More on Hidradenitis Suppurativa and Inflammatory Bowel Disease

In a population-based inception cohort study (S Yada et al. Clin Gastroenterol Hepatol 2016; 14: 65-70) of 679 patients with inflammatory bowel disease (IBD) followed for a median of more than 19.8 years, it was determined that patients with IBD were ~9 times more likely to develop hidradenitis suppurativa (HS) compared with general population. 8 of 679 patients developed HS; only one had HS prior to IBD.

Other findings:

  • Most Crohn’s disease patients with HS had perianal disease.  Most ulcerative colitis patients developed HS after colectomy.
  • Female sex and obesity were risk factors for HS.

In a second retrospective study (N Kamal et al. Clin Gastroenterol Hepatol 2016; 14: 71-9), the authors identified 15 patients with CD and HS.  10 patients had perianal disease.  In this population, “both diseases were characterized by their severity, requirement of systemic medical therapies including anti-TNF and high operative rate.” this article contained some very helpful pictures.

Unrelated article: F Wang, JL Kaplan, BD Gold et al. Cell Reports; 2016: 14: 945-55.  This highly technical study used two independent cohorts of patients with Crohn’s disease and non-IBD controls.  One cohort, RISK, had over 700 patients and ~30,000 mean number of reads per sample; the other cohort, PIBD-CC, and 87 patients and ~3000 mean number of reads per sample.  Overall, the study showed associations between Crohn’s disease and bacteria in the lumen and the study helps provide an information-based method to depict dysbiosis.

Related blog post: Add it to the list

San Juan

San Juan

Add it to the list

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.)