CED
Clinical dermatology • Concise report
Clinical and Experimental Dermatology
Atypical hidradenitis suppurativa H. Naasan and A. Affleck Department of Dermatology, Ninewells Hospital, Dundee, UK doi:10.1111/ced.12655
Summary
Hidradenitis suppurativa (HS) is a chronic relapsing inflammatory disease resulting in subcutaneous abscesses, sinus tracts and scarring, arising predominantly in apocrine gland-bearing skin. The sites affected, in order of frequency, are the axillae, groins, perianal and perineal region, mammary and submammary skin, buttocks, and pubic region. Other sites that may be affected more rarely include the chest, eyelids, scalp, retroauricular and preauricular skin, thighs, and abdomen. Research has suggested that mechanical friction and follicular occlusion is the primary cause of HS. HS is one of the conditions of the ‘follicular occlusion tetrad’, which share a similar pathogenesis. HS at atypical sites may mimic other conditions both clinically and pathologically, so misdiagnosis is possible. We present a case series that illustrates a range of atypical sites affected. Our patients all had severe disease. Satisfactory control has not been achieved despite years of medical and surgical interventions.
Hidradenitis suppurativa (HS) is a chronic, relapsing, inflammatory disease, caused by mechanical friction and follicular occlusion.1 Subclinical inflammation in response to commensal microorganisms causes epidermal hyperplasia and keratosis in the follicular infundibulum, which is aggravated by smoking and mechanical friction. The hair follicles become plugged and enlarged, forming cysts, which then rupture, leading to abscesses and inflammatory nodules. Later, sinus tracts and scarring develop in more advanced cases.1–3 The follicular occlusion tetrad, consisting of HS, acne conglobata, dissecting scalp cellulitis and pilonidal sinus, share a similar pathogenesis, and so patients may have a history of any or all of these conditions. An underlying genetic predisposition may be present, with a positive family history, and recent studies have postulated several genetic subtypes with different physical correlates. Obesity is often associated with HS, presumably because of increased mechanical friction at skin flexures. Common sites for HS are those rich in apocrine glands: the axillae, groins, perianal and perineal region, Correspondence: Dr Hannah Naasan, Department of Dermatology, Ninewells Hospital, Dundee DD1 9SY, UK E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 14 November 2014
ª 2015 British Association of Dermatologists
mammary and submammary skin, buttocks, and pubic region. Other sites can be affected more rarely, including the chest,4 eyelids,4 scalp,5 retroauricular6 and preauricular7 skin, thighs, and abdomen. The terminology of the condition is disputed. ‘Hidradenitis suppurativa’ is the original name, and continues to be used most widely. In 2004, Sellheyer and Krahl encouraged the use of the term ‘acne inversa‘ instead, as follicular occlusion is common to both acne vulgaris and HS, which affects intertriginous regions not commonly affected by acne vulgaris.8 However, in 2007, this was rejected by Bazex et al.9 in France, stating that acne inversa is an incorrect term, as sebaceous glands are not involved. Furthermore, they felt that the differences between HS and acne vulgaris, including the role of smoking, the different clinical appearance, and the lack of response to acne treatments in HS, would be confusing if the term acne inversa was used.
Report We present four cases of patients who developed HS at atypical, nonflexural, apocrine-sparse sites (Fig. 1). All patients also had typical apocrine-rich flexural sites affected. Patient 1, a 41-year-old man, who first presented to us at the age of 25 years. He is obese and a smoker. He
Clinical and Experimental Dermatology
1
Atypical hidradenitis suppurativa H. Naasan and A. Affleck
Figure 1 Patient 1: occipital scalp hidradenitis suppurativa (HS).
Patient 3: HS of the posterolateral thigh, Patient 4: HS of the popliteal fossa.
was 23 years old at the onset of HS, which developed at the atypical sites of the posterior neck/scalp, pre-auricular cheek, lateral chest wall, and lateral hip region. Concurrent conditions were pilonidal sinus and pyoderma
2
Clinical and Experimental Dermatology
gangrenosum. Biological therapy with adalimumab was unsuccessful, and infliximab has only given partial response so far. Patient 2, 52-year-old a male smoker, developed HS at the age of 13 years and has attended Dermatology since the age of 24 years. The disease affects the beard and posterior scalp areas, in addition to the typical flexural sites including the groin, perineum and axillae. He also has history of acne conglobata. Treatment with long-term tetracyclines, isotretinoin, prednisolone and dapsone has given little benefit. Biological therapy has been offered but refused. Patient 3, a 61-year-old male smoker, developed HS at typical sites including the groin and axillae, and at the age of 52 a large discharging abscess on his posterolateral thigh. An associated disease is pilonidal sinus. Treatments tried include ciclosporin with limited effectiveness and acitretin with no success, but he has had partial response to infliximab. Patient 4, a 35-year-old female smoker, developed HS at the age of 14 years. In addition to having ‘typical’ HS in the axillae and perianal region, she has developed lesions on her back, and in the inframammary and lower sternal chest regions. She has also developed disease at her popliteal fossa and antecubital fossa (both sites not reported previously to our knowledge). An associated disease is acne conglobata. Treatment with recurrent courses of isotretinoin has had limited benefit, a trial of dapsone was unsuccessful, and 1 year of infliximab showed some initial benefit. There has been partial response to biological therapy with adalimumab, which she is still receiving. HS at atypical sites has been reported. One case series of four patients comprised one patient with HS at the jawline and posterior neck, two with HS at the posterior neck, and one with postauricular HS. Two patients in another study also had a typical sites of HS.5 A case report in 2012 presented a 30-year-old man with preauricular HS in isolation.7 There was also a case report in 2011 of a 28-year-old man with bilateral preauricular and earlobe HS, whose father was reported to have identical lesions that had improved with age.6 A 44-year-old patient with a left leg amputation developed recurrent inflammatory lesions in the stump due to the friction and occlusion from his prosthesis, and was diagnosed with HS.10 Recently, the classification of HS has been revisited. The previous classification included typical (recurrent painful nodules, papules and abscesses in apocrine-rich sites such as the axilla and groin) and atypical (more widespread lesions). In 2013, a study analysed clinical variables from 618 patients, using latent class
ª 2015 British Association of Dermatologists
Atypical hidradenitis suppurativa H. Naasan and A. Affleck
analysis, to report a new empirical classification for HS. They described the subtypes as ‘axillary–mammary’ (previously ‘typical’ HS), ‘follicular’ and ‘gluteal’ (both ‘atypical’ forms of HS).11 The ‘axillary–mammary’ class includes patients with axillary and breast involvement and hypertrophic scars. The ‘follicular’ class also had ear, chest, neck or leg involvement, and follicular lesions including epidermal cysts, pilonidal sinus and comedones. Compared with the patients with axillary–mammary HS, patients with follicular HS have higher disease severity, earlier onset and longer duration, and there is a higher proportion of men and of current/former smokers. There is also a higher proportion of men, and presence of higher mean body mass index (BMI), greater disease severity and longer duration in patients with follicular than in patients with gluteal disease. The gluteal class had gluteal involvement, follicular papules and folliculitis. These patients were more often smokers, had lower BMI and less severe disease despite longer duration than patients with axillary–mammary disease.11 In the future, it may be that treatment can be tailored to suit the phenotype (with or without an identified underlying genetic abnormality), in addition to addressing modifiable risk factors such as smoking and obesity.
Learning points HS can affect typical and atypical sites. ‘Acne inversa’ may be a preferable term as
apocrine glands are not necessary for local disease activity. Disease severity varies between subtypes of HS. Friction and follicular occlusion contribute to the pathogenesis in genetically predisposed individuals. Smoking status and obesity are modifiable risk factors in some patients.
ª 2015 British Association of Dermatologists
References 1 van der Zee HH, Laman JD, Boer J, Prens EP. Hidradenitis suppurativa: viewpoint on clinical phenotyping, pathogenesis and novel treatments. Exp Dermatol 2012; 21: 735–9. 2 Mortimer PS, Lunniss PJ. Hidradenitis suppurativa. J R Soc Med 2000; 93: 420–2. 3 Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol 2009; 4: 539–61. 4 Slade DEM, Powell BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis and management. BJPS 2003; 56: 451–61. 5 Syed ZU, Hamzavi IH. Atypical hidradenitis suppurativa involving the posterior neck and occiput. Arch Dermatol 2011; 147: 1343–4. 6 Satter E. Hereditary hidradenitis suppurativa restricted to the auricular region. Dermatol Online J 2012; 18: 8. 7 Jain S, Sardana K, Garg VK, Sinha S. Hidradenitis suppurativa involving the preauricular region: an unusual location. Indian J Dermatol Venereol Leprol 2012; 78: 229. 8 Sellheyer K, Krahl D. “Hidradenitis suppurativa” is acne inversa! An appeal to (finally) ban a misnomer. Int J Dermatol 2005; 44: 535–40. 9 Bazex J, Bayle P, San B. Hidradenitis suppurativa is acne inversa. Int J Dermatol 2007; 46: 330–2. 10 de Winter K, van der Zee HH, Prens EP. Is mechanical stress an important pathogenic factor in hidradenitis suppurativa? Exp Dermatol 2012; 21: 176–7. 11 Canoui-Poitrine F, Le Thuaut A, Revuz JE et al. Identification of three hidradenitis suppurativa phenotypes: latent class analysis of a cross-sectional study. J Invest Dermatol 2013; 133: 1506–11.
Clinical and Experimental Dermatology
3
Clinical dermatology • Concise report
Clinical and Experimental Dermatology
Atypical hidradenitis suppurativa H. Naasan and A. Affleck Department of Dermatology, Ninewells Hospital, Dundee, UK doi:10.1111/ced.12655
Summary
Hidradenitis suppurativa (HS) is a chronic relapsing inflammatory disease resulting in subcutaneous abscesses, sinus tracts and scarring, arising predominantly in apocrine gland-bearing skin. The sites affected, in order of frequency, are the axillae, groins, perianal and perineal region, mammary and submammary skin, buttocks, and pubic region. Other sites that may be affected more rarely include the chest, eyelids, scalp, retroauricular and preauricular skin, thighs, and abdomen. Research has suggested that mechanical friction and follicular occlusion is the primary cause of HS. HS is one of the conditions of the ‘follicular occlusion tetrad’, which share a similar pathogenesis. HS at atypical sites may mimic other conditions both clinically and pathologically, so misdiagnosis is possible. We present a case series that illustrates a range of atypical sites affected. Our patients all had severe disease. Satisfactory control has not been achieved despite years of medical and surgical interventions.
Hidradenitis suppurativa (HS) is a chronic, relapsing, inflammatory disease, caused by mechanical friction and follicular occlusion.1 Subclinical inflammation in response to commensal microorganisms causes epidermal hyperplasia and keratosis in the follicular infundibulum, which is aggravated by smoking and mechanical friction. The hair follicles become plugged and enlarged, forming cysts, which then rupture, leading to abscesses and inflammatory nodules. Later, sinus tracts and scarring develop in more advanced cases.1–3 The follicular occlusion tetrad, consisting of HS, acne conglobata, dissecting scalp cellulitis and pilonidal sinus, share a similar pathogenesis, and so patients may have a history of any or all of these conditions. An underlying genetic predisposition may be present, with a positive family history, and recent studies have postulated several genetic subtypes with different physical correlates. Obesity is often associated with HS, presumably because of increased mechanical friction at skin flexures. Common sites for HS are those rich in apocrine glands: the axillae, groins, perianal and perineal region, Correspondence: Dr Hannah Naasan, Department of Dermatology, Ninewells Hospital, Dundee DD1 9SY, UK E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 14 November 2014
ª 2015 British Association of Dermatologists
mammary and submammary skin, buttocks, and pubic region. Other sites can be affected more rarely, including the chest,4 eyelids,4 scalp,5 retroauricular6 and preauricular7 skin, thighs, and abdomen. The terminology of the condition is disputed. ‘Hidradenitis suppurativa’ is the original name, and continues to be used most widely. In 2004, Sellheyer and Krahl encouraged the use of the term ‘acne inversa‘ instead, as follicular occlusion is common to both acne vulgaris and HS, which affects intertriginous regions not commonly affected by acne vulgaris.8 However, in 2007, this was rejected by Bazex et al.9 in France, stating that acne inversa is an incorrect term, as sebaceous glands are not involved. Furthermore, they felt that the differences between HS and acne vulgaris, including the role of smoking, the different clinical appearance, and the lack of response to acne treatments in HS, would be confusing if the term acne inversa was used.
Report We present four cases of patients who developed HS at atypical, nonflexural, apocrine-sparse sites (Fig. 1). All patients also had typical apocrine-rich flexural sites affected. Patient 1, a 41-year-old man, who first presented to us at the age of 25 years. He is obese and a smoker. He
Clinical and Experimental Dermatology
1
Atypical hidradenitis suppurativa H. Naasan and A. Affleck
Figure 1 Patient 1: occipital scalp hidradenitis suppurativa (HS).
Patient 3: HS of the posterolateral thigh, Patient 4: HS of the popliteal fossa.
was 23 years old at the onset of HS, which developed at the atypical sites of the posterior neck/scalp, pre-auricular cheek, lateral chest wall, and lateral hip region. Concurrent conditions were pilonidal sinus and pyoderma
2
Clinical and Experimental Dermatology
gangrenosum. Biological therapy with adalimumab was unsuccessful, and infliximab has only given partial response so far. Patient 2, 52-year-old a male smoker, developed HS at the age of 13 years and has attended Dermatology since the age of 24 years. The disease affects the beard and posterior scalp areas, in addition to the typical flexural sites including the groin, perineum and axillae. He also has history of acne conglobata. Treatment with long-term tetracyclines, isotretinoin, prednisolone and dapsone has given little benefit. Biological therapy has been offered but refused. Patient 3, a 61-year-old male smoker, developed HS at typical sites including the groin and axillae, and at the age of 52 a large discharging abscess on his posterolateral thigh. An associated disease is pilonidal sinus. Treatments tried include ciclosporin with limited effectiveness and acitretin with no success, but he has had partial response to infliximab. Patient 4, a 35-year-old female smoker, developed HS at the age of 14 years. In addition to having ‘typical’ HS in the axillae and perianal region, she has developed lesions on her back, and in the inframammary and lower sternal chest regions. She has also developed disease at her popliteal fossa and antecubital fossa (both sites not reported previously to our knowledge). An associated disease is acne conglobata. Treatment with recurrent courses of isotretinoin has had limited benefit, a trial of dapsone was unsuccessful, and 1 year of infliximab showed some initial benefit. There has been partial response to biological therapy with adalimumab, which she is still receiving. HS at atypical sites has been reported. One case series of four patients comprised one patient with HS at the jawline and posterior neck, two with HS at the posterior neck, and one with postauricular HS. Two patients in another study also had a typical sites of HS.5 A case report in 2012 presented a 30-year-old man with preauricular HS in isolation.7 There was also a case report in 2011 of a 28-year-old man with bilateral preauricular and earlobe HS, whose father was reported to have identical lesions that had improved with age.6 A 44-year-old patient with a left leg amputation developed recurrent inflammatory lesions in the stump due to the friction and occlusion from his prosthesis, and was diagnosed with HS.10 Recently, the classification of HS has been revisited. The previous classification included typical (recurrent painful nodules, papules and abscesses in apocrine-rich sites such as the axilla and groin) and atypical (more widespread lesions). In 2013, a study analysed clinical variables from 618 patients, using latent class
ª 2015 British Association of Dermatologists
Atypical hidradenitis suppurativa H. Naasan and A. Affleck
analysis, to report a new empirical classification for HS. They described the subtypes as ‘axillary–mammary’ (previously ‘typical’ HS), ‘follicular’ and ‘gluteal’ (both ‘atypical’ forms of HS).11 The ‘axillary–mammary’ class includes patients with axillary and breast involvement and hypertrophic scars. The ‘follicular’ class also had ear, chest, neck or leg involvement, and follicular lesions including epidermal cysts, pilonidal sinus and comedones. Compared with the patients with axillary–mammary HS, patients with follicular HS have higher disease severity, earlier onset and longer duration, and there is a higher proportion of men and of current/former smokers. There is also a higher proportion of men, and presence of higher mean body mass index (BMI), greater disease severity and longer duration in patients with follicular than in patients with gluteal disease. The gluteal class had gluteal involvement, follicular papules and folliculitis. These patients were more often smokers, had lower BMI and less severe disease despite longer duration than patients with axillary–mammary disease.11 In the future, it may be that treatment can be tailored to suit the phenotype (with or without an identified underlying genetic abnormality), in addition to addressing modifiable risk factors such as smoking and obesity.
Learning points HS can affect typical and atypical sites. ‘Acne inversa’ may be a preferable term as
apocrine glands are not necessary for local disease activity. Disease severity varies between subtypes of HS. Friction and follicular occlusion contribute to the pathogenesis in genetically predisposed individuals. Smoking status and obesity are modifiable risk factors in some patients.
ª 2015 British Association of Dermatologists
References 1 van der Zee HH, Laman JD, Boer J, Prens EP. Hidradenitis suppurativa: viewpoint on clinical phenotyping, pathogenesis and novel treatments. Exp Dermatol 2012; 21: 735–9. 2 Mortimer PS, Lunniss PJ. Hidradenitis suppurativa. J R Soc Med 2000; 93: 420–2. 3 Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol 2009; 4: 539–61. 4 Slade DEM, Powell BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis and management. BJPS 2003; 56: 451–61. 5 Syed ZU, Hamzavi IH. Atypical hidradenitis suppurativa involving the posterior neck and occiput. Arch Dermatol 2011; 147: 1343–4. 6 Satter E. Hereditary hidradenitis suppurativa restricted to the auricular region. Dermatol Online J 2012; 18: 8. 7 Jain S, Sardana K, Garg VK, Sinha S. Hidradenitis suppurativa involving the preauricular region: an unusual location. Indian J Dermatol Venereol Leprol 2012; 78: 229. 8 Sellheyer K, Krahl D. “Hidradenitis suppurativa” is acne inversa! An appeal to (finally) ban a misnomer. Int J Dermatol 2005; 44: 535–40. 9 Bazex J, Bayle P, San B. Hidradenitis suppurativa is acne inversa. Int J Dermatol 2007; 46: 330–2. 10 de Winter K, van der Zee HH, Prens EP. Is mechanical stress an important pathogenic factor in hidradenitis suppurativa? Exp Dermatol 2012; 21: 176–7. 11 Canoui-Poitrine F, Le Thuaut A, Revuz JE et al. Identification of three hidradenitis suppurativa phenotypes: latent class analysis of a cross-sectional study. J Invest Dermatol 2013; 133: 1506–11.
Clinical and Experimental Dermatology
3
