Not Crying Wolf

Alexis Eastman
PGY‐3
Internal Medicine
(No financial relationships to disclose, just student loans)

The Mysterious Lump
• 56 year‐old female with SLE, DM2 with
proteinuria, PHTN, on hydroxychloroquine
and prednisone
• Two weeks with new 1.5 cm right forearm
mass
• No constitutional symptoms, no other skin
changes

Exam
• Firm, freely mobile 1.5 cm mass on right
volar forearm
• Mildly tender to palpation, no overlying
skin changes, no fluctuance or drainage
• Remainder of exam unremarkable

One week later
• Returned to clinic with a 2.5 cm mass, now
with mild overlying erythema
• WBC 4.6 k/uL, Hb 10.5 g/dL, Plts 337k/uL
Thomas Ray, Univ of IA Dept of Dermatology, Hardin MD, Univ of IA
http://hardinmd.lib.uiowa.edu/ui/tray/LE‐profund‐005.html

Ultrasound
• Subcutaneous edema and fat lobules with
increased flow, concern for infection,
malignancy, early post‐traumatic ossification

And so it grows…
• Over the next two weeks, increased to 4 cm
• Increased tenderness, moderate erythema
• Patient endorsed fevers (unmeasured) and
fatigue but no night sweats or chills

Differential
• Infectious panniculitis
• Insect‐bite panniculitis
• Abscess
• Malignancy, particularly cutaneous lymphoma
• Lipoma
• Neurofibroma
• Trauma with ossification or keloid
• Cutaneous lupus

Nervousness ensues
• MRI obtained: No Abscess (whew!)
• Biopsy: cultures negative

Tissue is the issue….
• Histopathology: dense lymphoplasmacytic
infiltrate of the dermis extending into the
subcutis with rare vessels showing fibrinoid
necrosis. Lymphoid infiltrate composed of
small lymphocytes and immunoblasts, with
morphologically unremarkable plasma cells.
• Most consistent with

Histology
microcalcification
Follicular plugging
peripheral nerve with
lymphocytic infiltration
Basement membrane
hyalinization
Lymphocytic vasculitis
Lobular lymphocytic infiltrate
with septal karyorrhexis
(Thanks, Dad)

Lupus Erythematosus Profundus
• Cutaneous lupus without systemic disease: 3/100,000
• Similar to prevalence of SLE
• Majority is Chronic cutaneous lupus (CCLE)
• Lupus erythematosus profundus is a subtype
• Usually independent of SLE (10‐42% of cases)
• Only 1‐5% of patients with pre‐existing SLE will develop LEP
• 2‐9:1 female:male ratio, median age of onset in the
mid‐40s

Presentation
• Nodules
• Tender, subcutaneous
• Asymmetric on face, proximal upper extremities, trunk
• Very rarely on lower extremities
• In African‐Americans or Asians may be periorbital or salivary
• Overlying erythema is common
• Concomitant discoid lupus
• Relapsing‐remitting course
• Lesions resolve with significant lipoatrophy
and permanent skin depressions

Diagnosis
• Labs are of little help
• Usually ANA and anti‐dsDNA negative
• Occasional lymphopenia, anemia, low C4, positive RF, positive anti‐cardiolipin
• Biopsy is the gold standard, recently proposed criteria include:
• Major:
• Hyaline fat necrosis
• Lymphocytic aggregates
• Lymphoid follicle formation
• Periseptal or lobular lymphocytic
panniculitis
• Microcalcifications
• Minor:
• Discoid lupus in overlying skin
• Lymphocytic vasculitis
• Subepidermal hyalinization
• Mucin deposition
• Histiocytes and small granulomas
• Plasma cell or eosinophil
infiltrates
• Lymphoid follicles are particularly useful as they are not seen in
lymphoma. Subcutaneous T‐cell lymphomas (STCL) can have significant
overlap with LEP.

Treatment
• Mainstay: anti‐malarials, primarily
hydroxychloroquine
• Approximately 60% of patients respond to treatment
• Methotrexate can be added to refractory cases
• Intralesional steroids not recommended
• can increase the lipoatrophy upon healing

Prognosis
• Nodules regress with atrophic changes
• Can cause significant facial disfigurement
• Frequently recurs:
• 2010 retrospective case analysis, 76% of the patient
experienced recurrence within 14 months after
treatment finished
• Progresses to systemic disease in 7‐50% of patients

Take Home Points
• It can always be Lupus!
• Always rule out malignancy and infection prior to treatment
• One of the leading causes of death in SLE
• Watch out for STCL and its overlap on pathology
• Biopsy is the only way to accurately diagnose cutaneous lupus
• Treatment consists of anti‐malarials, such as hydroxychloroquine, and/or
methotrexate
• Intralesional steroids should be avoided as they can increase lipoatrophy
in the healing process
• Patients without SLE need close follow‐up, as they are at increased risk
of developing SLE

Further Reading
• Fraga J and García‐Díez A. “Lupus erythematosus panniculitis,”
Dermatology Clinics 2008; 26:453‐63.
• Park HS, Choi JW, Kim BK, and Cho KH. “Lupus erythematosus
panniculitis: clinopathological, immunophenotypic, and molecular
studies,” American Journal of Dermatopathology 2010; 32(1):24‐30.
• Obermoser G, Sontheimer RD, and Zelger B. “Overview of common,
rare, and atypical manifestations of cutaneous lupus erythematosus
and histopathological correlates,” Lupus 2010; 19:1050‐70.

Thanks!