Presented by: Jaryd Freedman, MD and Carrie Ann Cusack, MD
Drexel University College of Medicine
May 16, 2016
Non-infectious granulomatous dermatitis (NIGD) is a diverse group of disorders with both clinical and pathological overlap. These entities include, but are not limited to, granuloma annulare, sarcoidosis, interstitial granulomatous dermatitis (IGD), palisaded neutrophilic and granulomatous dermatitis (PNGD), necrobiosis lipoidica, rheumatoid nodules, granulomatosis with polyangiitis, annular elastolytic giant cell granuloma, and drug reactions. While some patients will often fall nicely into one of these categories, there are many patients who have features of many NIGD types but are not easily classified.
One commonality amongst all the NIGD is that their precise etiologies are unknown. This group of disorders has been linked to many disease states especially autoimmune conditions. There are no reported cases in the literature of concomitant isolated NIGD and IPAH, however both of these conditions are frequently linked to connective tissue disease. PNGD, IGD, and to a lesser extent sarcoidosis, have been linked to autoimmune connective tissue disease. Likewise, IPAH has been associated with connective tissue disease, most frequently scleroderma. While sarcoidosis has been linked to pulmonary arterial hypertension, this is in the setting of lung granulomatous disease and fibrosis, therefore representing a distinct condition from IPAH.
Immune dysregulation has been hypothesized to be the etiological factor for both NIGD and IPAH. Many of the NIGD can benefit from immunomodulation therapy with antimalarials, tetracyclines, methotrexate and other immunosuppressants. IPAH patient life expectancies have been increased considerably through treatment with calcium channel blockers, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostanoid analogues.
Peroni A, Colato C, Schena D, Gisondi P, Girolomoni G. Interstitial granulomatous dermatitis: a distinct entity with characteristic histological and clinical pattern. Br J Dermatol 2012; 166:775–783.
Rosenbach M, English JC. Reactive Granulomatous Dermatitis: A Review of Palisaded Neutrophilic and Granulomatous Dermatitis, Interstitial Granulomatous Dermatitis, Interstitial Granulomatous Drug Reaction, and a Proposed Reclassification. Dermatologic clinics. 2015 Jul 31;33(3):373-87.
Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, Sanchez MA, Kumar RK, Landzberg M, Machado RF, Olschewski H. Updated clinical classification of pulmonary hypertension. Journal of the American College of Cardiology. 2013 Dec 24;62(25):D34-41.
McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension: A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association Developed in Collaboration With the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53(17):1573-1619.