Reumatología Clínica (English Edition) Reumatología Clínica (English Edition)
Reumatol Clin 2017;13:53-4 - Vol. 13 Num.1 DOI: 10.1016/j.reumae.2015.11.021
Images in Clinical Rheumatology
Neutrophilic Dermatosis of the Hands (Localized Sweet's Syndrome)
Dermatosis neutrofílica de las manos (Sweet de las manos)
Belén Lozano-Masdemonta,, , Irene Molina-Lópeza, Verónica Parra-Blancob, Ricardo Suárez-Fernándeza
a Servicio de Dermatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain
b Anatomía Patológica, Hospital General Universitario Gregorio Marañón, Madrid, Spain

We present the case of a 67-year-old Caucasian woman, who had been diagnosed 9 months before with primary immune thrombocytopenia. She had developed painful palmar lesions 1 week earlier, but had no fever or other accompanying symptoms. We observed erythematous violaceous plaques on the ulnar border of both hands, with a pustule of about 4cm on the right (Fig. 1a). A biopsy showed a dense dermic neutrophilic infiltrate, with marked edema, and no vasculitis (Fig. 1b). Cultures to detect bacteria, virus and fungi produced no evidence. Except for the patient's thrombocytopenia, her complete blood count was normal, and laboratory testing found a C-reactive protein of 1mg/L. A study of the extension ruled out an underlying neoplastic disease. She responded completely to oral prednisone at 0.5mg/kg/day for 2 weeks, with no recurrence after 1 year of follow-up. The neutrophilic dermatosis of dorsal hands, recently described,1 consists of the presence of erythematous violaceous plaques on acral sites, with blisters or pustules, histologically constituted by a dermic neutrophilic infiltrate, with or without association of leukocytoclastic vasculitis.1–8 Generally, there is no respiratory involvement, fever or laboratory abnormalities, which differentiates it from conventional Sweet's syndrome.1–5,9 It has been associated with hematologic neoplasms (frequently, myelodysplasia and leukemias), carcinomas, inflammatory bowel disease and arthritis, among other conditions.1–5 Available data show a rate of association with hematological diseases similar to that of classical Sweet's syndrome.4,9,10 The case we report is the first that associates it with primary immune thrombocytopenia, a disease that has been found exceptionally in conventional Sweet's syndrome.10 Our case, although the involvement is palmar, has a characteristic pustular morphology. Thus, we consider the term acral or hand neutrophilic dermatosis to be more correct.9 The differential diagnosis should include skin infections, arthritis, vasculitis and cutaneous lymphomas. It responds well to oral corticosteroids or dapsone, and its recurrence is occasional.1–5

Fig. 1.

(a) A large pustule in an erythematous violaceous plaque on the palm of the patient's hand. (b) Hematoxylin and eosin (10×). Dense dermic neutrophilic infiltrate with marked edema, without leukocytoclastic vasculitis.

Ethical DisclosuresProtection of human and animal subjects

The authors declare that no experiments were performed on humans or animals for this study.

Confidentiality of data

The authors declare that they have followed the protocols of their work center on the publication of patient data.

Right to privacy and informed consent

The authors declare that no patient data appear in this article.

Conflict of Interest

The authors declare they have no conflict of interest.

G. Strutton,D. Weedon,I. Robertson
Pustular vasculitis of the hands
J Am Acad Dermatol, 32 (1995), pp. 192-198
N.A. Galaria,J.M. Junkins-Hopkins,D. Kligman,W.D. James
Neutrophilic dermatosis of the dorsal hands: pustular vasculitis revisited
J Am Acad Dermatol, 43 (2000), pp. 870-874
D.J. DiCaudo,S.M. Connolly
Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands: a report of 7 cases and review of the literature
Arch Dermatol, 138 (2002), pp. 361-365
H.W. Walling,C.J. Snipes,P. Gerami,W.W. Piette
The relationship between neutrophilic dermatosis of the dorsal hands and Sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum
Arch Dermatol, 142 (2006), pp. 57-63
J. Del Pozo,F. Sacristán,W. Martínez,S. Paradela,B. Fernández-Jorge,E. Fonseca
Neutrophilic dermatosis of the hands: presentation of eight cases and review of the literature
R.H. Cook-Norris,J.S. Youse,L.E. Gibson
Neutrophilic dermatosis of the hands: an underrecognized hematological condition that may result in unnecessary surgery
Am J Hematol, 84 (2009), pp. 60-61
T. Leecy,A. Anderson,J. von Nida,N. Harvey,B. Wood
Neutrophilic dermatosis of the dorsal hands: an often under recognised and mistreated entity
Y.S. Wang,A. Tan
Neutrophilic dermatosis of the dorsal hands: an emerging entity
J Eur Acad Dermatol Venereol, 23 (2009), pp. 451-452
A. Nofal,M. Assaf,R. Elakad,M. Fawzy,E. Nofal
Neutrophilic dermatosis of the dorsal hands: a localized variant of Sweet's syndrome or a distinct entity?
Int J Dermatol, 54 (2015), pp. e66-e67
C.D. Hensley,S.W. Caughman
Neutrophilic dermatoses associated with hematologic disorders
Clin Dermatol, 18 (2000), pp. 355-367

Please cite this article as: Lozano-Masdemont B, Molina-López I, Parra-Blanco V, Suárez-Fernández R. Dermatosis neutrofílica de las manos (Sweet de las manos). Reumatol Clin. 2017;13:53–54.

Corresponding author. (Belén Lozano-Masdemont
Copyright © 2015. Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología
Reumatol Clin 2017;13:53-4 - Vol. 13 Num.1 DOI: 10.1016/j.reumae.2015.11.021