Final Diagnosis -- Neutrophilic dermatosis of the hands

FINAL DIAGNOSIS

NEUTROPHILIC DERMATOSIS OF THE HANDS

DISCUSSION

Neutrophilic dermatosis of the hands is a recently described clinical entity. It is considered a localized form of Sweet's syndrome (acute febrile neutrophilic dermatosis), starting on the hands and possibly spreading to other locations 1-6. It has been associated with a diverse number of systemic diseases and usually do not respond to antibiotics treatment. Instead, they have a good clinical response to topical and/or systemic steroids 1-6.

Historically, this entity was first recognized by Strutton et al.7 (1995) who described six patients who had presented with papules, hemorrhagic plaque pustules and bullous lesions located on the dorsum of the hands and showing a histopathological picture of neutrophilic dermatosis with the presence of leukocytoclastic vasculitis and showing good response to steroidal treatment. These authors considered these cases as a localized variant of Sweet's syndrome and named them "pustular vasculitis of the dorsal hands".

Soon after, Hall et al.8 reported the first case with lesions beginning on the hands and spreading to other locations. In 2000, Galaria et al.9 described three new cases with variable degrees of endothelial damage in the histopathological study. Recurrence and a good response to sulphone treatment were present. In addition, they proposed that the term "pustular vasculitis of the dorsal hands" change to "neutrophilic dermatosis of the dorsal hands", which is the term that has been used to describe this lesion since then. In 2004, Weenig et al.10 presented four new cases of neutrophilic dermatosis of the hands with involvement of the dorsal and palmar surface of the hands.

The histopathological findings vary among the cases. Dicaudo et al.11 presented seven new cases, five of them with lesions on the hands. One of these cases presented a dorsal hand lesion with leukocytoclastic vasculitis and a back lesion with neutrophilic dermatosis without presence of endothelial damage. Malone et al.12 reviewed 28 biopsy specimens from 21 patients with Sweet's syndrome and described a variable degree of vascular endothelial damage, between endothelial swelling and true vasculitis with fibrinoid necrosis. Cohen13 referred to the vasculitis present in skin lesions of Sweet's syndrome and its dorsal hand variant as an oxymoron or an epiphenomenon.

Other publication followed showing the association of these lesions with systemic diseases such as metastatic renal adenocarcinoma 14, ocular lesions 15, myeloproliferative neoplasm16, lip lesions17 and hepatitis C virus 18. Few published cases of neutrophilic dermatosis of the dorsal hands were related to thermal injury19, chemical fertilizer20, and Streptococcus infection21 resembling pustular vasculitis of the hands.

The differential diagnosis include other neuthrophilic dermatosis such as an infectious process, leukocytoclastic vasculitis, pyoderma grangrenosum, erythema elevatum diutinum and bowel bypass syndrome 22-24.

After the biopsy the patient's lesions were regarded as a more intense form of the disease and therefore the patient was started on systemic steroids. Five days later, the patient's symptoms improved and he was discharged home. Since neutrophilic dermatosis of the hands has been shown to be associated with systemic diseases the patient will be worked-up as an out-patient for infection, lymphoproliferative process, inflammatory bowel disease, drug induced dermatosis and autoimmune disease.

REFERENCES

  1. Kung EF, Arora A, Petronic-Rosic V, Tsoukas MM. Clinicopathologic challenge. Neutrophilic dermatosis of the dorsal hands--a variant of localized Sweet's syndrome. Int J Dermatol. 2009 Jun;48(6):555-7.
  2. Takahama H, Kanbe T. Neutrophilic dermatosis of the dorsal hands: a case showing HLA B54, the marker of Sweet's syndrome. Int J Dermatol. 2010 Sep;49(9):1079-80.
  3. Kung EF, Arora A, Petronic-Rosic V, Tsoukas MM. Clinicopathologic challenge. Neutrophilic dermatosis of the dorsal hands--a variant of localized Sweet's syndrome. Int J Dermatol. 2009 Jun;48(6):555-7.
  4. Wang YS, Tan A. Neutrophilic dermatosis of the dorsal hands: an emerging entity. J Eur Acad Dermatol Venereol. 2009 Apr;23(4):451-2.
  5. Duquia RP, Almeida HL Jr, Vettorato G, Souza PR, Schwartz J. Neutrophilic dermatosis of the dorsal of the hands: acral sweet syndrome? Int J Dermatol. 2006 Jan;45(1):51-2.
  6. Del Pozo J, Sacristán F, Martínez W, Paradela S, Fernández-Jorge B, Fonseca E. Neutrophilic dermatosis of the hands: presentation of eight cases and review of the literature. J Dermatol. 2007; 34:m243-7.
  7. Strutton G, Weedon D, Robertson I. Pustular vasculitis of the hands. J Am Acad Dermatol 1995; 32: 192-198.
  8. Hall AP, Coudge RJ, Ireton HJC, Burrell LM. Pustular vasculitis of the hands. Australas J Dermatol 1999; 40: 204-207.
  9. Galaria NA, Junkins-Hopkins JM, Kligman D, James WD. Neutrophilic dermatosis of the hands: pustular vasculitis revisited. J Am Acad Dermatol 2000; 43: 870-874.
  10. Weenig RH, Bruce AJ, McEvoy MT, Gibson LE, Davis MDP. Neutrophilic dermatosis of the hands: four new cases and review of the literature. Int J Dermatol 2004; 43: 95-102.
  11. DiCaudo DJ, Connolly SM. Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands. A report of seven cases and a review of the literature. Arch Dermatol 2002; 138: 361-365.
  12. Malone JC, Slone SP, Wills-Frank LA et al. Vascular inflammation (vasculitis) in Sweet syndrome. A clinicopathologic study of 28 biopsy specimens from 21 patients. Arch Dermatol 2002; 138: 345-349.
  13. Cohen PR. Skin lesions of Sweet syndrome and its dorsal hand variant contain vasculitis. An oxymoron or an epyphenomenon?. Arch Dermatol 2002; 130: 400-403.
  14. Curco N, Pagerols X, Tarroch X, Vives P. Pustular vasculitis of the hands: report of two men. Dermatology 1998; 196: 346-347.
  15. Benzimra J, Low-Beer J, Twomey J. A case of peripheral ulcerative keratitis associated with neutrophilic dermatosis of the dorsal hand. Int Ophthalmol. 2011 Apr;31(2):149-51.
  16. Fernández-Fernández FJ, Alvarez-Fernández JC, Romero-Picos E, Garrido JA, Sesma P. Neutrophilic dermatosis of the dorsal hands associated with a "myeloproliferative neoplasm, unclassifiable" and a simultaneous cancer of colon. Acta Medica (Hradec Kralove). 2010;53(3):153-6.
  17. Coelho JF, Lourenço S, Marques J, Mendonça P, Malhado JA. Neutrophilic dermatosis of the dorsal hands associated with chronic hepatitis C virus infection. Indian J Dermatol Venereol Leprol. 2008 Sep-Oct;74(5):478-80.
  18. Byun JW, Hong WK, Song HJ, Han SH, Lee HS, Choi GS, Shin JH. A case of neutrophilic dermatosis of the dorsal hands with concomitant involvement of the lips. Ann Dermatol. 2010 Feb;22(1):106-9. Epub 2010 Feb 28.
  19. Stransky L, Broshtilova V. Neutrophilic dermatosis of the dorsal hands elicited by thermal injury. Contact Dermatitis 2003; 49: 42.
  20. Aydin F, Senturk N, Yildiz L, Canturk M, Turanli A. Neutrophilic dermatosis of the dorsal hands in a farmer. J Eur Acad Dermatol Venereol 2004; 18: 716-717.
  21. Yung A, Merchant W, Sheehan-Dare R. Streptococcus induced pustular vasculitis affecting de hands resembling pustular vasculitis of the hands - first reported case. Clin Exp Dermatol 2005; 30: 366-368.
  22. Walling HW, Snipes CJ, Gerami P, Piette WW. The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. Arch Dermatol. 2006 Jan;142(1):57-63.
  23. Ayoub N, Tomb R. Neutrophilic dermatosis of the dorsal hands: a variant of erythema elevatum diutinum? Arch Dermatol. 2003 Jan;139(1):102.
  24. Gilaberte Y, Coscojuela C, García-Prats MD. Neutrophilic dermatosis of the dorsal hands versus pustular vasculitis. J Am Acad Dermatol. 2002 Jun;46:962-3

Contributed by Eumenia Castro, MD, PhD and Rajendra Singh, MD



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