at SciVerse ScienceDirect

DERMATOLOGICA SINICA 31 (2013) 165–167

Contents lists available

Dermatologica Sinica

journal homepage: http: / /www.derm-sinica.com

CORRESPONDENCE

Neutrophilic dermatosis of the hands

A 78-year-old Taiwanesewoman presented with fever and pain-ful skin lesions over both palms for 2 weeks. Her medical historywas unremarkable. On physical examination, infiltrating and juicyvesicular eruptions on violaceous bases were noted over the palmsas well as on the ventral and lateral aspects of the fingers (Figure 1).Very few lesions extended to the dorsal aspect of the fingers. Inaddition, no mucosal lesions were found. Laboratory studies onadmission revealed leukocytosis and neutrophilia with the whiteblood cell count at 12.54 � 103/mL with 91.6% neutrophils. The C-reactive protein level was also elevated (3.18 mg/dL). The patientwas treated under a provisional diagnosis of a cutaneous infection;however, the symptoms persisted despite a 5-day administration ofsystemic antibiotics. Further laboratory investigationsdincludingurinalysis, blood culture, and Tzanck smeardall demonstrated neg-ative results. A skin biopsy specimenwas obtained from a lesion onthe patient’s left palm. Histopathologic evaluation revealed mildepidermal acanthosis with subepidermal edemawith dense dermalneutrophilic infiltrate (Figure 2A). In addition, abundant leukocyto-clastic debris, extravasated red blood cells, and fibrinoid necrosis ofsmall vessels were evident (Figure 2B). Tissue culture and immuno-fluorescence revealed negative results. No associated underlyingmalignancy, infection, or inflammatory bowel disease was notedat the time of presentation. Based on the clinicopathologic correla-tions, the diagnosis of neutrophilic dermatosis of the hands wasmade, and the patient was treated with betamethasone 4 mg orallytwice per day. The patient’s fever subsided in 3 days and cutaneouslesions resolved without scarring after 2 weeks of treatment.

Figure 1 Infiltrating and juicy vesicular eruptio

1027-8117/$ – see front matter Copyright � 2013, Taiwanese Dermatological Associatiohttp://dx.doi.org/10.1016/j.dsi.2012.12.002

In 1995, Strutton et al1 first described six patients displayingpapules, hemorrhagic plaques, pustules, and bullous lesions overthe dorsal surface of the hands in whom histopathologic featuresrevealed dense dermal neutrophilic infiltrationwith leukocytoclas-tic vasculitis. The general clinical manifestations were also associ-ated with fever, leukocytosis with neutrophil predominance,sterile culture result, and nonrespone to antibiotic therapy butshowing rapid response to prednisone administration. This clinicalpresentation differed from, but was similar to, Sweet syndrome (SS)in terms of histologic features, particularly with respect to the pres-ence of leukocytoclastic vasculitis. Hence, Strutton et al named thisentity pustular vasculitis of the dorsal hands; however, these caseswere still considered as localized variants of SS. In 2000, Galariaet al2 reported three cases with similar clinical findings albeitwith no evidence of vasculitis on histopathology; therefore, this en-tity was renamed neutrophilic dermatosis of the dorsal handsinstead of pustular vasculitis of the dorsal hands. In most casesreported under this diagnosis, the dorsal aspects of the hands com-prised the most commonly affected sites; however, recently, a fewsimilar cases involving only the palms and lateral aspects of the fin-gers have been reported.3 Hence, the term neutrophilic dermatosisof the dorsal hands was replaced by the term neutrophilic derma-tosis of the hands (NDH).3

To date, although fewer than 100 cases of NDH have beenreported, it is possible that the incidence of this disease might havebeen underestimated. For NDH, the average age at diagnosis was60.5 years, with 58% of the patients being female. Clinically, the

ns on erythematous bases over the palms.

n. Published by Elsevier Taiwan LLC. All rights reserved.

Figure 2 (A) Subepidermal edema and dense dermal neutrophilic infiltration (hematoxylin and eosin, �100). (B) Abundant leukocytoclastic debris, extravasated red blood cells, andfibrinoid necrosis of small vessels (hematoxylin and eosin, �400).

Correspondence / Dermatologica Sinica 31 (2013) 165–167166

lesionsappearedasedematous,purpuricplaqueswithblistersand/orpustules on thedorsaland/orpalmaraspects of thehandsandthe lat-eral aspects of the fingers. Most cases of NDH are associated with fe-ver, peripheral leukocytosis with neutrophilia, and an elevatederythrocyte sedimentation rate or C-reactive protein level. Histo-pathologic features typically include subepidermal edema anddensedermal neutrophilic infiltrate with or without leukocytoclastic vas-culitis. Patients inwhomNDH is diagnosed shouldbe assessed for co-morbid medical conditions because previously reported cases havebeen associated with leukemia, myelodysplasia, other hematologicdiseases, inflammatory bowel disease, or preceding vaccination.4

The similar entities of SS, vesiculobullous pyoderma gangreno-sum (PG), and NDH present a difficult diagnostic dilemma (Table1). NDH is fairly similar to SS in terms of clinical presentation exceptthat the lesions are limited to the hands. Basically, the main histo-pathologic feature of NDH is similar to that of SS, which shows pap-illary dermal edema with dense neutrophilic infiltrate withoutvasculitis. However, vasculitis might be shown in some cases ofNDH; therefore, it has long been debated whether NDH comprisesa true variant of SS or a separate clinical entity. Some authors haveobserved that vasculitis is correlated with long-standing lesions inSS and NDH, where the damaged vessels could be considered as theso-called innocent bystander.5 In other words, vasculitis is consid-ered as a time-dependent change rather than a fundamental onefor the diagnosis of SS and NDH. Therefore, NDH continues to beregarded as a localized variant of SS.5 Vesiculobullous PG is anotherdisease entity resembling NDH. Clinically, vesiculobullous PG

Table 1 Common and distinct features among different neutrophilic dermatoses.

SS NDH

Clinical manifestation Asymmetrically distributed on the face,trunk, and limbs

Limitedorsa

Histopathologicfinding

Dermal edema with dense infiltration by neutrophils;vasculitis may be found in long-standing lesions

Dermby ne

Possible comorbidity Internal malignancy, infection, pregnancy, vaccination,inflammatory bowel disease

Interninflam

Prognosis Good response to systemic steroid administration.Overall outcome depends on underlying disease.

GoodOvera

NDH [ neutrophilic dermatosis of the hands; PG ¼ pyoderma gangrenosum; SS ¼ Swee

presents as hemorrhagic bullae predominantly located on the dor-sal hands. Histologically, the condition is characterized by denseneutrophilic infiltrate with a prominent vascular reaction, but nottrue vacuities. Thus, both its clinical and histologic features are con-siderably similar to those of NDH. Because of this overlap in clinicaland microscopic features, some authors consider that NDH, SS, andvesiculobullous PG possibly represent different aspects of the samedisease spectrum.5 NDHmight be a preferred term to represent thisacral neutrophilic dermatosis.

Many of the reported cases of NDHdincluding the currentcasedwere initially diagnosed as localized cutaneous infections,such as cellulitis, and treated with systemic antibiotics. However,antimicrobial therapy achieves no response in these patients.Hence, differentiating this clinical entity from cellulitis is importantfor accurate and timely therapy. In general, cellulitis clinically pres-ents as an ill-defined erythematous patch, in contrast to NDH,which presents as well-demarcated violaceous vesicles on eryth-ematous bases. Although cellulitis requires systemic antibiotic ther-apy, NDH shows good response to oral corticosteroids, potassiumiodide, minocycline, or dapsone.5 The skin lesions showgradual res-olution without scarring in variable periods (from 3 weeks to 3months) and the overall outcome is good. However, in patients withunderlying disease including inflammatory bowel disease or malig-nancy, the clinical outcome is correlated with the associated disease.

In conclusion, for patients with tender and/or hemorrhagic pal-mar vesiculobullous rashes accompanied by fever and leukocytosis,NDH should be included in the differential diagnosis, with skin

Vesiculobullous PG

d on the palmar surface and/orl aspects of hands

Usually limited on the extremities,especially dorsal aspects of hands

al edema with dense infiltrationutrophils; with/without vasculitis

Dermal edema with denseinfiltration by neutrophils;without vasculitis

al malignancy, infection, vaccination,matory bowel disease

Internal malignancy, rarelyinflammatory bowel disease

response to systemic steroid administration.ll outcome depends on underlying disease

Moderate response to systemicsteroid administration.Overall outcome depends onunderlying disease.

t syndrome.

Correspondence / Dermatologica Sinica 31 (2013) 165–167 167

biopsy being the recommended examination to reach an accuratediagnosis. Based on the resemblance of the clinical and histopath-ologic features of NDH, SS, and vesiculobullous PG, we considerneutrophilic dermatosis of the hands to be the most appropriatedesignation for such acral neutrophilic eruptions.

Ching-Fu HuangDepartment of Dermatology, Tri-Service General Hospital, National Defense Medical

Center, Taipei, Taiwan

Wei-Ming Wang*Department of Dermatology, Tri-Service General Hospital, National Defense Medical

Center, Taipei, Taiwan

Department of Biochemistry, National Defense Medical Center, Taipei, Taiwan

*Corresponding author. Department of Biochemistry, National Defense MedicalCenter, No. 325, Sec. 2, Chenggong Rd., Taipei 114, Taiwan. Tel.: þ886 87923311.

E-mail address: ades0431@ms38.hinet.net

References

1. Strutton G, Weedon D, Robertson I. Pustular vasculitis of the hands. J Am AcadDermatol 1995;32:192–8.

2. Galaria NA, Junkins-Hopskins JM, Kligman D, James WD. Neutrophilic dermato-sis of the dorsal hands. J Am Acad Dermatol 2000;43:870–4.

3. Weenig RH, Bruce AJ, McEvoy MT, Gibson LE, Davis MD. Neutrophilic dermatosisof the hands: four new cases and review of the literature. Int J Dermatol 2004;43:95–102.

4. Wolf R, Barzilai A, Davidovici B. Neutrophilic dermatosis of the hands after influ-enza vaccination. Int J Dermatol 2009;48:66–8.

5. Walling HW, Snipes CJ, Gerami P, Piette WW. The relationship between neutro-philic dermatosis of the dorsal hands and Sweet syndrome: report of 9 cases andcomparison to atypical pyoderma gangrenosum. Arch Dermatol 2006;142:57–63.

Received: May 30, 2012Revised: Dec 5, 2012

Accepted: Dec 17, 2012