Neutrophilic dermatosis of the dorsal hand L98.2

Strutton et al. 1995

Neutrophilic dermatosis of the back of the hand is a rare disease that is often misdiagnosed as a localized skin infection. It is classified as a variant of neutrophilic dermatosis(Sweet syndrome) and is clinically reminiscent of pyoderma gangraenosum.

w>m; prevalence unknown, probably underestimated.

The average age of the patients was 62.1 years (Micallef D et al. 2023)

Patients with NDH exhibit fever, peripheral neutrophilia, leukocytosis and/or an increased erythrocyte sedimentation rate or an increased level of C-reactive protein, albeit to a much lesser extent than in Sweet syndrome.

Dermatologically, it is characterized by tender erythematous plaques, pustules and bullae on the back of the hands. Overall, both backs of the hands were affected in 78.0% of cases, and in almost a third of cases other areas were also affected (Micallef D et al. 2023). In contrast to Sweet syndrome, in which bullae are quite rare, especially in the early stages, bullae and ulcerations also occur in NDH.

Associated diseases are found in about 40% of patients, with the most common associations being hematologic diseases (21% - gammopathies, myelodysplasias or malignancies), recent bacterial and viral infections, vaccinations, solid organ tumors and inflammatory bowel disease (19%).

Epidermal hyperplasia, dense neutrophilic dermatitis, no evidence of leukocytoclasia.

Small vessel vasculitis: Cutaneous small vessel vasculitis is characterized by a palpable purpura that often occurs on the extremities and may rarely be bullous. Histology shows leukocytoclasis, neutrophil/mixed infiltrates, extravasated erythrocytes and fibrinoid necrosis. Neutrophilic dermatosis of the dorsal hands (NDDH) was originally described as pustular vasculitis1 but was renamed NDDH due to the dense neutrophilic infiltrates in the skin; the vasculitis may vary in severity in early lesions.2,3

Classical pyoderma gangraenosum: Pyoderma gangrenosum (PG) begins with painful pustules that develop into ulcerations with rolled, purple edges. Classic PG usually occurs on the lower extremities and is associated with significant pain. Histologically, PG may show neutrophilic infiltrates with or without leukocytoclastic vasculitis.

Dermatitis herpetiformis: Dermatitis herpetiformis is a pruritic eruption associated with celiac disease and manifests as small vesicles on the extensor surfaces of the extremities and buttocks.

Intestinal-associated dermatosis-arthritis syndrome: Intestinal-associated dermatosis-arthritis syndrome is a non-infectious neutrophilic dermatosis that includes erythematous patches, papules and vesiculopustules on the proximal extremities and trunk and is associated with arthritic symptoms that often occur after bariatric surgery. Histologically, the lesions show perivascular, nodular neutrophilic infiltrates with skin edema.

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

Treatment with corticosteroids usually leads to a rapid improvement with complete healing. Rapid improvement can also be achieved with low-dose therapy with dapsone (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. (2023) Neutrophilic dermatosis of the dorsal hands: A review of 123 cases. J Am Acad Dermatol 88:1338-1344.
5. Paparone PP et al (2013) Neutrophilic dermatosis of the dorsal hand. Wounds 25:148-152.
6. Walling HW et al. (2006) The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. Arch Dermatol 142:57-63 (illustrations by Claudia)
7. Wolf R et al. (2017) Acral manifestations of Sweet syndrome (neutrophilic dermatosis of the hands). Clin Dermatol 35:81-84.