 57–63.

2 Ayoub N, Tomb R. Neutrophilic dermatoses of the dorsal

hands: a variant of erythema elevatum diutinum. Arch

Dermatol 2003; 139: 102.

3 Callen JP. Pyoderma gangrenosum. Lancet 1998; 351:

581–5.

4 Strutton G, Weedon D, Robertson I. Pustular vasculitis of

the hands. J Am Acad Dermatol 1995; 32: 192–8.

5 Ayoub N, Charuel JL, Diemert MC et al. Antineutrophil

cytoplasmic antibodies of IgA class in neutrophilic derma-

toses with emphasis on erythema elevatum diutinum. Arch

Dermatol 2004; 140: 931–6.

(a)

(b)

Figure 1 (a) Multiple haemorrhagic and ulcerated plaques were

present on the dorsa of the hand, with distinct pustules on the

middle finger and the thumb; (b) the lower lip was swollen and

covered with haemorrhagic crusts.

� The Author(s)

2 CED � 2012 British Association of Dermatologists • Clinical and Experimental Dermatology

ANCA-positive neutrophilic dermatosis of the dorsal hands • M. Malik et al.