Dermatology Online Journal

A rare case of idiopathic neutrophilic dermatosis of the hands
Ellecia Cook DO, Rachel Epstein DO, Richard Miller DO
Dermatology Online Journal 17 (11): 11

NOVA Southeastern University Largo Medical Center, Largo, Florida

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Abstract

Neutrophilic dermatosis of the hands is a rare localized variant of Sweet syndrome. The following is a case report of a 68-year-old man who presented to our clinic with progressive redness, swelling, and decreased mobility of the fingers. Examination revealed symmetric, violaceous, edematous, annular plaques and nodules on the dorsal and lateral surfaces of the thumb and index fingers. Biopsy demonstrated a dense neutrophilic infiltrate in the papillary dermis without evidence of vasculitis, changes consistent with Sweet syndrome. A thorough work up revealed no concurrently associated condition. Treatment with prednisone 10 mg daily, colchicine 0.6 mg twice daily, and pentoxifylline 400 mg three times daily resulted in significant improvement in this case of idiopathic neutrophilic dermatosis of the hands.

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Case report

Figure 1	Figure 2
Figures 1 and 2. Violaceous, edematous plaques and nodules on the dorsal and lateral surfaces of the thumb and index fingers

Figure 3	Figure 4
Figures 3 and 4. Dense dermal neutrophilic infiltrate with no evidence of vasculitis

A 68-year-old male presented to our clinic with progressive redness and swelling of the bilateral fingers for seventy-two hours, along with decreased mobility of the involved digits. Past medical history was significant for asthma, osteoarthritis, coronary artery disease, hypertension, hypercholesterolemia, and depression. The patient denied any constitutional symptoms or recent respiratory or gastrointestinal infection. Physical examination revealed symmetric, violaceous, edematous, annular plaques and nodules on the dorsal and lateral surfaces of the thumb and index fingers (Figures 1 and 2). Histologic evaluation revealed a dense neutrophilic infiltrate in the papillary dermis without evidence of vasculitis, changes consistent with Sweet syndrome (Figures 3 and 4). The only significant findings upon a thorough work up, including blood work and imaging, was an elevated erythrocyte sedimentation rate and positive rheumatoid factor. Evaluation by the rheumatology department concluded that there was not enough clinical or laboratory evidence of systemic rheumatologic disease. Therefore, in this case, no associated condition was found, making this a case of idiopathic neutrophilic dermatosis of the hands. The patient was started on prednisone 10 mg daily, along with colchicine 0.6 mg twice daily and pentoxifylline 400 mg three times daily by the rheumatology consultant with significant improvement one month following initial presentation (Figures 5 and 6).

Figure 5	Figure 6
Figures 5 and 6. Significant improvement one month following presentation and treatment

Discussion

Robert Douglas Sweet first described Sweet syndrome (SS) in 1964 [1]. SS, also known as acute febrile neutrophilic dermatosis is one entity in a group of inflammatory disorders called non-infectious neutrophilic dermatoses. SS usually starts acutely, with high-grade fever, diffuse pain, and generalized malaise. A recent history of respiratory or gastrointestinal infection is common. Patients often present with tender inflammatory papules coalescing into irregular plaques. Histology reveals an intense neutrophilic infiltrate, papillary dermal edema, and leukocytoclastic vasculitis [2]. A common finding on laboratory evaluation is leukocytosis and polymorphonuclear leukocytes over 10,000 mm, but this may not always be present, such as in the current case [1, 3]. The following are included in the differential diagnosis of SS: pyoderma gangrenosum, erythema multiforme, urticarial vasculitis, erythema elevatum diutinum, panniculitis, halogenoderma, pyoderma, septic vasculitis, deep fungal infection, mycobacterial infection, leishmaniasis, lymphoma cutis, and metastatic carcinoma [3]. The presence of 2 major criteria and 2 minor criteria is required for the diagnosis of SS. Major criteria include the abrupt onset of typical cutaneous lesions and histopathology consistent with SS [2]. Minor criteria include antecedent fever or infection, accompanying fever, arthralgia, conjunctivitis, underlying malignancy, leukocytosis, and a good response to systemic corticosteroids without response to antibiotics [2]. Systemic corticosteroids are the therapeutic gold standard for SS [3]. Potassium iodide and colchicine are other first-line oral systemic agents. Indomethacin, clofazamine, cyclosporine, and dapsone are second-line oral systemic agents [3]. High potency topical corticosteroids and intralesional corticosteroids can be utilized in localized lesions [3]. Spontaneous resolution with no therapeutic intervention occasionally occurs [3].

Neutrophilic dermatosis of the hands (NDH) is a localized variant of Sweet syndrome [2]. The presentation of violaceous papulonodules on the radial aspect of the dorsal hands was first described by Strutton et al and five years later the name, neutrophilic dermatosis of the dorsal hands, was coined by Galaria [2, 4, 5]. A little over 60 cases have been reported since then. The majority of cases involve the dorsal hands, but there have been a minority of cases involving the lateral and palmar aspect of the hand. Thus, “dorsal” was dropped from the name [6]. The literature suggests that atypical pyoderma gangrenosum, SS, and pustular vasculitis of the hands are variations of a single disease entity designated NDH [2]. NDH has been associated with the following conditions: acute myelogenous leukemia, ulcerative colitis, metastatic lung cancer, B cell lymphoma, influenza vaccine, glomerulonephritis dialysis, streptococcus tonsillitis, chemotherapy, hypopharyngeal carcinoma, IgA gammopathy, Crohn disease, seropositive arthritis, sarcoidosis, hepatitis C, urinary tract infection, trauma, breast carcinoma, colon carcinoma, renal carcinoma, laryngeal carcinoma, thalidomide, and fertilizer exposure [2, 4-10, 12, 13]. Diagnosis and treatment of NDH is identical to that of classical SS. NDH has been misdiagnosed as infectious in a number of cases and treated unnecessarily with antibiotics. One case reports a patient who was misdiagnosed with gangrene, with the involved digit being amputated. Lesions reappeared at the base of the amputation and was clinically diagnosed as NDH by a dermatologist and verified by histologic evaluation of the amputated digit [11]. Furthermore, this patient was found to have an underlying myelodysplastic syndrome. Therefore, dermatologists should be able to recognize NDH and initiate a thorough work up including blood work and imaging studies for potential associated conditions. In addition, long term follow up is recommended to monitor for the development of associated diseases.

In conclusion, neutrophilic dermatosis of the hands is a rare localized variant of Sweet Syndrome. Idiopathic cases, as the case presented here, represent the minority of cases. The vast majority are associated with potentially serious systemic conditions. This warrants awareness of NDH in the dermatologic community allowing for clinical recognition and a prompt, thorough work-up.

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