Neutrophilic Dermatosis of the Dorsal Hands L98.2

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).

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The average age of the patients is 62.1 years

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.

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.

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.

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

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.