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Periocular Intralymphatic Histiocytosis Or Localized Melkersson-Rosenthal Syndrome?

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We describe three cases of periocular edema with histopathologic features of intralymphatic histiocytosis without extravascular granulomas. All were elderly males with no other significant medical problems. Previous reports of periocular Melkersson-Rosenthal syndrome are identical clinically, and some reports show illustrations of intralymphatic histiocytosis histopathologically, in addition to other features typical of the syndrome. Given the lack of associated diseases or other features of the Melkersson-Rosenthal triad, some of these cases may be better defined as periocular intralymphatic histiocytosis.
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J Cutan Pathol 2015: 42: 289 –294
doi: 10.1111/cup.12419
John Wiley & Sons. Printed in Singapore
© 2014 John Wiley & Sons A/S.
Published by John Wiley & Sons Ltd
Journal of
Cutaneous Pathology
Periocular intralymphatic
histiocytosis or localized
MelkerssonRosenthal syndrome?
We describe three cases of periocular edema with histopathologic
features of intralymphatic histiocytosis without extravascular
granulomas. All were elderly males with no other signicant
medical problems. Previous reports of periocular
MelkerssonRosenthal syndrome are identical clinically, and
some reports show illustrations of intralymphatic histiocytosis
histopathologically, in addition to other features typical of the
syndrome. Given the lack of associated diseases or other features
of the MelkerssonRosenthal triad, some of these cases may be
better dened as periocular intralymphatic histiocytosis.
Keywords: histiocytes, intravascular, lymphatic,
MelkerssonRosenthal syndrome, periocular
Emanuel PO, Lewis I, Gaskin B, Rosser P, Angelo N. Periocular
intralymphatic histiocytosis or localized MelkerssonRosenthal
syndrome?.
J Cutan Pathol 2015; 42: 289294. © 2014 John Wiley & Sons A/S.
Published by John Wiley & Sons Ltd
Patrick O Emanuel1,2,
Isabelle Lewis1, Brent Gaskin3,
Paul Rosser3and Neville
Angelo2
1University of Auckland, Auckland, New
Zealand,
2Diagnostic Medlab, Ellerslie, Auckland, New
Zealand, and
3Auckland Eye, Auckland, New Zealand
Patrick O Emanuel
Auckland District Health Board, 10 Harrison
Road, Ellerslie, Auckland 1071, New Zealand
Tel: +64 9 5716448
Fax: +64 9 7648737
e-mail: patricke@adhb.govt.nz
Accepted for publication October 27, 2014
Intralymphatic histiocytosis has now been well
described in quite a diverse range of clinical
scenarios which were well encapsulated in a
large cases series and review by Requena et al.1
Intralymphatic histiocytosis was rst described
by O’Grady et al.2Then Rieger et al.3described
two similar patients, one of them with a history
of rheumatoid arthritis. Autoimmune disease as
a possible etiologic factor was further reinforced
by Pruim et al.4who described two patients
with intralymphatic histiocytosis, also with
long-standing rheumatoid arthritis. Less than
50% of the cases described to date appear to
have suffered from known rheumatoid arthritis.
Disease associations have included malignancy,
joint replacements and what appears to be a
signicant idiopathic group. Given that intra-
lymphatic histiocytes are seen with a specic
diagnosis as well as in isolation, it may be that
intralymphatic histiocytosis may be regarded as
a tissue reaction pattern.
Clinically, intralymphatic histiocytosis mani-
fests as quite non-descript erythematous patches
or plaques. Livedoid, papular, vesicular, or nodu-
lar lesions have been described.1Histopatholog-
ically there are dermal lymphatics containing
aggregates of mainly histiocytes, with adjacent
perivascular lymphoid aggregates.
Reports of patients (Table 1)
Patient 1
A 74-year-old man presented with asymptomatic
progressive bilateral periocular edema without
erythema over a period of approximately 8
years (Fig. 1). Numerous biopsies were per-
formed leading to various diagnoses including
rosacea, dermatomyositis and folliculitis. Ocu-
loplastic surgery was performed and in these
larger specimens there were convincing foci of
intralymphatic histiocytosis (Fig. 2) in addition
to Demodex infestation, dermal edema and
289
Emanuel et al.
Table 1. Summary of cases
Case Diagnosis
Age
(years) Sex
Unilateral or
bilateral
Other Melkersson
Rosenthal syndrome
clinical features Other pathologic features Demodex
1 Intralymphatic histiocytosis 74 M Bilateral No Similar to rosacea Yes
2 Intralymphatic histiocytosis 61 M Bilateral No No Focally
3 Intralymphatic histiocytosis 78 M Bilateral No Seborrheic keratosis Yes
Fig. 1. Patient 1. A 74-year-old man presented with asymp-
tomatic progressive bilateral periocular edema without ery-
thema over approximately an 8-year period.
Fig. 2. A hallmark of this disorder is dilated lymphatic spaces
stuffed with histiocytes and associated lymphocytes.
lymphangiectasis (Fig. 3). The surgery resulted
in a good cosmetic and functional result (post-
op 1 year). Doxycycline 30 mg was also adminis-
tered.
Patient 2
A 61-year-old man presented with mild
progressive bilateral periocular edema over
Fig. 3. The dermis shows massive edema.
Fig. 4. The dermis shows lymphangiectasia.
a period of approximately 2 years (Fig. 4).
His past medical history was largely unremark-
able aside from sinus surgery 5 years earlier.
Excisions targeted at a good cosmetic result
were performed on each upper eyelid and
revealed intralymphatic histiocytosis in addition
to dermal edema. There were Demodex species
but these were difcult to nd. The cosmetic
result was satisfactory following the surgery.
There has been no recurrence six months post-
surgery.
290
Periocular intralymphatic histiocytosis?
Fig. 5. Patient 2. A 79-year-old man presented with mild asymp-
tomatic progressive bilateral periocular edema over approxi-
mately 2 years.
Fig. 6. In addition to intralymphatic histiocytosis, there were
numerous dilated follicles infested with Demodex species.
Patient 3
A 78-year-old man presented with numerous
non-melanoma skin cancers that were variously
excised and biopsied. A seborrheic kerato-
sis was biopsied on the eye. Adjacent to this
were numerous dilated follicles infested with
Demodex species. There was also underlying
intralymphatic histiocytosis and dermal edema
(Fig. 5). Subsequent communication with the
treating surgeon revealed mild lid edema. Unfor-
tunately, the patient did not consent to a clinical
photograph but consented to the pathologic
images being used.
In none of these patients were there extravas-
cular granulomas. None of these patients have
any history of an autoimmune or connective
tissue disease. In all patient biopsies, endothe-
lial cells lined dilated lumina and expressed
immunoreactivity for CD31 (Fig. 6), CD34
Fig. 7. In all cases, immunoreactivity with CD31 highlighted
the lymphatic lining as well as some of the histiocytes. D2-40
also highlighted the endothelium, indicating lymphatic origin.
Fig. 8. In all cases, the intralymphatic mononuclear cells were
positive for CD68.
and D2-40, conrming lymphatic origin. Intra-
lymphatic mononuclear epithelioid cells were
positive for CD68 (Fig. 7) and in areas for CD31
(Fig. 8). S-100 protein, CD1a and cytokeratin
were negative in the intravascular mononuclear
cells.
Discussion
Localized eyelid edema is caused by an
obstructed lymphatic system resulting from
surgical obstruction, radiation, malignancy,
infection, intralymphatic carcinomatous emboli
or rare other causes. One of the rare causes
is MelkerssonRosenthal syndrome, which is a
locally inltrative granulomatous disease causing
a triad of idiopathic eyelid or lip edema, facial
palsy and ssured tongue. The eyelid swelling
291
Emanuel et al.
Table 2. Similar cases described in the literature
Case Diagnosis
Age
(years) Sex
Unilateral or
bilateral
Other microscopic
features Demodex
10676788 Cockerham
et al.11
MR 74 M Bilateral Not mentioned Not mentioned
Belliveau et al.13 MR 60 M Bilateral Focal extravascular
granulomas
Not mentioned
Rawlings et al. case 15MR 73 M Bilateral Perifollicular granulomas Present in
photomicrograph
Rawlings et al.5MR 59 M Unilateral Dermal granulomata Not mentioned
Rawlings et al. case 45MR 52 F Bilateral Dermal granulomata No; granulomatous
folliculitis mentioned
Shapiro et al.14 MR 41 M Unilateral Dermal granulomata Not mentioned
Chen et al.6MR 45 M Unilateral Dermal granulomata Not mentioned
Requena et al.1Intralymphatic
histiocytosis
68 M Unilateral Dermal granulomata Not mentioned
is painless and non-erythematous.5Usually,
extravascular granulomas are seen associated
with vascular ectasia, inammation and edema
in this disorder.6
Our three cases highlight a clinicopatho-
logic scenario which bares striking similarities
with cases previously described as periocu-
lar MelkerssonRosenthal syndrome.712 In
addition to the clinical similarities, intra-
lymphatic collections of histiocytes have
also been described in lesions of periocular
MelkerssonRosenthal syndrome (Table 2),712
One of the cases presented by Requena et al.1
was similar to ours clinically (and was clinically
thought to be MelkerssonRosenthal syndrome
by the referring surgeon). However, in their
description, there are sterile suppurative gran-
ulomas and the authors question whether this
case could be best classied as ‘granulomatous
lymphangitis’.15,16
It is also interesting to note that previous cases
of periocular MelkerssonRosenthal syndrome
have occurred without other features of the
disease triad (note: the complete symptom
complex is estimated to occur in only 25% of
cases17). It could be that our cases lie at one
end of the spectrum of this disorder, a con-
cept recently proposed by Kaminagakura and
Jorge.18 They described two histopathologic
forms of MelkerssonRosenthal syndrome: In
the complete form, the inammation was char-
acterized by non-necrotizing granulomas; in the
monosymptomatic form, the inammatory cells
were dispersed within lymphatics and connective
tissues without granuloma formation. However,
given the given the sole periocular involvement
and the absence of extravascular granulomata,
the proposal that this is a manifestation of
MelkerssonRosenthal syndrome may appear
untenable.
The pathogenesis MelkerssonRosenthal syn-
drome remains a mystery. Certainly some cases
represent a manifestation of Crohn’s disease and
sarcoidosis.19 For the large group of patients who
have no associated disease, numerous theories
have been proposed, e.g. allergies, chronic infec-
tion and immune dysregulation.11,14 We found it
interesting that Demodex infestation was identi-
ed in all three cases described herein. In two
of the cases, the infestation was impressive and
associated with follicular dilation and a perifollic-
ular chronic inammatory inltrate. Demodex
and folliculitis can also be seen in two previously
reported cases.5These ndings raise the possi-
bility that rosacea could be a contributing patho-
genetic factor.
Rosacea was raised as a diagnostic possibility in
two of the cases histopathologically (case 1 and
3). In contrast to the described cases, the bilat-
eral eyelid swelling in periocular rosacea usually
also is erythematous and telangiectatic. Gran-
ulomatous rosacea features clusters of epithe-
lioid histiocytes, sometimes with caseation necro-
sis requiring special stains to rule out mycobac-
terial infection. Intralymphatic granulomas and
histiocytic aggregates have not been previously
described.6
The histopathologic differential diagno-
sis of intralymphatic histiocytosis includes
intravascular reactive angioendotheliomato-
sis, intravascular carcinoma or melanoma,
intravascular lymphoma, mesothelial prolifera-
tions and other inammatory conditions with
intralymphatic collections.1,20
292
Periocular intralymphatic histiocytosis?
The intravascular cells in intralymphatic
histiocytosis are usually CD68-positive and
sometimes also exhibit some CD31 positiv-
ity. S100, CD1a and cytokeratin markers are
negative that excludes melanoma, carcinoma
and other histiocytoses such as Langerhans or
RosaiDorfman disease. Intravascular-reactive
angioendotheliomatosis can be excluded with
lymphatic endothelial markers. As seen in our
cases, D2-40 stains the lymphatic endothelium
but not the histiocytes. Other lymphatic markers
(podoplanin, Lyve-1 and Prox-1) can serve the
same function.1
Immunohistochemical studies can also allow
distinction from multilocular mesothelial
proliferation20 that may be morphologically sim-
ilar. These rare lesions arise from the peritoneal
mesothelium following abdominal surgery.
Immunohistochemical staining for calretinin
and D2-40 will be positive in this disorder.
A recent report describes striking periorbital
edema with giant cell angiobroma and cases of
lymphedema with ‘giant cell angiobroma-like’
changes.21 Interestingly, they show some over-
lapping features with cases reported herein
and those previous reported as perioccular
MelkerssonRosenthal syndrome, namely, der-
mal edema, vascular ectasia and granulomas.
However, intralymphatic histiocytosis is not a
noted feature with giant cell angiobroma.
Additionally, the lesional cells of giant cell
angiobroma are spindled or multinucleated
and CD34-positive. There were isolated stellate
broblasts in the dermis, but these were few in
number and similar to broblasts commonly
seen in an edematous stroma. We therefore
believe there are sufcient histopathologic
differences to warrant distinction.
Described cases of intralymphatic histiocytosis
have had a chronic progressive clinical course.
Numerous therapies have been tried with dis-
appointing results, including radiotherapy,
corticosteroids, cyclophosphamide and various
antibiotics. Periocular MelkerssonRosenthal
syndrome also has minimal therapeutic options.
Surgical debulking provided a good cosmetic
result in patients presented herein but recur-
rence is certainly a possibility given the nature
of the process. Given that two of our patients
were thought to have unusual rosacea at some
point, these cases were trialed on a low dose of
doxycycline which apparently had some effect.
Low-dose doxycycline has been used to some
effect in some instance.6,22
In conclusion, we describe three cases of
periorbital edema that histopathologically
conrmed intralymphatic histiocytosis. These
cases bare striking similarities with previous
reports of periocular MelkerssonRosenthal
syndrome. Given the lack of other features
of the triad and lack of other histopathologic
ndings, we believe it is more sensible to
regard these cases as a variant of intralymphatic
histiocytosis.
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... 10,11 The clinical differential diagnosis in such cases may be broad, and includes thyroidassociated orbitopathy (an autoimmune condition affecting the orbital and periorbital soft tissue), angioneurotic edema (angioedema), Ascher syndrome (eyelid swelling, thyroid enlargement, "double upper lip"), connective tissue diseases, and rosacea. [12][13][14] Considering angioedema, patients typically experience focal pruritus, and the swelling resolves quickly when the offending agent is discontinued. 15 The treatment of facial swelling in MRS is a point of difficulty due to its idiopathic nature and tendency to progress/recur despite therapy. ...
... 20 Other therapies have also included radiotherapy, corticosteroids, cyclophosphamide, methotrexate, and antibiotics such as dapsone and doxycycline to variable effect. 12,13,25 In conclusion, we present an unusual presentation of MRS, which is itself a rare syndrome. Such a presentation is difficult to diagnose clinically, and benefits from histopathological evaluation where the presence of intralymphatic noncaseating granulomas serves as a diagnostic clue. ...
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To report the clinicopathologic features of a series of patients with Melkersson-Rosenthal syndrome (MRS). Patients in this clinicopathological case series were identified through retrospective review. Five Caucasian patients (2 women and 3 men; age range, 46 to 73 years) with isolated eyelid swelling presented over an 11-month period to two Canadian tertiary Oculoplastic referral centres. All underwent clinical examination and had tissue biopsies. The patients presented with non-pitting, non-pruritic, painless and recurrent eyelid swelling. None had evidence of cranial nerve palsy or fissured tongue. The duration of symptoms before referral was between 1-5 years. Histopathological examination showed granulomatous inflammation in four patients. Granulomatous folliculitis was a novel finding in three patients. Monosymptomatic patients with MRS require a high index of suspicion. Histopathological confirmation is vital for the diagnosis.