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.
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