Diseases associated with hidranitis suppurativa: part 2 of a series on hidradenitis

Abstract

Hidradenitis suppurativa (HS), a pathologic follicular disease, impacts patients' lives profoundly and usually occurs in isolation. The diseases with the strongest association are obesity, depression, and pain. HS is associated with many diseases including acne conglobata (AC), dissecting cellulitis, pilonidal cysts, and obesity. Pyoderma fistulans sinifica (fox den disease) appears to be the same entity as Hurley Stage 2 of 3 HS. The rate of acne vulgaris in HS patients mirrors unaffected controls. The most common, albeit still uncommon, association is with seronegative, haplotype unlinked arthritis (most importantly B27), in particular spondolyarthritis. Crohn disease and HS occur together at a rate that varies from 0.6% to 38% in retrospective cases series. Ulcerative colitis occurred with HS in 14% of patients in one series. The next most common association is with pyoderma gangrenosum, but this association is likely under-reported. Synovitis-Acne-Pustulosis Hyperostosis-Osteitis (SAPHO) syndrome, which is rare, has more than 10 reports linking it to HS. Nine case reports have linked Dowling-Degos disease (DDD) to HS and two reports related HS to Fox-Fordyce disease (FF), but because both occur in the axilla this might be a mere coincidence. HS is rarely associated with ophthalmic pathology. Specifically, more than 5 reports link it to Keratitis-Ichthyosis-Deafness syndrome (KID); greater than10 cases link it to interstitial keratitis and 2 cases are linked to Behçet's disease. The presence of proteinuria and acute nephritis link HS to the kidney, especially since and reports have documented resolution of HS after renal transplant. Florid steatocystoma multiplex, Sjogren Syndrome, and HS have been linked and their reports likely underestimate their coincidence because all these entities involve occlusion (albeit by different mechanisms). Three reports link HS and amyloid, but both share some common genetic underpinnings and thus the coincidence of these diseases is likely underreported. Pyoderma vegetans has been noted in 2 cases of HS and 4 cases of Inflammatory Bowel Disease (IBD) and is likely a clue to the linkage of the pathology of IBD and HS. Pityriasis rubra pilaris, in particular Type VI related to HIV, has a relationship more commonly with acne conglobata, but with HS also. Single case reports of diseases associated with HS include systemic lupus erythematosus, acromegaly, Down syndrome, Bazex-Dupre´-Christol, and prurtis ani, but these might be coincidences. Pyogenic Arthritis, Pyoderma gangrenosum, and Acne (PAPA Syndrome) and Pyoderma gangrenosum, Acne, and Suppurative Hidradenitis (PASH Syndrome) are pyodermic-arthritic syndromes that are associated with HS. Erythema nodosum and granulomatous lobular mastitis have been reported with HS but the significance of these reports is uncertain. Because of scarring, HS can result in lymphedema including scrotal elephantiasis and verrucous lymphedema. HS is sometimes accompanied by obesity, hypertension, and anemia and can be considered a disease in the s...

Hidradenitis suppurativa and relapsing polychondritis: An uncommon association

“…Musculoskeletal association with HS has been reported 2 . It was shown that there is an association with skin flares and that it tends to occur years after the onset of skin disease.…”

“…The disease primarily affects the axillary, perineal, inguinal, intermammary, and inframammary regions 1 . Several autoimmune conditions and rheumatologic disorders have been associated with HS 2 . Herein, we report an unusual case of a patient with HS and relapsing polychondritis (RPC).…”

“…It was shown that there is an association with skin flares and that it tends to occur years after the onset of skin disease. Spondyloarthropathies F I G U R E 1 A,B, Hypertrophic scars, nodules, and draining sinuses linking inflammatory lesions in the axillary and gluteal regions common association 2. To the best of our knowledge, there is no reported case in the literature in which HS was associated with RPC.…”

“…Hurley stage I is characterized by a single nodule with no sinus tracts, stage II has more than one nodule with little tunneling, and stage III has multiple nodules with a lot of sinus tracts and scars involving an entire area of the body, as in the case of our patient. 1Musculoskeletal association with HS has been reported 2. It was shown that there is an association with skin flares and that it tends to occur years after the onset of skin disease.…”

A Case of Overlapping Clinical Features of Idiopathic Granulomatous Mastitis, Hidradenitis Suppurativa, and Pyoderma Gangrenosum Successfully Treated with Adalimumab

“…In the literature, multiple cases of concurrent HS and PG (syndromic HS) have been described [ 16 , 17 ], with HS commonly preceding PG lesions by several months to years [ 18 ]. Several autoinflammatory arthritic-pyodermic syndromes like PG, acne, and HS (PASH) and PG, acne, pyogenic arthritis, and HS (PAPASH) have been recognized, some of them manifesting systemic symptoms of high fever and joint pain along with increased inflammatory activity in the blood and/or skin such as CRP, ESR, TNF-α, and interleukin-1 [ 19 , 20 , 21 ]. Moreover, a case of overlapping IGM and HS has been reported [ 22 ], in which the authors suggested that IGM might be a localized form of HS and that the link between these two entities may have been missed since IGM and HS patients are seen by gynecologists and dermatologists, respectively [ 22 ].…”