A Mysterious

Rash

Zokir Takhirov 190169

 Clinical Case Presentation – The
Patient
• Case:
• A 35-year-old African American
woman presents with a 3-month
history of multiple skin
lesions on her face and arms.
• Mild cough and occasional
shortness of breath.
• No fever, weight loss, or systemic
symptoms.
• No history of similar lesions or
trauma.
Initial Dermatologic
Examination

• Multiple violaceous, indurated

plaques on the nose and cheeks.
• Several brownish-red papules on
the forearms.
• No pustules, vesicles, or ulcerations.
Differential Diagnosis of Papulonodular
Lesions

Granuloma annulare

Lupus
erythematosus

Sarcoidosis

Leprosy Cutaneous tuberculosis

Condition Clinical Clues Histology Other Tests

Red-brown papules/plaques, often on Non-caseating ↑ ACE, hypercalcemia,

Sarcoidosis
face or scars; lupus pernio (nose) granulomas CXR: hilar adenopathy

Lupus Basal vacuolar

Malar rash, photosensitivity, discoid ANA (+), anti-dsDNA
erythematos degeneration, perivascular
plaques (+)
us lymphocytes

Granuloma Annular, flesh-colored plaques, often Degenerated collagen, Self-limited, no

annulare on extremities palisading histiocytes systemic markers

Chronic ulcers, verrucous plaques; Caseating granulomas TB skin test (+), PCR,
Cutaneous TB
endemic areas with acid-fast bacilli culture

Hypopigmented anesthetic patches Granulomas with acid- Skin slit smear,

Leprosy
(tuberculoid), nodules (lepromatous) fast bacilli Lepromin test
Punch Excision
al

Skin Biopsy
A biopsy of the papular lesion was performed.
Biopsy results
•Histopathology: Non-caseating
granulomas in the dermis without
central necrosis.
•Negative for fungal or mycobacterial
organisms.
Systemic • Pulmonary function tests: Mild
restrictive pattern.

Evaluation
Lab tests
•↑ Serum ACE – Due to granuloma-associated
macrophages.
•Hypercalcemia & Hypercalciuria – Macrophages in
granulomas produce 1α-hydroxylase, increasing
vitamin D activation.
•↑ Alkaline phosphatase (ALP) – If liver involvement is
present.
•↓ Lymphocyte count (Lymphopenia) – Due to
sequestration in granulomas.
•↑ CD4/CD8 ratio in bronchoalveolar lavage (BAL)
(>3.5)
Sarcoidosis!
Sarcoidosis
Definition: Chronic granulomatous disease
of unknown etiology, characterized by non-
caseating granulomas in multiple organs.

Pathophysiology:
• Immune dysregulation: Exaggerated Th1
response → ↑ IFN-γ & TNF-α → macrophage
activation → granuloma formation.
• Non-caseating granulomas: Composed of
epithelioid macrophages, multinucleated
giant cells, and lymphocytes.
 Sarcoidosis Subtypes in
Dermatology

Plaque
sarcoidosis

Papular sarcoidosis – Most Lupus pernio – Chronic, disfiguring

common; brown-red papules on plaques on the nose, cheeks, and ears.
the face and trunk.

Erythema nodosum –
Tender subcutaneous
nodules on the legs
(often self-limited). •Scar sarcoidosis – Old scars
Subcutaneous Nodular
sarcoidosis become raised and inflamed. sarcoidosis
Treatment Options
•First-line:
Topical/intralesional
steroids.
•Severe cases: Systemic
corticosteroids (prednisone).
•Steroid-sparing agents:
Methotrexate,
hydroxychloroquine.
•Refractory cases: TNF-
alpha inhibitors (infliximab).
  Cutaneous sarcoidosis may resolve
spontaneously or persist.

Prognosis  Lupus pernio = worse prognosis

(associated with chronic pulmonary disease).
 Spontaneous resolution: ~60% of cases,
especially with isolated hilar adenopathy.
 Chronic disease: ~30%, with progressive
fibrosis in lungs or other organs.
 Severe complications: ~10%, including
pulmonary hypertension, cardiac involvement,
or neurosarcoidosis.
 Mortality: ~1-5%, mainly from respiratory
failure, cardiac arrhythmias, or CNS disease.
 Skin findings can be the
first clue to systemic
Final sarcoidosis.
Always biopsy suspicious
Takeawa lesions to confirm
ys & granulomas.
Rule out TB and
Discussio infections before
n diagnosing sarcoidosis.
Multidisciplinary approach:
Dermatology +
Pulmonology.
Definition: Chronic granulomatous disease of unknown etiology, Cardiac Manifestations (5%)
characterized by non-caseating granulomas in multiple organs.
• Conduction abnormalities: AV block is most common.

Pathophysiology: • Restrictive or dilated cardiomyopathy.

• Immune dysregulation: Exaggerated Th1 response → ↑ IFN-γ &

TNF-α → macrophage activation → granuloma formation. Neurologic Manifestations (5-10%)

• Non-caseating granulomas: Composed of epithelioid • Facial nerve palsy (Bell’s palsy).

macrophages, multinucleated giant cells, and lymphocytes. • Meningitis, seizures, or peripheral neuropathy.

Pulmonary Manifestations (Most Common, ~90%) Other Systemic Features

• Bilateral hilar lymphadenopathy (BHL): Classic CXR finding. • Löfgren syndrome: BHL, erythema nodosum, fever,
• Interstitial lung disease: Reticular opacities, fibrosis (restrictive polyarthritis (good prognosis).
pattern). • Heerfordt syndrome: Uveitis, parotitis, fever, facial nerve
• Hypercalcemia: Due to increased 1α-hydroxylase in palsy.
granulomas → ↑ 1,25-(OH)₂D.
Diagnosis:

Dermatologic Manifestations (30%) • CXR: BHL, interstitial lung disease.

• Lupus pernio: Violaceous plaques on nose, cheeks, ears • Serum ACE ↑ (nonspecific).
(specific for sarcoidosis). • Hypercalcemia (↑ 1,25-vitamin D).
• Erythema nodosum: Tender red nodules on shins (good • Biopsy: Non-caseating granulomas (gold standard).
prognosis, often self-limiting).
• Maculopapular lesions: Common but non-specific. Treatment:
• Asymptomatic or mild cases: Observation.
Ophthalmologic Manifestations (25%)
• Symptomatic (lungs, eyes, heart, neuro): Corticosteroids
• Uveitis: Blurred vision, photophobia, conjunctival granulomas. are first-line.
• Sicca syndrome: Lacrimal gland involvement → dry eyes. • Refractory cases: Methotrexate, TNF-α inhibitors
 Summary
A: Waxy interscapular skin plaques – cutaneous
sarcoidosis.
B: Lupus pernio – violaceous facial plaques, a chronic
sarcoidosis feature.
C: Anterior uveitis with synechiae – ocular
involvement, common in sarcoidosis.
D: Enlarged, nodular lacrimal gland – sarcoid-related
lacrimal gland involvement.
E: Endobronchial cobblestoning – bronchial sarcoidosis.
F: Peripheral facial-nerve palsy with hearing loss –
Heerfordt syndrome.
G: Spinal cord mass – neurosarcoidosis.
H: Multi-organ involvement on gallium scan –
sarcoidosis distribution.
I: Hypermetabolic liver, spleen, and lymph nodes – PET
scan of sarcoidosis.
J: Aspergilloma in lung cavity – sarcoid-associated
fungal colonization.
K: Hypodense splenic nodules – splenic sarcoidosis.
L: Optic chiasm involvement – neurosarcoidosis with
visual risk.
M: Granulomatous humeral lesion – bone sarcoidosis.
Summary of sarcoidosis
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