Not Crying Wolf
Not Crying Wolf
Not Crying Wolf
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Alexis Eastman<br />
PGY‐3<br />
Internal Medicine<br />
(No financial relationships to disclose, just student loans)
The Mysterious Lump<br />
• 56 year‐old female with SLE, DM2 with<br />
proteinuria, PHTN, on hydroxychloroquine<br />
and prednisone<br />
• Two weeks with new 1.5 cm right forearm<br />
mass<br />
• No constitutional symptoms, no other skin<br />
changes
Exam<br />
• Firm, freely mobile 1.5 cm mass on right<br />
volar forearm<br />
• Mildly tender to palpation, no overlying<br />
skin changes, no fluctuance or drainage<br />
• Remainder of exam unremarkable
One week later<br />
• Returned to clinic with a 2.5 cm mass, now<br />
with mild overlying erythema<br />
• WBC 4.6 k/uL, Hb 10.5 g/dL, Plts 337k/uL<br />
Thomas Ray, Univ of IA Dept of Dermatology, Hardin MD, Univ of IA<br />
http://hardinmd.lib.uiowa.edu/ui/tray/LE‐profund‐005.html
Ultrasound<br />
• Subcutaneous edema and fat lobules with<br />
increased flow, concern for infection,<br />
malignancy, early post‐traumatic ossification
And so it grows…<br />
• Over the next two weeks, increased to 4 cm<br />
• Increased tenderness, moderate erythema<br />
• Patient endorsed fevers (unmeasured) and<br />
fatigue but no night sweats or chills
Differential<br />
• Infectious panniculitis<br />
• Insect‐bite panniculitis<br />
• Abscess<br />
• Malignancy, particularly cutaneous lymphoma<br />
• Lipoma<br />
• Neurofibroma<br />
• Trauma with ossification or keloid<br />
• Cutaneous lupus
Nervousness ensues<br />
• MRI obtained: No Abscess (whew!)<br />
• Biopsy: cultures negative
Tissue is the issue….<br />
• Histopathology: dense lymphoplasmacytic<br />
infiltrate of the dermis extending into the<br />
subcutis with rare vessels showing fibrinoid<br />
necrosis. Lymphoid infiltrate composed of<br />
small lymphocytes and immunoblasts, with<br />
morphologically unremarkable plasma cells.<br />
• Most consistent with
Histology<br />
microcalcification<br />
Follicular plugging<br />
peripheral nerve with<br />
lymphocytic infiltration<br />
Basement membrane<br />
hyalinization<br />
Lymphocytic vasculitis<br />
Lobular lymphocytic infiltrate<br />
with septal karyorrhexis<br />
(Thanks, Dad)
Lupus Erythematosus Profundus<br />
• Cutaneous lupus without systemic disease: 3/100,000<br />
• Similar to prevalence of SLE<br />
• Majority is Chronic cutaneous lupus (CCLE)<br />
• Lupus erythematosus profundus is a subtype<br />
• Usually independent of SLE (10‐42% of cases)<br />
• Only 1‐5% of patients with pre‐existing SLE will develop LEP<br />
• 2‐9:1 female:male ratio, median age of onset in the<br />
mid‐40s
Presentation<br />
• Nodules<br />
• Tender, subcutaneous<br />
• Asymmetric on face, proximal upper extremities, trunk<br />
• Very rarely on lower extremities<br />
• In African‐Americans or Asians may be periorbital or salivary<br />
• Overlying erythema is common<br />
• Concomitant discoid lupus<br />
• Relapsing‐remitting course<br />
• Lesions resolve with significant lipoatrophy<br />
and permanent skin depressions
Diagnosis<br />
• Labs are of little help<br />
• Usually ANA and anti‐dsDNA negative<br />
• Occasional lymphopenia, anemia, low C4, positive RF, positive anti‐cardiolipin<br />
• Biopsy is the gold standard, recently proposed criteria include:<br />
• Major:<br />
• Hyaline fat necrosis<br />
• Lymphocytic aggregates<br />
• Lymphoid follicle formation<br />
• Periseptal or lobular lymphocytic<br />
panniculitis<br />
• Microcalcifications<br />
• Minor:<br />
• Discoid lupus in overlying skin<br />
• Lymphocytic vasculitis<br />
• Subepidermal hyalinization<br />
• Mucin deposition<br />
• Histiocytes and small granulomas<br />
• Plasma cell or eosinophil<br />
infiltrates<br />
• Lymphoid follicles are particularly useful as they are not seen in<br />
lymphoma. Subcutaneous T‐cell lymphomas (STCL) can have significant<br />
overlap with LEP.
Treatment<br />
• Mainstay: anti‐malarials, primarily<br />
hydroxychloroquine<br />
• Approximately 60% of patients respond to treatment<br />
• Methotrexate can be added to refractory cases<br />
• Intralesional steroids not recommended<br />
• can increase the lipoatrophy upon healing
Prognosis<br />
• Nodules regress with atrophic changes<br />
• Can cause significant facial disfigurement<br />
• Frequently recurs:<br />
• 2010 retrospective case analysis, 76% of the patient<br />
experienced recurrence within 14 months after<br />
treatment finished<br />
• Progresses to systemic disease in 7‐50% of patients
Take Home Points<br />
• It can always be Lupus!<br />
• Always rule out malignancy and infection prior to treatment<br />
• One of the leading causes of death in SLE<br />
• Watch out for STCL and its overlap on pathology<br />
• Biopsy is the only way to accurately diagnose cutaneous lupus<br />
• Treatment consists of anti‐malarials, such as hydroxychloroquine, and/or<br />
methotrexate<br />
• Intralesional steroids should be avoided as they can increase lipoatrophy<br />
in the healing process<br />
• Patients without SLE need close follow‐up, as they are at increased risk<br />
of developing SLE
Further Reading<br />
• Fraga J and García‐Díez A. “Lupus erythematosus panniculitis,”<br />
Dermatology Clinics 2008; 26:453‐63.<br />
• Park HS, Choi JW, Kim BK, and Cho KH. “Lupus erythematosus<br />
panniculitis: clinopathological, immunophenotypic, and molecular<br />
studies,” American Journal of Dermatopathology 2010; 32(1):24‐30.<br />
• Obermoser G, Sontheimer RD, and Zelger B. “Overview of common,<br />
rare, and atypical manifestations of cutaneous lupus erythematosus<br />
and histopathological correlates,” Lupus 2010; 19:1050‐70.
Thanks!