Dermatology Online Journal

Neutrophilic dermatosis of the dorsal hands associated with hypopharyngeal carcinoma
Mariana Cravo MD, José C Cardoso MD, Oscar Tellechea MD PhD, Margarida R Cordeiro, José Pedro Reis MD, Américo Figueiredo MD PhD
Dermatology Online Journal 14 (7): 5

Clinic of Dermatology, University Hospital, Coimbra, Portugal. mariana.cravo@netcabo.pt

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Abstract

Neutrophilic dermatosis of the dorsal hands (NDDH) is a rare and recently described disorder regarded as a subset of neutrophilic dermatosis, similar to superficial pyoderma gangrenosum and pustular vasculitis. Many currently consider it to be a localized variant of Sweet Syndrome. We describe the case of a 63-year-old male patient with NDDH associated with hypopharyngeal carcinoma. A biopsy demonstrated changes consistent with those seen in Sweet Syndrome, with leukocytoclasia but no signs of true vasculitis. Treatment with oral prednisolone resulted in complete resolution of the lesions in 1 month, with no recurrences. The clinical presentation, the histological features, and the excellent response to oral corticosteroid therapy allowed us to classify this case as NDDH. We emphasize the rare association of this entity with a solid tumor.

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Introduction

Neutrophilic dermatosis of the dorsal hands (NDDH) is a rare and recently described disorder regarded as a subset of neutrophilic dermatosis, considered to be a localized variant of Sweet Syndrome (SS). We report a case of NDDH associated with a solid tumor, hypopharyngeal carcinoma.

Case report

A 63-year-old male patient presented with a one-month history of pustular plaques, localized to the dorsum and border of both hands, with central ulceration and centrifugal growth. The lesions progressed to slightly painful, ulcerated plaques with a pustular and erythematous border (Figs. 1 & 2). There were no systemic symptoms such as fever, malaise, or arthralgias; the patient had a history of hypopharyngeal carcinoma. Initially, cutaneous infection had been suspected and unsuccessfully treated with topic and systemic antibiotics.

Figure 1	Figure 2
Figures 1 & 2. Ulcerated plaques with a pustular and erythematous border on the dorsum and border of both hands.

Figure 3	Figure 4
Figures 3 & 4. Papillary dermal edema and intense dermal neutrophilic infiltrate with leucocytoclasia but lack of vasculitis (H&E, original magnification 25x and 100x)

Laboratory evaluation disclosed normal complete blood count, chemistry profile and erythrocyte sedimentation rate. Fungal, bacterial, and mycobacterial cultures of the specimens obtained from the lesions were also negative. A skin biopsy demonstrated papillary dermal edema and an intense dermal perivascular and interstitial neutrophilic infiltrate with leukocytoclasia, but no signs of vasculopathy (Figs. 3 & 4).

Altogether, the clinical presentation and the histological features were consistent with the diagnosis of NDDH. Although there was a history of hypopharyngeal carcinoma, tests to exclude more common associations with this dermatosis were performed, such as blood and urine cultures, serum protein immunoelectrophoresis, HCV and HBV serologies, bone marrow smear, Rh factor, SACE, lysozyme, autoantibodies (ANA, ANCA), bone X-rays, and thoracoabdominal CT. They all disclosed normal or negative results. Due to the fact that the patient didn't report any gastrointestinal symptoms, an endoscopic study was not performed.

Figure 5
Figure 5. Complete healing of the lesions with minimal scarring

Treatment with 32 mg oral prednisolone daily, gradually tapered, resulted in complete resolution of the lesions in one month with minimal scarring (Fig. 5). There were no recurrences, but the patient died 6 months later due to hypopharyngeal carcinoma.

Comment

In 1995 Strutton et al. described 6 patients with a dermatosis exclusively localized to the dorsal hands characterized by erythematous plaques, pustules, and hemorrhagic bullae. Clinical and histological findings were similar to SS, but showed evident leukocytoclastic vasculitis. Therefore, he named this entity pustular vasculitis of the hands [1]. In 2000, Galaria et al. described 3 cases that clinically resembled this entity but lacked leucocytoclastic vasculitis and systemic symptoms; he proposed the designation of NDDH [2]. It is almost consensual now, between clinicians and dermatopathologists, to consider NDDH as a subset of neutrophilic dermatosis and to recognize that superficial pyoderma gangrenosum, pustular vasculitis of the dorsal hands, and atypical SS represent points on a spectrum of the same entity, which is best named NDDH. Furthermore, this is recognized as a localized variant of SS, due to the clinical, histological, and treatment similarities between these two entities [2-9].

About 63 cases of this disorder have been reported [3-6, 10, 11]. NDDH is more common in women [3]. In the majority of the cases the lesions are entirely localized on the dorsal hands. Nevertheless, as in our case, there can be lateral hand or palmar involvement and similar lesions in other locations may be found [3-7, 12]. The occurrence of local pain or systemic symptoms, such as fever, malaise, arthralgias, or lymph node enlargement, is inconsistent [1-8, 10-14].

The most common associated disorders are hematologic (myelodisplasia, IgA gammopathy, B cell lymphoma) and inflammatory (ulcerative colitis, Crohn disease, seropositive arthritis, sarcoidosis). Infectious diseases (hepatitis C, urinary tract infection), trauma, and solid carcinomas (breast, colon, renal, lung, laryngeal) [3, 5, 6, 12, 13] are less frequently associated. There are also reports of this entity associated with the thalidomide derivative, lenalidomide [10], and with exposure to a chemical fertilizer containing ammonium nitrate and calcium salts [14].

Even though laboratory findings may disclose leukocytosis with neutrophilia, elevated erythrocyte sedimentation rate, or elevated C-reactive protein levels, patients may lack these features [3]. Histological changes consistent with SS, namely papillary dermal edema and an intense dermal neutrophilic infiltrate, are invariable. However, leucocytoclastic vasculitis and vasculopathy are inconsistent and may reflect differences in the temporal course of the disease, as well as in the degree of inflammation at the time of the biopsy [3, 5, 8, 9, 12, 15].

Several systemic treatments, including corticosteroids, colchicine, and dapsone, have effected a prompt response. Other less frequently used treatments include cyclosporine, azathioprine, methotrexate, potassium iodide, indomethacin, and minocycline. Reports show complete healing with minor or absent scarring [1-8, 10-12]. The use of topical corticosteroids/tacrolimus is effective when there is minor involvement [11, 12]. Despite the fact that the majority of the reported cases were treated with oral or topical drugs, there are also cases of spontaneous healing [5, 11, 14]. Most patients remain without recurrences; although, if they occur, they can be easily treated with oral steroids or dapsone and, if necessary, prevented with small maintenance doses of dapsone [2, 3, 7, 8, 13].

In the present case we emphasize the clinical presentation, the absence of systemic symptoms and laboratory changes, the presence of histological findings consistent with SS, the lack of leucocytoclastic vasculitis, the rare association with a solid tumor, and the excellent response to oral corticosteroids without recurrence. All these features together allow us to classify this case as NDDH.

References

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