New insights into the diagnosis of hidradenitis suppurativa: Clinical presentations and phenotypes
Hessel H. van der Zee, MD,a and Gregor B. E. Jemec, MDb
Rotterdam, The Netherlands, and Roskilde, Denmark
It has been suggested that the clear symptomatology (painful lesions recognized by patients as boils), the specific areas affected, and the recurrence/chronicity of the lesions are sufficient for reliable, self-reported diagnosis, but this approach requires additional validation.
Different clinical presentations:
In 2013, Canoui-Poitrine et al14 identified HS subgroups by latent class analysis with a priori hypotheses in a cohort of 618 patients. They identified 3 phenotypes (Table II).14 The classification has not been further validated against risk factors or treatment outcomes.
Clinical experience suggests several other possible subtypes that also require additional exploration. The authors suggested that the possible subtypes below be considered in future studies.
The regular type:
Patients with regular HS fulfill all of the diagnostic criteria. This is probably the most common type, and all HS patients who lack other specific characteristics belong in this category.
Frictional furuncle type:
These patients are usually overweight; in addition to regular HS, they are characterized by the presentation of multiple deep nodules and abscesses on sites exposed to enhanced friction, such as the abdomen, thighs, and buttocks. The formation of tunnels and fistulas in these areas is unusual.
Scarring folliculitis type:
Patients with the scarring folliculitis type of HS have, in addition to regular HS, pustules, cysts, superficial nodules, depressed cribriform scarring, and double-ended comedones. These lesions are frequently seen on the buttocks, inguinal region, and pubic region. The formation of sinus tracts and fistulas in these sites may be unusual, and although the inflammatory lesions are small and superficial (Hurley stage I), scarring typically occurs. These patients are also frequently overweight and often smoke.
These patients are characterized by cyst formation and acne conglobata lesions on the back especially, but also the face. HS usually runs in the family in this type, and is moderate to severe, Hurley stages II to III. Patients are usually men and are not overweight. The Chinese cases in whom the initial finding of gamma-secretase mutations were found may belong to this putative group.
Patients with syndromic type HS are characterized by concomitant manifestations, such as pyoderma gangrenosum and arthritis, in the syndromic constellation recognized as pyoderma gangrenosum, acne, and suppurative hidradenitis syndrome or pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis supportive syndrome.
The existence of an ectopic type involving the face has been proposed.
These proposed clinical subtypes are not based on disease severity as outlined in the Hurley staging system. All of the 3 Hurley stages can occur in these subtypes. Future studies should show the ratios between these clinical subtypes in the HS population and whether or not these clinical subtypes can be validated or whether new ones need to be added.Tags: Acne Inversa Case Study Comorbidity Dermatology Diagnosis Genetics Pathology