CLINICAL HISTORY:

Signs and symptoms:  A 41 yo Caucasian male presented to our clinic with the above chief complaint stating the lesions began to develop after he sustained an abrasion to his right third finger while at work. He began to note multiple similar lesions developing shortly thereafter and was admitted to a local hospital for presumed cellulitis . Unable to tolerate IV vancomycin, he was started on a short course of oral antibiotics and was referred to our service by the infectious disease physician for evaluation to rule out presumed pyoderma gangrenosum.

Previous Treatment:  Hospital treatment consisted of IV vancomycin and a p.o. course of Bactrim without improvement. A two week course of topical fluocinomide 0.1% cream and cephalexin 500mg TID was also unsuccessful.

Other information:  No pertinent past medical history. Social history included a 1-2 pk/day tobacco use, occasional ETOH with history of remote abuse, no illicit drug use, and no recent travel. Family hx was unremarkable. Review of systems significant for three year unintentional thirty pound weight loss. Due to lack of medical insurance, this had not been investigated previously.

PHYSICAL EXAM:

Multiple, well-circumscribed, erythematous/violaceous, ulcerated pustular plaques of the bilateral dorsal hands

LABORATORY TESTS:

CBC: RBC of 3.57 (low). Hg 13.3 (low), MCV 117.4 (high), MCH 37.3 (high), RDW 18.3 (high)
RBC morphology: 2+ macrocytosis, 1+ anisocytosis, Burr cells and hypersegmented neutrophils
CMP: WNL
Slightly elevated triglycerides, cholesterol, & LDL

DERMATOHISTOPATHOLOGY:

4mm punch biopsy showed a neutrophilc infiltrate within the dermis. There was extensive papillary dermal edema. Blood vessels away from the neutrophilic infiltrate showed no evidence of acute vasculitis. There was some vascular damage in the areas of dense neutrophilia with leuckocytoclasis. PAS staining unremarkable.

Actual histopath slides unavailable at time of publication. Photto courtesy of www.ojrd.com

DIFFERENTIAL DIAGNOSIS:

1.   Bullous pyoderma gangrenosum
2.   Deep fungal infection
3.   Neutrophilic dermatosis of the dorsal hands
4.   Erythema elevatum diutinum
5.   Cutaneous small vessel vasculitis

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Neutrophilic dermatosis of the dorsal hands

DISCUSSION:

Neutrophilic dermatosis of the dorsal hands is a fairly recently described disorder. Clinically, lesions are characterized by edematous, pustular or ulcerative nodules or plaques localized to the dorsal hands. Lesions frequently resemble those of pyoderma gangrenosum and/or Sweet’s syndrome. They are described by patients as exquisitely tender and painful, and rapidly progressive. They may or may not be associated with preceding or concurrent febrile illness. Misdiagnosis of localized cutaneous infection is common. Of the limited number of cases reported, some patients convey a history of minor trauma or chemical exposure to the hands prior to the eruption, while others have no such preceding events. 1-4
This entity was first reported in 1995 by Strutton, Weedon, and Robertson.5 They described lesions limited to the dorsal hands that histologically were characterized by a dense neutrophilic infiltrate, similar to that seen in neutrophilic dermatoses. At that time, the authors considered this a variant of pustular vasculitis because all of their cases demonstrated a leukocytoclastic vasculitis. In similar cases, evidence of vasculitis was notably absent, so the term neutrophilic dermatosis of the dorsal hands (NDDH) was proposed by Galaria et al1, who consider this entity as a localized subset of Sweet’s syndrome. Gilaberte et al3 reported a case with clinical and histological findings similar to those described by Galaria et al and concluded that the presence or absence of vasculitis depends on the evolution of the lesions.
Because relatively few reported cases of NDDH exist, the possible systemic associations in these patients are somewhat unclear. In previous reports, associations of uncertain significance have included a history of breast cancer, bowel disorders (diverticulosis, diverticulitis, acute proctitis), prior pharyngitis, concurrent UTI, metastatic renal adenocarcinoma, and Raynaud disease with arthritis.1-4 A definitive link between NDDH and systemic disease processes has yet to be made. If NDDH is truly a subset of Sweet’s syndrome, an association with preceding upper respiratory infection, myeloproliferative disorders, myelocytic leukemia, visceral malignancies, inflammatory bowel disease, connective tissue diseases, pregnancy, and drug reactions may be a possibility.2
Treatment of NDDH is similar to that of Sweet’s syndrome. Good response has been reported with both oral prednisone and dapsone. Topical steroids, systemic antibiotics, and anti-fungal agents have no proven role in the treatment of NDDH. 1-4

TREATMENT:

Our patient was started on 40mg/day of oral prednisone with 60% clearance by week 2. He was tapered off prednisone and dapsone was initiated for long term management.

At this time he requires a full hematologic and gastrointestinal workup to rule out possible underlying systemic disease that may be contributing to this dermatosis. Due to his lack of health insurance, he has declined any further evaluation at this time.

REFERENCES:

1. Galaria NA, Junkins-Hopkins JM, Kligman D, James WD. Neutrophilic dermatosis of the dorsal hands: pustular vasculitis revisited. J Am Acad Dermatol 2000;43:870-874
2. DiCaudo D, Connolly S. Neutrophilic Dermatosis (Pustular Vasculitis) of the Dorsal Hands. Arch Dermatol 2002;138:361-365
3. Gilaberte Y, Conscojuela C, Garcia-Prats MD. Neutrophilic dermatosis of the dorsal hands versus pustular vasculitis. J Am Acad Dermatol 2002;46:962-963.
4. Aydin F, Senturk N, Yildiz L, Canturk MT, Turanli AY. Neutrophilic dermatosis of the dorsal hands in a farmer. Euro Acad Dermatol Venereo 2004;18:716-717
5. Strutton G, Weedon D, Robertson I. Pustular vasculitis of the hands. J Am Acad Dermatol 1995; 32:192-198.

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