Correspondence 345

References 4 Bart BJ, Lussky RC. Bart syndrome with associated

1 Taifour Suliman TM, Quazi A. Aplasia cutis congenita of anomalies. Am J Perinatol 2005; 22: 365–369.
the trunk in a Saudi newborn. Br J Plast Surg 2004; 57: 5 Smith SZ, Cram DL. A mechanobullous disease of the
582–584. newborn. Bart’s syndrome. Arch Dermatol 1978; 114:
2 Bigliardi PL, Braschler C, Kuhn P, et al. Unilateral aplasia 81–84.
cutis congenita on the leg. Pediatr Dermatol 2004; 21:
454–457.
3 Zelickson B, Matsumara K, Kist D, et al. Bart’s syndrome.
Ultrastructure and genetic linkage. Arch Dermatol 1995;
131: 663–668.

Coexistence of bullous rheumatoid neutrophilic matoid neutrophilic dermatosis and palmoplantar pustu-
dermatosis and palmoplantar pustulosis in a patient losis in a patient with rheumatoid arthritis is presented.
with rheumatoid arthritis A 65-year-old female, suffering from severe seropositive
Rheumatoid arthritis is a chronic systemic inflammatory rheumatoid arthritis over 20 years, complained of skin
disorder that primarily involves the joint synovial mem- eruptions on the lower legs. She was currently treated with
brane. Various cutaneous manifestations are associated low-dose prednisolone and nonsteroidal anti-inflammatory
with rheumatoid arthritis (Table 1), including palisaded drugs. A physical examination revealed 5 mm sized, tense
neutrophilic granulomatous dermatitis, vasculitis, and vesicles and erosions scattered on the inner aspects of the
neutrophilic disorders. In this paper, coexistence of rheu- bilateral lower legs, accompanied by small pustules, scales,
and crusts on the sole (Fig. 1a,b). Potassium hydroxide
Table 1 Representative cutaneous manifestations associated (KOH) tests were negative, and cultures from the pustules
with rheumatoid arthritis on the sole were sterile. Also, mild erythemas, scales, and
a few pustules were found on the palms. She denied smok-
Specific Nonspecific ing and had no pain of sternocostoclavicular regions. She
Necrobiotic conditions Characteristic neutrophilic disorders
mentioned that pustular lesions appeared on the bilateral
Rheumatoid nodule Pyoderma gangrenosum soles prior to 6 months, and recognized bullae on the
Palisaded neutrophilic Sweet’s syndrome lower legs 2 weeks before, which gradually increased in
granulomatous dermatitis Erythema elevatum diutinum number. A biopsy taken from the bullous lesion on the
Rheumatoid papule Palmoplantar pustulosis
lower leg revealed subepidermal bullae and prominent
Rheumatoid neutrophilic Vasculitis
dermatosis Miscellaneous conditions
neutrophil infiltration in the upper dermis, but no features
of vasculitis. Results of the direct immunofluorescence

(a) (b)

Figure 1 (a) Scattered vesicles on the

lower leg and small pustules, scales,
and crusts on the sole. (b) Close
view of the lower leg showing tense
vesicles.

ª 2010 The International Society of Dermatology International Journal of Dermatology 2010, 49, 334–348
346 Correspondence
Table 2 Summary of the patients with bullous rheumatoid neutrophilic dermatosis
Age/Sex Location Precedence (years)
56/f Elbow, forearm, thigh, palm, sole RA (10)
35/m Elbow, knee, forearm lower extremity RA (18)
78/f Lower extremity RA (15)
65/f Lower extremity RA (20)
tests were negative for deposition of IgA, IgM, IgG, and
C3. Although biopsies were not carried out from either
the palms or soles, the palmoplantar lesions were diag-
nosed as palmoplantar pustulosis. Topical corticosteroid
ointment has been applied to both lesions.
Rheumatoid neutrophilic dermatosis occurs in patients
with active and severe rheumatoid arthritis, and clinically
presents with symmetric erythematous papules, nodules,
plaques, urticaria-like erythemas, and rarely vesicles, over
the trunk and extremities. Bullous-type rheumatoid neutr-
ophilic dermatosis is rarely reported,1–3 which predomi-
nantly involves the lower extremities. The bullae are
tense, and scattered or grouped. Patients including our
case are three women and one man (Table 2). All cases
have severe disabling seropositive rheumatoid arthritis for
long periods; however, the mechanism of vesicle forma-
tion is uncertain. Effective treatments include oral dapson
and steroids.
The clinical manifestations of palmoplantar pustulosis
resemble psoriasis; namely, thin, scaly erythemas, or
rarely pustules occasionally appear on the extra-palmopl-
antar areas such as extremities and trunk. Because bullous
lesions were the main skin condition on the lower legs in
Pyoderma gangrenosum following surgical procedures
To Editor,
Pyoderma gangrenosum (PG) most often presents with
refractory, sterile, deep ulcers on the lower leg, in associa-
tion with inflammatory bowel syndrome, hematologic
malignancy, Takayasu’s arteritis, neutrophilic dermatoses,
and rheumatoid arthritis. It is well known that minor
trauma precipitates PG, which plays a role as pathergy;
however, the cause of PG is still unknown. Peristomal PG
and PG following mammoplasty reduction are frequently
seen. We herein describe two young females with PG
which occurred following appendectomy and cesarotomy,
respectively.
A 28-year-old female delivered her baby by cesarian
operation at a maternity clinic. Five days later, she com-
plained of redness and pain of the abdomen. Under the
suspicion of stitch abscess, systemic antibiotics were
administered; however, the ulcer was rapidly enlarged.
When she was referred to our hospital 18 d after the
International Journal of Dermatology 2010, 49, 334–348
Other diseases Treatment Ref.
– Spontaneous resolution 1
– Dapson (100 mg/d) 2
Diabetes mellitus, hypertension Prednisone (50 mg/d) 3
Palmoplantar pustulosis Topical steroid Present case
the presented case, the lesions were diagnosed as rheuma-
toid neutrophilic dermatosis, but not extra-palmoplantar
lesions of palmoplantar pustulosis. Both cutaneous lesions
in this case are speculated to be caused by activated
neutrophils, but other immunological, such as T-cell
mediated, mechanisms may also be involved.
Toshiyuki Yamamoto, MD, PhD
Department of Dermatology, Fukushima Medical
University, Fukushima, Japan
E-mail: toyamade@fmu.ac.jp
References
1 Lowe L, Kornfield B, Clayman J, Golitz LE. Rheumatoid
neutrophilic dermatitis. J Cutan Pathol 1992; 19: 48–
53.
2 Lu CI, Yang CH, Hong HS. A bullous neutrophilic
dermatosis in a patient with severe rheumatoid arthritis
and monoclonal IgA gammopathy. J Am Acad Dermatol
2004; 51: S94–S96.
3 Kreuter A, Rose C, Zillikens D, Altmeyer P. Bullous
rheumatoid neutrophilic dermatosis. J Am Acad Dermatol
2005; 52: 916–918.
delivery, a physical examination revealed a large, deep
ulceration with elevated edematous borders on the lower
abdomen (Fig. 1). The surface was reddish granulation
and the ulcer was surrounded with erythema. Cultures
for bacteria were sterile. Laboratory examination showed
increased white blood cell counts (25 700/ll), elevated
levels of erythrocyte sedimentation rate (ESR) (50 mm/h)
and C-reactive protein (CRP) (11.6 mg/dl), whereas
decreased level of serum IgG (702 mg/dl; normal range
870–1700). Histological examination showed dense infil-
tration of neutrophils and mononuclear cells in the lower
dermis and subcutaneous tissue. She was initially treated
with systemic prednisolone (40 mg/d), which resulted in
sufficient effects. After prednisolone was tapered at
10 mg/d, meshed skin graft was performed.
An 18-year-old female underwent appendectomy, but
soon developed suture diastasis. Two months later, she
again received surgery for a cosmetic procedure in the
department of plastic surgery. Three days later, she devel-
ª 2010 The International Society of Dermatology