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Panniculitis is classified histologically into
distinct categories based on the location of inflammation in the subcutaneous
tissue, and the presence of vasculitis. Nodular vasculitis (NV) is defined as
predominantly lobular panniculitis with large vessel vasculitis. We present the
case of a 73-year-old Japanese woman presenting with painful, annular,
erythematous plaques on the trunk and extremities. Each erythematous plaque
developed centrifugally and subsided with reticular pigmentation in a few
months. Histological examination revealed lobular panniculitis and vasculitis,
involving both the arteries and veins; thus, she was diagnosed with NV. The erythema
continued to flare intermittently, and systemic administration of prednisolone
alleviated the eruptions. We believe that the present case is unique in both
annular morphology and distribution of eruptions.
INTRODUCTION
Nodular vasculitis (NV) is defined
as predominantly lobular panniculitis with large vessel vasculitis [1-4]. The
indurated, painful, erythematous plaques of NV are typically found in the lower
extremities. Here, we present a rare case of annularly developing erythema
diagnosed histologically as NV, and the differential diagnosis
REPORT
OF CASE
A 73-years old Japanese woman presented with a 10-month history of
painful, annular, erythematous plaques on her trunk and extremities.
Examination revealed tender, infiltrated erythema, appeared as painful
infiltrated erythema and developed centrifugally and acquired an annular
pattern (Figure 1A). The erythematous plaque improved as they expanded
annularly without treatment for a few months (Figure 1B). On the annular
erythema, we observed partial crusts but no ulcers. Inside the annular erythema,
the plaques subsided with reticular pigmentation. The patient had a medical
history of non-insulin-dependent diabetes mellitus, but she did not experience
any fever, malaise, or arthralgia or other systemic symptom, since the
eruptions had developed.
With the exception of an elevated
C-reactive protein (CRP) level (4.39 mg/dL; normal range, 0–0.30 mg/dL),
results of laboratory investigations including pancreatic enzymes and liver
functions, were normal. Autoantibodies, such as anti-nuclear antibody, anti-DNA
antibody, proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA), and
myeloperoxidase ANCA (MPO-ANCA), were not detected.
Histological examination showed slight perivascular lymphocyte
infiltration in the upper dermis, with and erythrocytes leaking through the
dermis. In the fat lobules, dense infiltration consisting of lymphocytes and
neutrophils was found (Figure 2A). At the center of the fat lobule, we detected
vasculitis composed of neutrophils and lymphocytes, which involved both the
arteries and veins.
At the area of lobular panniculitis, we also observed granulomatous
vasculitis composed of histiocytes and giant cells (Figure 2B). Caseous necrosis was not found. Immunohistological testing
found no IgG, IgA, IgM, or C3 deposition around the blood vessels or in the dermal
epidermal basement membrane zone (DE-BMZ). Tubercular tests such as purified
protein derivative intradermal injection, mycobacteria culture, and QuantiFERON
test indicated negative results for tuberculosis.
Non-steroidal anti-inflammatory drugs were ineffective
in in providing symptomatic relief, and systemic administration of prednisolone
(20 mg/day) alleviated her pain, and the annular erythema flared intermittently.
After discontinuing prednisolone, the erythema relapsed occasionally. During
recurrence, dapsone therapy was attempted, but no effect was observed. However,
cyclosporine administration helped improve the erythema.
DISCUSSION
Bazan first described erythema induratum (EI) as “erythema indurated
scrofulosorum” to designate indurated erythema on the posterior lower legs [1].
Montgomery later proposed the term “nodular vasculitis” for this condition [2].
Currently, there appears to be a consensus for considering EI and NV as the
same entity for the most common variant of mostly lobular panniculitis with
vasculitis [3,4]. NV typically presents as purple-red erythema on the lower
legs, and developed to be brownish subcutaneous nodule. Histologically, the
inflammation is subcutaneous as lobular panniculitis with vasculitis [5]. There
is some controversy regarding the type of vasculitis and the site of vessels in
NV. In a histopathological analysis of 101 specimens with vasculitis in EI,
Segura et al reported that vasculitis was evident in 91 cases [6]. They found
that the vasculitis affected different vessels, such as small vessels at the
center of the fat lobule as well as large septal arteries or veins. In our
case, panniculitis was prominent in fat lobules and vasculitis was observed in
both the arteries and veins. Therefore, we made histopathological diagnosis of
NV, and ruled out polyarteritis nodosa cutanea (PNC), in which panniculitis is
mostly septal and vasculitis is neutrophilic.
Our case is unique in that the patient’s erythema developed in an annular
pattern and was found in atypical areas of the trunk or upper extremities.
Thus, it is important to consider other differential diagnoses given the
unusual presentation of NV in this case.
Erythema nodosum migrans is a
subtype of erythema nodosum [7,8]. The erythema appears on lower legs, and
develops centrifugally with central fading and healing and has a migratory
pattern. Histological examination reveals a mostly septal panniculitis, similar
to erythema nodosum, without the presence of ulcers or occurrence of subsidence
after improvement. Differentiating between erythema nodosum and EI
histologically can be difficult, but in our case, the panniculitis was lobular.
In lupus erythematosus profundus (LEP), the erythema is occurs in the
other areas such as the lower limbs and develops into a deep subsidence of the
affected area. The panniculitis is both lobular and septal with mucin
deposition, on DE-BMZ or wall vessels, deposition of immunoglobulin or
complement is found [3]. In our case, there was no deposition, and autoimmunity
was not detected serologically.
In antiphospholipid syndrome, reticular livedo or ulceration is found in
the presence of thrombosis caused by antiphospholipid antibody or lupus
anticoagulant. Histologically, thrombosis is prominent and vasculitis is
generally missing through the dermis to subcutaneous tissue. In our case we
observed reticular pigmentation after the improvement of erythema but not
livedo, and antiphospholipid autoantibodies were not detected serologically.
The difference between PNC and the present case warrants a detailed
discussion, as vasculitis is identified in subcutaneous tissue. Histological
examination of PNC reveals mostly septal panniculitis and leukocytoclastic
vasculitis [3]. Thus, the etiology of PNC is believed to be mainly vasculitic.
In comparison, histological study of NV shows predominantly lobular
panniculitis and the vasculitis is mostly granulomatous and subsequent to the
affected area of panniculitis. In our case, a specimen consisting of early
coin-sized erythema was found to have, septal panniculitis and no vasculitis.
Therefore, we consider our case to be septal panniculitis and diagnosed the
patient with NV.
Annular leukocytoclastic vasculitis (ALV) is a rare variant of
leukocytoclastic vasculitis and develops annularly. Criber et al first defined
ALV, reporting approximately 30 cases thus far [9-11]. The course and affected
area of ALV is similar to our case. However, in ALV, dapsone should result in
complete resolution of all lesions. Our case failed to respond to dapsone.
Furthermore, the vasculitis in ALV is dermal leukocytoclastic. Hence, our case
does not fulfill the diagnostic criteria of ALV.
Erythema
elevatum diutinum is considered a chronic form of chronic leukocytoclastic
vasculitis and can occasionally manifest with an annular pattern [12]. However
our case did not show any fibrosis in the dermis and dapsone was not effective.
The mechanism
of the annular form in NV for our case remains unknown. By reviewing the
differential diagnosis, some conditions of panniculitis with or without
vasculitis are suggested as an annular “variant” subtype. To the best of our
knowledge, our case is the first case of an “annular variant” of NV.
Furthermore in NV, plaques usually appear on the lower legs and atypical forms
of NV are noted involving shins, thighs, or other sites. Our case is also
unique with regard to the affected area, such as the trunk, buttock, and
thighs.
- Bazin E (1861) Leçons Thèoriques et Cliniques sur la Scrofule. (2nd edn.), Paris.
- Montgomery H, O’Leary PA, Barker NW (1945) Nodular vascular diseases of the legs: erythema induratum and allied conditions. JAMA 128: 335-345.
- Requena L, Sanchez Yus E (2001) Panniculitis. part II. mostly lobular panniculitis. J Am Acad Dermatol 145: 325-361.
- Sanz Vico MD, De Diego V, Sanchez Yus E (1993) Erythema nodosum versus nodular vasculitis. Int J Dermatol 32: 108-112.
- Chopra R, Chhabra S, Thami GP, Punia RP (2010) Panniculitis: Clinical overlap and the significance of biopsy findings. J Cutan Pathol 37: 49-58.
- Segura S, Pujol RM, Trindade F, Requena L (2008) Vasculitis in erythema induratum of bazin: A histopathologic study of 101 biopsy specimens from 86 patients. J Am Acad Dermatol 59: 839-851.
- Campalani E, Higgins E (2003) Erythema nodosum migrans. Clin Exp Dermatol 28: 679-680.
- Lazaridou E, Apalla Z, Patsatsi A, Trigoni A, Ioannides D (2009) Erythema nodosum migrans in a male patient with hepatitis B infection. Clin Exp Dermatol 34: 497-499.
- Cribier B, Cuny JF, Schubert B, Colson A, Truchetet F, Grosshans E (1996) Recurrent annular erythema with purpura: A new variant of leucocytoclastic vasculitis responsive to dapsone. Br J Dermatol 135: 972-975.
- Chiu CS, Chang YC, Chung WH, et al. (2004) Annular leucocytoclastic vasculitis induced by chlorzoxazone. Br J Dermatol 150:153.
- Imanishi H, Tsuruta D, Ishii M, Kobayashi H (2009) Annular leucocytoclastic vasculitis. Clin Exp Dermatol 34: 120-122.
- Di Giacomo TB, Marinho RT, Nico MM (2009) Erythema elevatum diutinum presenting with a giant annular pattern. Int J Dermatol 48: 290-292.
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