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Systemic Mastocytosis-associated Leonine

Facies and Eyebrow Loss
Hubert M. Chodkiewicz, BA, Philip R. Cohen, MD
South Med J. 2011;104(3):236-238.

Abstract and Introduction

Abstract

Leonine facies or loss of eyebrows, or both, occurring concurrently or in succession, can be associated with
numerous etiologies. A 62-year-old woman with systemic mastocytosis who developed both leonine facies and
eyebrow loss is described. The differential diagnosis of neoplasms that may present with leonine facies is
summarized and conditions characterized by concurrent or sequential eyebrow loss and leonine facies are reviewed.
Introduction

Leonine facies can be associated with various conditions, including neoplasms.[1] Eyebrow loss can occur as an
isolated finding or in association with leonine facies. We describe a woman who developed leonine facies and
eyebrow loss secondary to systemic mastocytosis, and summarize neoplastic processes with leonine facies, as well
as conditions with eyebrow loss and leonine facies.

Case Report
A 62-year-old woman presented with pruritus, shortness of breath, wheezing, and skin lesions on her face extending
to her chest, back and abdominal area. A lesional skin biopsy showed an infiltrate of mast cells and a systemic work
up was performed. Bone marrow biopsy showed multifocal dense infiltrates of small to intermediate mast cells
comprising approximately 45% of the total bone marrow cellularity. Her serum tryptase was elevated at 235.0 ng/mL
(normal, <11.5 ng/mL). These findings fulfilled the criteria for systemic mastocytosis. Incidentally, the bone marrow
biopsy also discovered IgG kappa multiple myeloma.
The patient was treated with oral imatinib mesylate 400 mg daily for mastocytosis, and her pruritus and skinlesions
resolved. For her multiple myeloma, she received oral revlimid 25 mg daily and oral dexamethasone 20 mg for two
cycles, followed by intravenous velcade 2.6 mg and oral dexamethasone 20 mg for four cycles. She discontinued
taking imatinib mesylate after eight months, due to nausea and decreased taste sensation.
Subsequently, one month after discontinuation of imatinib mesylate, she presented with asymptomatic papules on her
medial upper eyelids, proximal bridge of nose and glabella, giving her a leonine facies appearance (Fig. 1). A biopsy
of one of the papules showed the dermis to be infiltrated with mast cells related to her systemic mastocytosis.

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Figure 1.

A woman with systemic mastocytosis whose face shows papules on her eyelids, nose, and glabella, giving a leonine
facies appearance.
At follow up three months later, the patient had severe pruritus, a more prominent leonine facies, and bilateral
eyebrow loss (Fig. 2). Symptomatic treatment for pruritus included daily fexofenadine 180 mg and topical
betamethasone dipropionate 0.05% ointment. She was also restarted on oral imatinib mesylate 400 mg daily, and
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prochlorperazine 10 mg daily to prevent nausea. After two months of treatment, the pruritus resolved, the facial
papules decreased in size, and her eyebrows sparsely reappeared.

Figure 2.

Three months after diagnosis, the leonine facies is more pronounced and the patients eyebrows are almost
completely absent.

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Discussion
Systemic mastocytosis is characterized by neoplastic mast cell infiltration of extracutaneous organs. Dermal mast cell
infiltrates can result in diffuse erythrodermic mastocytosis, solitary mastocytomas, telangiectasia macularis eruptiva
perstans, and urticaria pigmentosa.[2,3] To this list, we have added leonine facies.
Leonine facies is defined by thickening, furrowing, and coarsening of the facial skin, producing a "lion-like"
resemblance. Facial features include a prominent supraorbital ridge, a thick glabellar region, and deep furrows on the
malar and infraorbital regions. Diffuse dermal infiltration with papules fusing together into plaques on the face creates
the manifestation of leonine facies.[1] Leprosy and cutaneous T-cell lymphoma are more common causes for leonine
facies; however, other malignancies including systemic mastocytosisalbeit rarelycan also result in the
development of leonine facies ().[1,419]
Table 1. Malignancy-associated leonine facies a

Hematopoietic dyscrasias
Leukemia cutis
Acute lymphocytic leukemia4
Acute myelomonocytic leukemia57
Chronic lymphocytic leukemia1,79
Lymphoma
Cutaneous B-cell lymphoma10
Cutaneous T-cell lymphoma1114
Hodgkin's disease4
Plasmacytoma15
Systemic mastocytosis16.current report
Solid tumors
Metastatic breast carcinoma17
aLeonine facies resulted from infiltration of the skin by neoplastic cells; however, leonine facies has also been
described in patients whose tumor has elicited sebaceous hyperplasia (secondary to bronchial carcinoid)18 or

subcutaneous eosinophilic necrosis (associated with myelodysplastic syndrome).19

Eyebrow loss can be observed in patients with autoimmune conditions, endocrinopathies, genetic diseases,
infections, neoplasms, primary dermatoses, and trauma.[20] Partial or complete loss of eyebrows can also coexist with
leonine facies ().[4,6,1214,16,2125] Leprosy is a prevalent mycobacterial infection; hence, it is not unexpected that the
lepromatous variant is the most frequent cause of concurrent or sequential eyebrow loss and leonine facies.[20]
Table 2. Conditions with eyebrow loss and leonine facies a

Granulomatous conditions
Sarcoidosis21
Infectious
Leprosy22
Inherited syndrome
Setleis syndrome23
Neoplastic
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Cutaneous T-cell lymphoma

Mycosis fungoides13,14
Unspecified12
Leukemia cutis
Acute lymphocytic leukemia4
Acute myelomonocytic leukemia6
Systemic mastocytosis16.current report
Other conditions
Alopecia mucinosa24
Viral-associated trichodysplasia25
aEyebrow loss occurred concurrently or sequentially with the development of leonine facies.

Our patient had multiple myeloma and systemic mastocytosis. Biopsy of a glabella papule confirmed that her leonine
face and eyebrow loss was secondary to mast cell infiltration of the skin. We have identified only one other patient
with a similar presentationa 7-year-old boy with systemic mastocytosis having an increase of the facial skin lines
and the facial appearance of an aged man, as well as loss of eyebrows. Inspection of the clinical figure included in the
published article reveals that the child's face was leonine.[16]

Conclusion
Malignancy-associated leonine facies can occur either as a result of infiltration of the neoplastic cells,[1,417] or
secondary to changes in the dermis or subcutaneous fat elicited by the primary cancer.[18,19] Indeed, leonine facies
may be a distinctive pattern of leukemia cutis in patients with either chronic lymphocytic leukemia,[1,79] acute
myelomonocytic leukemia,[57] or acute lymphocytic leukemia.[4] Cutaneous T-cell lymphoma[1214]and less
commonly leukemia cutis[4,6] and systemic mastocytosis[16]can be characterized not only by leonine facies but also
eyebrow loss.

Sidebar
Key Points

Leonine facies is defined by thickening, furrowing, and coarsening of the skin on the face, producing a "lionlike" resemblance.
Eyebrow loss can be observed in patients with autoimmune conditions, endocrinopathies, genetic diseases,
infections, neoplasms, primary dermatoses, and trauma.
Leprosy and cutaneous T-cell lymphoma are more common causes of leonine facies.
Systemic mastocytosis is characterized by neoplastic mast cell infiltration of extracutaneous organs.
Cutaneous T-cell lymphoma, leukemia cutis, and systemic mastocytosis can be characterized by leonine
facies and eyebrow loss.
References

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The authors did not receive any funding or grants, and they have no commercial, proprietary, or financial interests to
disclose.
South Med J. 2011;104(3):236-238. 2011 Lippincott Williams & Wilkins

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