EN + Eosinofilia

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A congenital melanocytic nevus of the scalp is occa-

sionally associated with intracranial pigmentation, which


can be a benign asymptomatic coexisting melanomatosis
or an invasive extension of the cutaneous lesion or a
paracrinopathy from the overlying nevus.
6
Although the differential diagnosis of pigmentation and
bone abnormality includes Albright syndrome, it could be
excluded based on pathologic diagnosis of pigmentary
lesion. In Albright syndrome, pigmentation should be of
type caf-au-lait spots, not melanocytic nevus.
10
To our
knowledge, this is the rst case of congenital melanocytic
nevus of the scalp with underlying skull defect.
Although the association between a congenital melano-
cytic nevus of the scalp, underlying skull defect, and the
development of melanoma from the congenital melanocy-
tic nevus of the scalp are unclear, we suggest that clini-
cians should evaluate the skull in the patients who have
congenital melanocytic nevi of the scalp and have regular
follow-up examinations.
In our case, the small size of the specimen obtained by
a 3-mm punch biopsy instead of total excision might not
be representative for histopathological examination and
patient was informed and recommended regular visits at
least every 6 months.
Yeon Sook Kwon, MD
Jin Mo Park, MD
Hee Jung Kim, MD
Min-Geol Lee, MD, PhD
Department of Dermatology and Cutaneous Biology
Research Institute
Yonsei University College of Medicine
Seoul
Korea
E-mail: mglee@yuhs.ac
Funding sources: None.
Conict of interest to disclosure: None declared.
The authors have no relevant nancial interest in this
article.
References
1 Tannous ZS, Mihm MC Jr, Sober AJ, et al. Congenital
melanocytic nevi: clinical and histopathologic features,
risk of melanoma, and clinical management. J Am Acad
Dermatol 2005; 52: 197203.
2 Reed WB, Becker SW Sr, Becker SW Jr, et al. Giant
pigmented nevi, melanoma, and leptomeningeal
melanocytosis. Arch Dermatol 1965; 91: 100118.
3 Ho N, Roig C, Diadori P. Epidermal nevi and localized
cranial defects. Am J Med Genet 1999; 83: 187190.
4 Menter MA, Griessel PJC, de Klerk DJ. Giant blue
naevus of the scalp with underlying skull defect. Br J
Dermatol 1971; 85: 7375.
5 Silverberg GD, Kadin ME, Dorfman RF, et al. Invasion
of the brain by a cellular blue nevus of the scalp. A case
report with light and electron microscopic studies. Cancer
1971; 27: 349355.
6 Findler G, Hoffman HJ, Thomson HG, et al. Giant
nevus of the scalp associated with intracranial
pigmentation. Case report. J Neurosurg 1981; 54: 108
112.
7 Marano SR, Brooks RA, Spetzler RF, et al. Giant
congenital cellular blue nevus of the scalp of a newborn
with an underlying skull defect and invasion of the dura
mater. Neurosurgery 1986; 18: 8589.
8 Madaree A, Ramdial PK, Du Trevou M. Giant congenital
naevus of the scalp and cranium: case report and review
of the literature. Br J Plast Surg 1997; 50: 2025.
9 Kousseff BG. Hypothesis: Jadassohn nevus phakomatosis:
a paracrinopathy with variable phenotype. Am J Med
Genet 1992; 43: 651661.
10 Albright F, Butler AM, Hampton AO, Smith P.
Syndrome characterized by osteitis brosa disseminata,
areas of pigmentation and endocrine dysfunction, with
precocious puberty in females: report of ve cases. New
Eng J Med 1937; 16: 727746.
Erythema nodosum with eosinophilic panniculitis
Erythema nodosum (EN) is manifested by the sudden
onset of tender erythematous nodules and plaques on the
legs. The lesions show septal panniculitis chiey inl-
trated by lymphohistiocytes. Inltrated eosinophils are
usually in a minority. We describe here a patient with EN
who showed septal panniculitis with an admixture of
numerous eosinophils.
A 41-year-old Japanese woman visited our hospital
with a 3-month history of a painful eruption affecting the
left lower leg. She was in otherwise good health and used
no medication. Examination showed a subcutaneous nod-
ule, 9 10 cm, with an erythematous surface on the ante-
rior of left lower leg (Fig. 1). A skin biopsy specimen
showed the presence of lymphohistiocytic inltrate chiey
in the septa of the subcutaneous tissues with minor inl-
tration near the septa (Fig. 2a). The overlying dermis had
a moderate, deep perivascular inltration. In the subcutis,
the inltrate was admixed with a number of eosinophils,
some of which showed degranulation (Fig. 2b). Clusters
of eosinophils were also present. Staining for fungi or
acid-fast bacilli was negative. Based on clinical and histo-
pathologic ndings, we diagnosed this case as EN with
eosinophilic panniculitis. Full blood count, electrolytes,
2010 The International Society of Dermatology International Journal of Dermatology 2010, 49, 960969
Correspondence 965
liver function tests, and antistreptolysin O titer were all
within normal limits. No peripheral eosinophilia was
found. An intradermal tuberculin test was positive
(20 18 mm induration). However, there were no abnor-
mal shadows on chest X-ray and no colonies of Mycobac-
terium tuberculosis in the sputum culture. Two months
later the lesion cleared spontaneously, and the patient has
had no recurrence of the skin lesion over the following
period of 12 months.
The inltrates in EN chiey consist of lymphohistio-
cytes, and eosinophils are usually in a minority. On the
other hand, eosinophilic panniculitis shows prominent
inltration with eosinophils in septa and/or lobules of the
subcutis. As eosinophilic panniculitis is associated with a
diverse range of local and systemic diseases including EN,
parasitic infection, and hypereosinophilic syndrome,
eosinophilic panniculitis is a histopathologic pattern
rather than a distinct entity.
14
We consider our case as
EN with eosinophilic panniculitis, because the clinical
and histopathologic features were consistent with those of
EN except numerous eosinophil inltration. EN is
thought to be a reactive phenomenon that may occur in
response to antigen stimuli. In our case, the exact patho-
mechanism is not understood, but the inltration may be
the result of some antigen expression which induces
eosinophils in the subcutis. In addition, eosinophilic inl-
tration may develop in several diseases, including non-
episodic type of angioedema with eosinophila, insect bite,
drug eruption, and Wells syndrome. However, our case
did not match that noted in them. Eosinophilic
inltration in EN may be more common than has been
thought.
Teie Egawa, MD, PhD
Ryuhei Okuyama, MD, PhD
Hachiro Tagami, MD, PhD
Setsuya Aiba, MD, PhD
(a)
(b)
Figure 2 Histopathologic nding: inltration in and near the
septa (a) and the admixture of eosinophils (b), some of which
showed degranulation (hematoxylin and eosin stain: a 100;
b 400)
Figure 1 Clinical nding: a subcutaneous nodule with an
erythematous surface on the lower leg
International Journal of Dermatology 2010, 49, 960969 2010 The International Society of Dermatology
Correspondence 966
Department of Dermatology,
Tohoku University Graduate School of Medicine,
Aoba-ku,
Sendai, Japan
Ryuhei Okuyama
Department of Dermatology
Tohoku University Graduate School of Medicine
1-1 Seiryo-machi
Aoba-ku, Sendai 980-8574
Japan
E-mail: rokuyama@mail.tains.tohoku.ac.jp
References
1 Adame J, Cohen PR. Eosinophilic panniculitis: diagnostic
considerations and evaluation. J Am Acad Dermatol 1996;
34: 229234.
2 Burket JM, Burket BJ. Eosinophilic panniculitis. J Am
Acad Dermatol 1985; 12: 161164.
3 Winkelmann RK, Frigas E. Eosinophilic panniculitis: a
clinicopathologic study. J Cutan Pathol 1986; 13: 112.
4 Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, et al.
Fitzpatricks Dermatology in General Medicine. 7th
edn; New York: Mcgraw-Hill, 2008; 585.
Primary cutaneous peripheral T-cell non-Hodgkin
lymphoma, not otherwise specied, with cytotoxic
features
We have read with great interest the recently published
case report by Yamamoto et al.
1
describing a patient with
primary cutaneous aggressive T-cell lymphoma that
exhibited cytotoxic phenotype, however, without the
expression of CD8 antigen. In the light of this publica-
tion, we would like to present a similar patient history,
which, in our opinion, provides further data in regard of
this unique type of cutaneous lymphoma.
A 53-year-old man, with a 25-year history of tobacco
smoking, presented with a large, slightly painful ulcera-
tion in the right groin. The lesion started as a small, dark
reddish nodule that progressed within 2 weeks into a rap-
idly growing ulceration. About 6 months later, when the
patient came to our department, a moist ulceration of
about 35 cm diameter with a raised, inltrated border
was found (Fig. 1a). Until then only topical and systemic
antimicrobial agents were administered by the patient as
treatment. The patient was in good general condition;
peripheral lymph nodes were not enlarged and no other
clinically relevant abnormalities were stated during physi-
cal examination. The family history was irrelevant. Abdo-
men sonography revealed only mild hepatomegaly. The
patient also had slight leukocytosis (11 000 ll) with neu-
trophilia (74%) and accelerated erythrocyte sedimentation
rate (76 mm after 1 h). No other abnormalities were
found in laboratory and imaging examination (including
chest CT scan). To establish the diagnosis, a biopsy was
performed from the ulcer edge. The histology showed a
dense inltrate of medium- to large-sized lymphoid cells,
involving mainly dermis and lower layers of epidermis
(Fig. 2a,b). Majority of these cells were positive for CD3
(Fig. 2c), CD5, CD7, Ki-67, granzyme B (Fig. 2e), and
TIA-1 (Fig. 2f), while only some of them (< 20%) showed
expression of CD30. The tumor cells were also negative
for CD4, CD8, CD20, CD25, CD34, CD56, CD57,
CD68, CD117, pancytokeratin, HMB-45, Melan A, S100
protein, myeloperoxidase, perforin, ALK-1, and alfa-
actin. The CD8 positive staining was only restricted to
small cells representing a reactive inltrate (Fig. 2d). In
(a)
(b)
Figure 1 (a) Primary cutaneous epidermotropic cytotoxic lym-
phoma manifesting as an isolated, large ulceration with a
raised border in the right groin. (b) Local relapse of the tumor
2010 The International Society of Dermatology International Journal of Dermatology 2010, 49, 960969
Correspondence 967

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