Neutrophilic Dermatosis of the Dorsal Hands L98.2

Last updated on: 22.09.2022

Definition
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Neutrophilic dermatosis of the dorsum of the hand is a rare, localized variant of Sweet syndrome. The disease was first described in 1995. It is characterized by erythematous plaques, pustules, and blisters on the dorsum of the hand (Micallef D et al. 2019).

Occurrence/Epidemiology
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Manifestation
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The average age of the patients is 62.1 years

Clinical features
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Patients with this disease presented with mild fever and erythematous plaques, pustules, and blisters confined to the dorsum of the hands and fingers, characterized histologically by a dense neutrophilic infiltrate with leukocytoclasia. In a larger review, bilateral involvement of both hands was seen in 78.0% of cases. In almost one third of the cases, other skin areas were also affected (note: differentiation from Sweet syndrome!). In about 40 % of the patients an underlying disease was found, the most frequent associations being hematologic diseases (gammopathies, myelodysplasias or malignancies), recent infections, tumors of solid organs and inflammatory bowel diseases.

Histology
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Biopsy specimens showed a neutrophilic infiltrate of the dermis with leukocytoclasia but without evidence of necrotizing vasculitis. Histologically, the picture was reminiscent of Sweet syndrome.

Therapy
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Systemic and/or topical corticosteroids were used for treatment in 88.1% of cases, while dapsone, colchicine, and tetracyclines were the most commonly used steroid-sparing agents.

Progression/forecast
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Frequently, rapid improvement occurred with low-dose dapsone. Complete healing was the norm (Galaria NA et al. 2000).

Literature
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  1. Byun JW et al (2010) A case of neutrophilic dermatosis of the dorsal hands with concomitant involvement of the lips. Ann Dermatol 22:106-109. - Fig
  2. DiCaudo DJ et al (2002) Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands: a report of 7 cases and review of the literature. Arch Dermatol 138:361-365.
  3. Galaria NA et al (2000) Neutrophilic dermatosis of the dorsal hands: pustular vasculitis revisited. J Am Acad Dermatol 43:870-874.
  4. Micallef D et al (2019) Neutrophilic dermatosis of the dorsal hands: A review of 123 cases. J Am Acad Dermatol S0190-9622(19)32678-7.

Incoming links (1)

Sweet syndrome;

Outgoing links (1)

Sweet syndrome;

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Last updated on: 22.09.2022