Add it to the list

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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
For pediatric gastroenterologists, hidradenitis suppurative is another to add to the Crohn’s differential diagnosis list:
 

Infectious etiologies: 

Histoplasma , Amebiasis, CMV, C. diff, Klebsiella oxytoca, Actinomycosis, HIV

Noninfectious etiologies:

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.)