A recent review article (NEJM 2012; 366: 158-64) describes a common, chronic recurrent granulomatous inflammatory disease that manifests as painful, deep-seated inflamed lesions (including sinus tracts and abscesses); it typically starts after puberty. This disease is due in part to aberrant healing processes and impaired mechanical integrity. Investigations have shown that tumor necrosis factor α is involved in the pathogenesis of hidradenitis suppurativa. There are a lot of similarities between hidradenitis suppurativa and Crohn’s disease as well as some overlap as up to 17% of individuals with Crohn’s disease have hidradenitis suppurativa. In addition, hidradenitis suppurativa can be confused with Crohn’s disease especially when buttocks/perianal lesions are present.
Key clinical points with this disease:
- Affects skin with apocrine glands
- Long delay in diagnosis is common though disorder may affect 1% of population
- Incision and drainage is not effective treatment –lesions recur
- Few randomized studies –most often treated with combination of antibiotics (eg. clindamycin & rifampin)
- More severe disease may benefit from TNF inhibitors or possibly complete surgical excision
Histoplasma , Amebiasis, CMV, C. diff, Klebsiella oxytoca, Actinomycosis, HIV
Sarcoidosis, Vasculitis (HSP,dermatomyositis, Wegener’s, SLE), FMF, allergic colitis, Hirschsprung’s, autoimmune enteropathy, Chronic granulomatous disease, glycogen storage dz, 1B, Hermansky-Pudlak syndrome, Wiskott Aldrich syndrome, NEMO (NF-kB essential modifier) deficiency, Leukocyte adhesion deficiency, SCID, Behcet’s, FELS/hemophagocytic lymphohistiocytosis, Typhlitis
Additional reference for hidradenitis suppurativa:
- -Br J Dermatol 2010; 162: 195-7.
Crohn’s Disease and differential diagnosis -references:
- -JPGN 2010; 51: 690. Discusses sarcoid (check angiotensin converting enzyme), CGD, Hermansky Pudlak
- -JPGN 2010; 50: 99. Perianal dz in young children may be due to autoimmune neutropenia.
- -IBD 2008; 14: 1443. Phagocyte dysfunction. Also, discusses Hermansky-Pudlak, GSD 1B, chronic granulomatous disease, Chediak-Higashi, leukocyte adhesion deficiency, cylic neutropenia, congenital neutropenia
- -JPGN 2006; 42: 405. Vasculitis mimicking IBD.
- -NEJM 2005; 352: 489-94.
- -J Clin Gastroenterol 1992; 15: 17-23.
- -NEJM 2003; 349 (26): 2541-49. Table 2. (Gold et al.)