Acute erythematous pustular plaques on the hands triggered by driving

Question

What is your diagnosis?

Answer

Diagnosis: Neutrophilic dermatosis of the dorsal hands (NDDH)

Investigations and follow-up

Histopathology revealed slight spongiosis in the epidermis, and dense neutrophilic infiltrate with focal leukocytoclasia in the reticular dermis [Figure 2]. A diagnosis of neutrophilic dermatosis of the dorsal hands (NDDH) was established. Blood tests revealed a borderline elevated C reactive protein (CRP) (6.35 mg/L; normal range 1–3) with remaining parameters within normal ranges. Malignancy screening revealed the absence of any neoplastic processes. Treatment with prednisone, 40 mg daily, was started in a tapering regimen for 4 weeks, resulting in complete resolution at the 2-month follow-up [Figure 3].

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Figure 3:

Improvement of neutrophilic dermatosis of dorsal hands after treatment.

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Discussion

Neutrophilic dermatosis of the dorsal hands (NDDH) was categorised as a localised variant of Sweet syndrome by Galaria et al.¹ in the year 2000. It was first described as ‘pustular vasculitis’ by Strutton et al. in 1995, who presented six patients with similar Sweet syndrome-like histopathological and clinical features limited to the dorsa of hands.²

Neutrophilic dermatosis comprises a group of skin disorders characterised by a dense dermal, epidermal, and/or subcutaneous neutrophilic infiltrate. Lesions are mostly located on the dorsum of the hands, nonetheless, they may present in other locations.¹ They manifest as papules, nodules plaques, bullae, and pustules, and may be associated with necrosis and ulceration giving an infectious appearance.² Hence, being a relatively new entity, it may be mistreated with antibiotics or unnecessary procedures.

When assessing a patient exhibiting cutaneous manifestations resembling NDDH, it is essential to explore a spectrum of differential diagnoses due to the overlapping clinical features. The differential diagnosis ranges from herpetiform dermatitis, erysipelas, erythema elevatum diutinum, contact dermatitis, pyoderma gangrenosum, dyshidrotic eczema, infections, or palmoplantar pustulosis.^3,4 In the present case, our differentials were contact dermatitis or neutrophilic dermatosis.

The diagnosis is established on histopathology characterised by the presence of a dense neutrophilic infiltrate in the dermis. Moreover, it is pathologically classified as a variant of Sweet syndrome with indistinguishable histological findings.³

Most patients have a history of triggering traumas, such as gardening injuries, cooking burns, intravenous line insertions, and intravenous drug use, which present within 7–10 days after the event. Furthermore, it is crucial to intentionally interrogate for a history of trauma, as the pathergy phenomenon is described in other neutrophilic disorders.⁵

Some haematological disorders, recent infections, solid organ tumours, and inflammatory bowel disease have been associated with NDDH. Therefore, a thorough systematic evaluation with full blood count and serum protein electrophoresis is recommended to exclude any associated diseases. The first-line treatment is systemic steroids. However, many cases have been successfully treated with topical steroids.³