A 44 year old woman with progressive pneumonitis and nodules on the elbows and tops of hands


By Alex Lin, MS IV, Johns Hopkins School of Medicine

 

Introduction: 
 A 44 year old woman was evaluated by the inpatient dermatology consultation service for tender nodules on the hands and elbows of 1 month duration.
   
History: 
 One month prior to admission she developed a progressive cough, shortness of breath and hemoptysis which did not improve with 3 weeks of oral levofloxacin given for presumed pneumonia. Several weeks before admission she developed dark spots on her elbows and the back of her hands. Two years earlier she had her mitral valve replaced because of severe mitral stenosis. Shortly afterward she had a Nissen fundoplication for treatment of gastroesophageal reflux disease. Chronic cough was noted atleast 1 year before the current admission.
 
Clinical Presentation: 
 On examination she had violaceous tender nodules with central crusts on the extensor surfaces of the elbows and over the metacarpal and proximal interphalyngeal joints. Her mucous membranes and lacrimal glands were normal. She had no purpura, periungual telangiectasias, organomegaly, or lymphadenopathy.
 
Pathology: 
 Transtrachial lung biopsy showed alveolar macrophages and mixed inflammatory cells with numerous eosinophils. Special stains were negative for acid fast bacteria, Pneumocystis carinii, Cytomegalovirus, virus, bacteria, and fungi. Skin biopsy demonstrated an ill defined area of palisading granulomatous dermatitis with central degeneration of collagen, prominent leukocytoclasis and rare eosinophils.
         
 
Violaceous nodules with central necrotic crust
Violaceous nodules with central necrotic crust

Violaceous nodules with central necrotic crust
Violaceous nodules with central necrotic crust
Transtracheal biopsy with alveolar macrophages and mixed inflammatory cells with numberous eosinophils
Transtracheal biopsy with alveolar macrophages and mixed inflammatory cells with numberous eosinophils
 
         
 
Skin biopsy (X40) with ill defined areas of palisading granulomatous dermatitis with central degeneration of collagen, prominent leukocytoclasis, and rare eosinophils
Skin biopsy (X40) with ill defined areas of palisading granulomatous dermatitis with central degeneration of collagen, prominent leukocytoclasis, and rare eosinophils
Skin biopsy (X100)
Skin biopsy (X100)
 
   
Question 1: 
 What is the differential diagnosis?
 
Question 2: 
 What further evaluation will this patient require?
 
Question 3: 
How would you treat this patient?
 
Question 4: 
What is her prognosis?
 

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