REVIEW ARTICLE
The Grenz Zone
Ossama Abbas, MD,* and Meera Mahalingam, MD, PhD, FRCpath†
Abstract: The grenz zone is a narrow area of the papillary dermis
uninvolved by underlying pathology. Historically believed to be a
feature unique to granuloma faciale, this feature has also been observed
in other cutaneous inflammatory conditions, infectious entities, and
neoplastic benign and malignant tumors. This review attempts to
enumerate cutaneous entities commonly displaying a grenz zone with
an emphasis on histopathological features that help in their differen-
tiation. It also attempts to answer the obvious question of why select
entities have this histopathologic feature by ascertaining the defining
structure of a grenz zone.
Key Words: grenz zone
(Am J Dermatopathol 2013;35:83–91)
INTRODUCTION
Historically, the use of “grenz”, a German word mean-
ing “border”, dates back to the 18th century with descriptions
of the Grenz infantry or Grenzers, which were light infantry
troops coming from the Croatian and Transylvanian Military
Frontier in Habsburg Monarchy (later the Austrian Empire
and Austria-Hungary).1 At the start of the war, the Grenz
infantry regiments formed about a quarter of the Habsburg
army. Grenzers were the successors to the irregular army of
Pandurs, which were also raised as a militia by the Habsburgs
in the 18th century to defend the border with the Ottomans
and also used as skirmishers in the 7 Years’ War.1
In cutaneous pathology, this zone is used to describe a
narrow uninvolved zone of papillary dermis that typically
occurs between the epidermis and the underlying inflamma-
tory or neoplastic infiltrate. It was initially reported as a
feature purportedly characteristic of granuloma faciale (GF).2
Later, in an article entitled “Dermal alterations with age and
sun damage”, the presence of a zone of normal-appearing
connective tissue just beneath the epidermis and overlying
actinic keratoses was noted in association with argyrophilic
fibers.3 The authors believed that this zone was produced as
new connective tissue, forming continually at the epidermal–
dermal junction, grows downward. However, this unique feature
has also been observed with other cutaneous inflammatory
dermatoses, infectious entities, and benign and malignant
neoplastic processes (Table 1).
From the *Department of Dermatology, American University of Beirut Medical
Center, Beirut, Lebanon; and †Department of Dermatology, Dermatopa-
thology Section, Boston University School of Medicine, Boston, MA.
The authors have no funding or conflicts of interest to declare.
Reprints: Meera Mahalingam, MD, PhD, FRCPath, 609 Albany Street, J-301,
Boston, MA 02118 (e-mail: mmahalin@bu.edu).
Copyright © 2013 by Lippincott Williams & Wilkins
Am J Dermatopathol  Volume 35, Number 1, February 2013
ETIOPATHOGENESIS
The obvious question is what is the defining structure of
a grenz zone? Why is it observed in only select entities?
Given the diverse etiopathogenesis, it would seem that it is
a function of structural alterations in this area. The depth of
the elastosis below the level of penetration of ultraviolet light
from natural sunlight, clarified by studies in amphibians,
suggests that newly formed dermal collagen is first deposited
at this site.4 It has been shown that in the lamprey, as in man,
the collagen fibrils abutting the epidermis are small but
mature becoming larger in the deeper dermal layers.3 This
led to the conclusion that “the epidermis grows outward
and the dermis grows inward, possibly pushing newly syn-
thesized elastotic fibers along with it”.3 The authors believed
that this satisfactorily explained the depth of elastotic fibers in
actinic elastosis below the level of penetration of ultraviolet
light and believed that this “also suggests that actinic elastosis
may be partially reversible by avoidance of further sun dam-
age because new connective tissue is forming continually at
the epidermal–dermal junction and growing downward.”
Favoring this theory, regression of actinic elastosis has been
reported in transplanted actinically damaged skin.5
In case of B lymphocyte–rich cutaneous infiltrates, the
grenz zone seems to form due to specific adhesive properties
of B lymphocytes and the effect of chemokinetic and chemo-
tactic signals that may influence the migration of B lympho-
cytes up until this “barrier layer”.6,7 Favoring, this is the fact
that B lymphocytes are typically not “epidermo- or folliculo-
tropic” as they, unlike T lymphocytes, do not seem to express
integrin receptors for specific membrane components.6 Thus,
B lymphocytes are only rarely detected in significant numbers
in the epidermis or papillary dermis, producing the character-
istic grenz zone seen in B-cell–rich cutaneous infiltrates. The
presence of epidermal Langerhans cells (LCs) perhaps partly
explains the selective affinity for the epidermis by T and not
B lymphocytes. Recent evidence indicates that in cutaneous
T-cell lymphoma, the interaction of epidermal LCs with
tumoral lymphocytes plays a crucial role in mediating epider-
motropism.8 The fact that LCs serve as antigen-presenting
cells for CD4-positive T lymphocytes explains, albeit in part,
the rare detection of B lymphocytes at the dermoepidermal
junction and in the papillary dermis.9
ENTITIES CONSISTENTLY EXHIBITING A
GRENZ ZONE
Inflammatory Dermatoses
Granuloma faciale
The most characteristic histopathologic feature of GF is
the presence of a grenz zone, seen in 74%–100% of GF
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Abbas and Mahalingam Am J Dermatopathol  Volume 35, Number 1, February 2013

that the presence of a grenz zone can no longer be used to

TABLE 1. Differential Diagnosis of Cutaneous Lesions With
differentiate between these 2 entities which have several over-
a GZ
lapping histopathologic features to begin with. The pathogen-
Entities Exhibiting a Grenz Zone on Microscopy esis of EED is not yet completely understood. It is currently
Entities Consistently Entities Uncommonly Emerging Entities believed to be a localized fibrosing small-vessel vasculitis that
Exhibiting a GZ Exhibiting a GZ Exhibiting a GZ probably develops as a reaction to persistent immune com-
Inflammatory Neoplastic Neoplastic plex deposition or as a hypersensitivity reaction to an, as yet,
GF Generalized eruptive Primitive non-neural undefined antigen.12,15–18 In favor of an autoimmune etiology
histiocytosis- granular tumor are reports of an association between EED and other autoim-
Leiomyoma
mune-mediated entities such as monoclonal or polyclonal
EED Atypical fibroxanthma TRAPP
gammopathies (especially immunoflobulin A hyperglobuline-
Infectious Metastatic melanoma CCS
mia),15 and rheumatoid arthritis.12 It may be that activation via
Leprosy
cytokines such as interleukin-8 allows for a selective recruit-
Post–kala-azar
dermal ment of leucocytes to tissue sites, whereas immune com-
leishmaniasis plexes and bacterial peptides sustain the persistent local
Lobomycosis inflammatory infiltrate and the leukocytoclastic vasculitis.19
Neoplastic
CLH
DF INFECTIOUS ENTITIES
Cutaneous B cell
lymphoma Leprosy
Leukemia cutis Histopathologically, a well-defined grenz zone is char-
DM acteristically observed in lepromatous leprosy (LL) (including
Primary dermal histoid leprosy which is a distinctive LL variant commonly
melanoma after partial chemotherapy or acquired drug resistance leading
GZ, grenz zone.
to bacterial relapse), but also in borderline lepromatous (BL),
and, albeit less commonly, in midborderline, (Figs. 1E, F).20–23
Of interest, one study demonstrated bacilli in the grenz zone
cases.10–12 In the largest study done on GF, 66 patients were that were found within macrophages and fibroblasts.24 More
evaluated, and a grenz zone was observed in 54 (74%) cases recently, acid-fast bacilli have been demonstrated even in the
(Figs. 1A, B).11 Additional features observed included the subepidermal zone of normal-appearing skin of all leprosy
presence of telangiectases (74%), hemosiderin deposition types with the number of bacilli increasing in the spectrum
(70%), leukocytoclasis (66%), dilated follicular ostia/keratotic from tuberculoid to LL.25 Based on this, the authors concluded
plug (61%), dermal fibrosis (45%), extravasated erythrocytes that the presence of acid-fast bacilli in this subepidermal zone
(19%), and rarely necrotizing vasculitis (7%). The precise may be of significance for transmission and dissemination of
pathogenesis of GF is unclear. One study revealed that the the disease.25
majority of nonmyelocytic hematopoetic cells present were of
the T-helper immunophenotype. The lymphocytes stained Post–kala-azar Dermal Leishmaniasis
strongly with anti–interleukin-2 receptor and with antilym- Only few reports describe the histopathology of post–
phocyte functional antigen 1a antibodies, suggesting that kala-azar dermal leishmaniasis, a chronic form of leishmaniasis
GF is a g-interferon–mediated process.13 The overlying ker- that clinically presents initially as hypopigmented macules
atinocytes did not stain with intercellular adhesion molecule over the trunk and extremities that progressively become ery-
1. A more recent study demonstrated high local interleukin thematous evolving into nonulcerated papules and nodules
5 levels possibly produced by clonally expanded skin-specific with marked involvement of the face. Although the hypopig-
CD4+ cells.14 Other views suggest that GF is a localized and mented macules exhibit a superficial perivascular lympho-
chronic cutaneous vasculitis with an autoimmune etiopatho- cytic infiltrate with scattered plasma cells and a decrease in
genesis.10–12 epidermal melanin, the papules and nodules show a dense
diffuse dermal mixed infiltrate composed of histiocytes, lym-
Erythema Elevatum Diutinum phocytes, and plasma cells with an overlying narrow grenz
Histopathologically, early lesions of erythema elevatum zone and an atrophic epidermis.26 In one study of 25 cases,
diutinum (EED) usually demonstrate leukocytoclastic vascu- a subepidermal clear zone, was seen in 11 of 23 (48%) biopsy
litis, whereas late lesions reveal a diffuse mixed-cell infiltrate specimens from nodules and in 3 of 20 (15%) specimens from
with a predominance of neutrophils, fibrosis, granulation macules.27 The precise reason for the presence of a grenz zone
tissue, and, occasional foci of leukocytoclastic vasculitis in some cases but not in others is not known.
(Figs. 1C, D). The absence of a grenz zone in EED has
usually been considered as a criterion that helps in the differ- Lobomycosis
entiation of EED from GF.15–18 However, more recently, Histopathological findings of lobomycosis include
Ziemer et al12 observed that, similar to GF, a well-defined a narrow grenz zone separating an atrophic epidermis from
grenz zone can be seen in 75% of EED cases, concluding an underlying dense dermal infiltrate of macrophages with

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Am J Dermatopathol  Volume 35, Number 1, February 2013 Grenz Zone

FIGURE 1. Representative examples of entities consistently exhibiting a grenz zone. GF: hematoxylin and eosin, low power (A); high
power (B); EED: hematoxylin and eosin, low power (C); high power (D); leprosy: hematoxylin and eosin, low power (E); Fite stain (F).

admixed giant cells, scattered lymphocytes and plasma cells. reports have demonstrated that, despite the presence of a grenz
Numerous organisms with a distinctive “lemon-like” appear- zone, transepidermal elimination of parasites occurs mainly
ance (averaging 10 mm in diameter) may be detected within through the infundibular epithelium.29,30 However, the signif-
and outside of the inflammatory cells.28 Of interest, several icance of this finding is not clear.

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Abbas and Mahalingam
NEOPLASTIC ENTITIES
Cutaneous Lymphoid Hyperplasia
A grenz zone is typically present in most cases of
cutaneous lymphoid hyperplasia (CLH) (Figs. 2A, B).31,32 In
a study comparing CLH and cutaneous marginal zone lym-
phoma (MZL), a grenz zone was identified in 9 of 15 cases
(60%) of CLH compared with 8 of 9 cases (89%) of MZL.31
In a study on 106 cases of Borrelia burgdorferi–associated
lymphocytoma cutis, a grenz zone was present in 93 of 106
(88%) cases.33 The grenz zone seems to form due to specific
adhesive properties of B cells, the effect of chemokinetic and
chemotactic signals that may influence the migration of B
cells up until this “barrier layer”, and the role of LCs in
serving as antigen-presenting cells mainly for CD4-positive
T lymphocytes.6,9
Dermatofibroma
A grenz zone can be seen in up to 70% (87 of 122 cases
in one study) of dermatofibroma (DF) cases and in most
variants of DF.34 In the same study, there were no specific
subtype differences based on presence of the grenz zone. In
another study on 59 cases of atypical DF, a grenz zone was
detected in 15 (25%) cases.35 Although the underlying path-
ogenesis for the formation of grenz zone in DF is unclear, it is
a well-established fact that DFs commonly exhibit “follicular
induction” in response to unidentified factors. This makes the
presence of a grenz zone in DF somewhat puzzling (Fig. 2C,
D). In a study comparing 59 DF cases with overlying follic-
ular basal cell hyperplasia (FBCH) to 199 DF cases without
FBCH, a statistically significant association was found
between the presence of FBCH and loss of the grenz zone.36
B-Cell Lymphomas
Histopathologically, most if not all variants of cutane-
ous B-cell lymphomas (CBCL) are characterized by a nodular
and/or diffuse dermal infiltrate with an overlying grenz
zone.7,31 Although a well-defined grenz zone can be seen in
the different types of primary B-cell lymphomas, it is more
commonly seen in primary cutaneous marginal zone B-cell
lymphoma (PCMZL) (MALT type) and cutaneous involve-
ment secondary to B-cell lymphoproliferative disorders such
as precursor B-cell lymphoblastic lymphoma and chronic
lymphocytic leukemia/small lymphocytic lymphoma. In one
study comparing CLH and PCMZL, a grenz zone was iden-
tified in 8 of 9 (89%) cases of MZL, whereas in another study,
a grenz zone was observed in all 22 cases of PCMZL.31,37 In
a recent study, epidermotropism was reported in only 2 of 53
cases of primary cutaneous large B-cell lymphoma, leg type
(PCLBCL-leg type), whereas a classical grenz zone was dem-
onstrated in 51 of 53 (96%) PCLBCL-leg type cases and in all
26 cases of PCLBCL-other.38 Although a grenz zone is also
commonly noted in secondary cutaneous lesions of precursor
B-cell lymphoblastic lymphoma, Shafer et al39,40 recently
demonstrated the presence of a grenz zone in skin biopsies
of 2 adult patients with primary cutaneous precursor B-cell
lymphoblastic lymphoma. Reasons for grenz zone formation
in select B-cell lymphomas are not fully defined but seem to
be the same as those for CLH.
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Am J Dermatopathol  Volume 35, Number 1, February 2013
Leukemia Cutis
Histopathologic findings in leukemia cutis vary depend-
ing on the leukemia subtype.41 Irrespective of the primary
entity, there is usually a perivascular, periadnexal, and/or
diffuse leukemic cell infiltrate with variable cellular cytologic
atypia depending on the leukemia subtype and an overlying
well-defined grenz zone. In one study on the specific cutane-
ous infiltrates in 26 patients with myelogenous leukemia, a
grenz zone was noted in all cases.42 In a study evaluating the
microscopic features of 55 biopsy specimens of 34 patients
with leukemia cutis, a grenz zone was observed in 61% of the
cases.43 In an earlier study on 42 cases of leukemia cutis,
a grenz zone was observed in 27 of 42 (64%) cases.44 How-
ever, the reason for its formation remains unknown.
Desmoplastic Melanoma
More recently, 2 distinct histopathologic subtypes of
desmoplastic melanoma (DM) with differing clinical corre-
lates have been recognized—the pure variant composed pre-
dominantly of spindled cells and a mixed variant that has a
significant epithelioid component.45,46 Although the reason
for its formation remains unknown, a grenz zone was dem-
onstrated in up to 41% of cases including 19 of 51 (37%)
cases of pure DM and 27 of 62 (44%) cases of mixed DM in
one study (Figs. 2E, F).45
Primary Dermal Melanoma
Although the reason for formation of a grenz zone is
unknown, only 1 study reported the presence of a grenz zone
in 6 of 7 (86%) cases of primary dermal melanoma, a term to
describe a melanoma subtype which histologically mimics
metastasis (involves the dermis and/or subcutaneous tissue)
and is associated with an unexpectedly prolonged survival
rate despite its depth (may show up to 100% survival rate on
5 years of follow-up.47
ENTITIES UNCOMMONLY EXHIBITING A
GRENZ ZONE
Generalized Eruptive Histiocytosis
Generalized eruptive histiocytosis is a rare cutaneous
histiocytosis that belongs to type IIa of histiocytic disorders in
which the lesional cells are believed to originate from dermal
dendrocytes.48 Clinically, it usually presents as symmetric
papules on face, trunk, and proximal extremities of adults
and may rarely be associated with underlying hematological
malignancies.49 Histopathologic features include dermal nod-
ular aggregates of large histiocytes with abundant granular to
foamy cytoplasm and irregular to indented nuclei intimately
admixed with lymphocytes and occasional epithelioid
cells.48,49 The presence of a grenz zone has been reported in
an anecdotal case report (Figs. 3A, B).48
Atypical Fibroxanthoma
Histopathologically, classical atypical fibroxanthoma
(AFX) is a dermal tumor with an epidermal collarette
composed of atypical, pleomorphic, epithelioid and spin-
dled cells arranged in disordered fascicles that commonly
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Am J Dermatopathol  Volume 35, Number 1, February 2013 Grenz Zone

FIGURE 2. Representative examples of entities consistently exhibiting a grenz zone. Benign lymphoid hyperplasia: hematoxylin
and eosin, low power (A); immunohistochemical stain CD20 (B); DF with follicular induction phenomenon: hematoxylin and
eosin low power (C); high power (D); DM: hematoxylin and eosin, low power (E); high power (F).

do not destroy adnexal structures and frequent atypical Cutaneous Leiomyoma

mitoses.50 A narrow grenz zone has been reported, albeit Histopathologic features of cutaneous leiomyoma
rarely, in association with (AFX) in 1 of 66 (1.5%) AFX include a well-circumscribed nonencapsulated tumor com-
cases.50 posed of interlacing fascicles of bland spindle cells with

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Abbas and Mahalingam Am J Dermatopathol  Volume 35, Number 1, February 2013

FIGURE 3. Representative examples of entities uncommonly exhibiting a grenz zone. Generalized eruptive histiocytosis: hema-
toxylin and eosin, low power (A); immunohistochemical stain CD68 (B); leiomyoma: hematoxylin and eosin, low power (C); high
power (D); metastatic melanoma: hematoxylin and eosin, low power (E); high power (F).

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Am J Dermatopathol  Volume 35, Number 1, February 2013 Grenz Zone

elongated nuclei and blunt ends. Mitotic activity up to 3
mitoses per 10 high-power fields may be seen in this entity.51
A narrow grenz zone overlying the proliferation has occasion-
ally been reported (Figs. 3C, D). In one study, 4 of 37 cases
(11%) showed a narrow grenz zone with overlying flattened
epidermis.52
Metastatic Melanoma
Among cutaneous metastases, metastatic melanoma
represents a significant common cause of skin metastasis and
may represent the first manifestation in up to 5% of patients.53
In a study evaluating 192 cases, 3 (1.6%) simulated DF and in
these cases, a narrow grenz zone was noted (Figs. 3E, F).53
In the largest series on 12 cases, cutaneous CCS typically
presented as a nodule on the extremities of young adults with
a female predominance.58 Cutaneous CCS usually involves
the dermis with occasional extension to the superficial sub-
cutaneous tissue.58 The tumor consists of delicate fibrous
septa encasing cellular aggregates that consist of nests and
fascicles of pale epitheloid and fusiform cells with clear or
finely granular, eosinophilic cytoplasm and oval elongated
nuclei with prominent nucleoli. Multinucleate giant cells
and scattered mitoses are also commonly observed. CCS
shows consistent evidence of melanocytic differentiation. A
grenz zone, not mentioned to date at least in the literature, is
a frequent observation (Figs. 4A, B).58

EMERGING ENTITIES EXHIBITING A CONCLUSIONS

GRENZ ZONE The enigma of the grenz zone continues. We still do not
know why this area is histopathologically spared in some
T-Cell–Rich Angiomatoid
Polypoid Pseudolymphoma
T-cell–rich angiomatoid polypoid pseudolymphoma
(TRAPP), a newly described variant of pseudolymphoma,
typically presents as a solitary, polypoid, erythematous,
2.5–7.5 mm papule mainly on the head, neck, and trunk
of adult females that clinically can resemble a pyogenic
granuloma.54 Histopathological features of TRAPP include
an epidermal collarette surrounding a dense dermal poly-
clonal infiltrate composed of a predominantly CD3-positive
T-lymphocyte–rich infiltrate with admixed B-lymphocytes,
plasma cells, histiocytes, and eosinophils.54 A well-defined
grenz zone has been described in association with (TRAPP)
in 11 of 17 (65%) cases in one study.54

Dermal Nonneural Granular Cell Tumor

Dermal nonneural granular cell tumor is a recently
described benign tumor that shows no obvious line of
differentiation.55,56 It usually presents as painless nodules
mainly on the extremities or face of young to middle-aged
adults, with slight female predominance. Histopathologically,
it is composed of polygonal or round cells to elongated spin-
dle-shaped cells with prominent granular cytoplasm and well-
defined cellular borders.56 Tumor nuclei are usually vesicular
and may show mild focal atypia and, rarely, moderate atypia
with mitotic activity ranging from 1 to 9 mitoses per 10 high-
power fields. Immunohistochemically, tumor cells are usually
positive for NKI-C3 but negative for S100 protein.56,57 A
grenz zone is a common finding as it has been observed in
2 of 2 (100%) cases in one report55 and 8 of 13 (62%) cases in
another.57

Clear Cell Sarcoma

Clear cell sarcoma (CCS), also known as malignant
melanoma of the soft parts because of the presence of melanin
and cytoplasmic melanosomes, is a rare soft-tissue tumor that
is currently considered as a distinctive neoplasm because of
the unique presence of the t(12;22)(q13;q12) translocation.58
It is characterized by multiple local recurrences with late FIGURE 4. Representative example of an emerging entity
metastases and a high tumor death rate. Although the tumor is exhibiting a grenz zone. CCS: hematoxylin and eosin, low power
usually deep seated, a cutaneous variant has been described. (A); high power (B); Per kind courtesy of H. Kutzner.

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Abbas and Mahalingam
entities and yet involved in others. Although the histological
differential diagnosis of cutaneous entities exhibiting a grenz
zone may be broad, an accurate diagnosis is usually reached
based on the characteristic clinical, histological, and immu-
nohistochemical features of each of the different entities that
may show this peculiar histological feature.
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