Research letter
A systematic review of terms used to describe hidradenitis suppurativa DOI: 10.1111/bjd.13940 DEAR EDITOR, The diagnosis of hidradenitis suppurativa (HS) is clinical and relatively straightforward for an experienced clinician. However, the terminology needed for a detailed and precise description of the lesions is more problematic. The aim of this study was to identify, enumerate and clearly define the primary lesions described in HS so as to compile a glossary that can be used to define clinical patient profiles. A review of the literature in English was performed on Medline (PubMed), extending from 1949 to October 2013. This review targeted semantic aspects. The keywords used were ‘hidradenitis suppurativa’, ‘Verneuil disease’ and ‘acne inversa’. We systematically perused all of the titles of the articles and abstracts retrieved from the database using these keywords. Titles relating to clinical description, semiology and HS morphology, and titles referring to the association between HS and other pathologies were selected. In the selection, only articles in English were retained. The selected articles were then all extracted and perused. A similar search was performed in several textbooks. Following this exploration, the terms used for clinical description, semiology and morphology were systematically noted. The descriptive terms collected in this way were then studied, defined and critically appraised so as to produce a glossary of terms specifically describing HS. In total 249 articles were studied, identifying 65 different English descriptive terms used. Of these, 40 terms had a consensus definition in the medical community. The other terms were less well established. Among these figurative terms, some were nevertheless unambiguous and easy to understand (e.g. blind boil, bump, ropelike lesion, pit, tunnel, ridge etc.). However, several of these terms were synonyms, so that only the most relevant were retained. A number of terms were rather ambiguous, often not very meaningful and had no apparent bearing on the object of the description, and hence were not liable to be reused. For example, we found the terms ‘box-car’,1 ‘scar entrapped lesion’,2 ‘toothbrush sign’,3 ‘worm-eaten lesion’1,4 and ‘collar button lesion’.5 These terms, whose link with a clinical lesion was hard to establish, were excluded from the glossary after critical analysis. The nouns were sorted from the adjectives, as adjectives can enable nouns to be more finely described. These adjectives, for the most part widely used in medical practice, 1298
British Journal of Dermatology (2015) 173, pp1298–1300
although not necessarily in dermatology, were not all included in the final glossary, but in some instances were used to refine the meaning of another term, when they complied with a generally accepted definition. The adjectives appearing the most useful to describe HS were included. Words relating solely to nosography rather than morphology were not used or retained in this purely descriptive approach. For example, we rejected these nosographic terms: abscess, (blind) boil, carbuncle-like lesion, epidermal cyst, fibrosis, scar and tumour. The terms derived from the literature that were retained and defined in this glossary were classified by way of a morphological approach, namely palpable lesions, either raised or depressed, with solid or liquid content; alterations of the skin surface – thinned, broken, or absent; and finally complex lesions combining several of the above (Table 1). The suggested glossary includes 13 nouns and 13 adjectives that are defined and classified. For clinically defined diseases, nosology and physician assessment of severity both depend on semiology. The establishment of a well-defined glossary is therefore a prerequisite for scientific development. In the absence of a pathognomonic test, these descriptive terms form the basis of our understanding and communication regarding the disease. Therefore we next performed a critical appraisal of the terms so as to form a glossary comprising solely nouns and adjectives that were clear in meaning and useful for the description of this condition. Among these terms derived from the literature we distinguished those relating to semiology alone, and those relating to nosography. Indeed nosography (the systematic description of diseases) integrates semiology and aetiology, and suggests a physiopathological mechanism, unlike semiology, which, strictly, is the study of signs. These are two different concepts, often confused, as was noted in this review of the literature. Yet for purely descriptive purposes, only the semiological terms should be used, which is the reason why we excluded all nosographic terms from the final glossary. We thus excluded the words abscess and scar, despite the fact that they are the most frequently used terms, because they assume a pathogenic process. Indeed, it may be speculated that what is described as an abscess generally corresponds clinically to an inflammatory nodule. But an inflammatory nodule is not necessarily synonymous with an abscess, as it can relate to an inflammatory flare of an epidermoid or trichilemmal cyst, in which case the pathology is very different. In fact, the description of an abscess is difficult because a ‘nodule’ would imply © 2015 British Association of Dermatologists
Research letter 1299 Table 1 Glossary of terms to be retained and used (in italics), and their definitions when required (A) Palpable lesions (1) Lesions that are generally raised Solid content Papule: raised lesion clearly outlined measuring ≤ 10 mm Plaque: raised lesion measuring > 10 mm, wider than high Nodule: raised lesion, round, diameter > 10 mm, three dimensional Ridge: palpable elongated fine lesion Cord: linear lesion suggesting a rope or cord on palpation Morphological variations on cord Bridle: a strip of skin that may or may not be a cord, linking two anatomical zones and causing adherences Strand: fine, narrow cord Adjectives enabling finer description of the morphology of solid palpable lesions Puckered Tender (for lesions with pain elicited on palpation) Liquid content Pustule: lesion < 5 mm containing cloudy fluid (pus) (2) Depressed lesions Adjectives enabling description of the morphology of depressed skin lesions Shallow Deep Pitted Punctate Cribriform: with several small, superficial, circular, cutaneous depressions dotted across the skin surface Honeycomb: with several, uniform depressions in honeycomb formation – a variant of cribriform Crypt: with irregular skin depressions with cracks or crevices (B) Alterations of the skin surface (1) Thinned surface Atrophy: thinning of the tegument, which retains a pinch mark; loss of elasticity; smooth pearly appearance (2) Broken surface Tunnel: opening of variable length and depth, ending at the skin surface, and sometimes oozing a fluid. Term to be preferred if the lesion extends beyond the skin, and if not related to a skin structure such as a pilosebaceous or sweat gland duct Adjectives enabling finer description of tunnel Draining Discharging Pore: opening not extending beyond the skin and usually related to a skin structure such as a pilosebaceous or sweat gland duct; for example, a comedo is a special type of pore Adjectives enabling finer description of pores and comedones Unipore Interconnected (3) Loss of skin surface Ulceration: complete loss of deep substance (C) Interconnecting lesions Bridge: a cord ‘bridging’ a cutaneous depression, linking the two edges of the depression
a raised rather than a subcutaneous lesion. It should be underlined that the terms fistula, sinus and comedo belong more to the area of nosography than semiology. However, there is no semiological terminology enabling a description of this type of break in the skin surface. Consequently, we suggest using the terms tunnel or pore. Tunnel should be used in case of a deep break, extending beyond the skin, usually not related to a skin structure such as a pilosebaceous or sweat gland duct, especially if it drains pus. Pore should be used in case of a superficial break restricted to the skin, usually in connection with a pilosebaceous or sweat gland duct. The comedo is a special type of pore. Consensus on semiotics is a prerequisite for precise communication. An analysis and clarification of the semiotics of HS is © 2015 British Association of Dermatologists
necessary in order to produce a detailed, precise description of the lesions, which, in turn, is a prerequisite for classification of patients into homogeneous groups and assessment of disease severity. Both of these goals are important, as careful clinical subclassification may identify groups with significant differences in prognosis or in which the therapeutic response may vary widely. A careful, well-defined description of the disease is the classical clinical approach to personalized medicine. This is all the more important because there are recognized monogenic and complex forms of HS, syndromic forms (e.g. PAPA syndrome: pyogenic arthritis, pyoderma gangrenosum and acne), forms associated with other diseases (e.g. Crohn disease) and isolated forms. It is not known whether there are significant clinical differences between these variants, for lack of British Journal of Dermatology (2015) 173, pp1298–1300
1300 Research letter
means to describe them adequately. In addition, our clinical experience has shown that there is marked clinical heterogeneity, and highly varied treatment response profiles among patients with HS. For example, some patients respond well to tumour necrosis factor-a antibodies, while others, among whom these treatments have failed, will respond to treatments inhibiting interleukin-1. HS most probably also presents a heterogeneous spectrum, where clinical classification is possible by the association of the lesions present and their distribution. To the best of our knowledge, no articles exploring the straightforward clinical description of HS lesions in such detail are available in the literature. There are a number of articles on the subject of physiopathology or treatment of the disease, but none that specifically addresses the morphological terminology. The restriction to the English language is a limitation of this work. However, it is suggested that the inclusion of other languages would not strengthen the findings. If the need for clarification of the glossary is apparent in the most commonly used scientific language, English, it may only be expected to be even greater if additional languages were included in the analysis. The main advantage of this study is thus the development of an inventory of lesions specific to HS so as to enable the definition of homogeneous patient groups in the future, on the basis of a classification of semiological terms that could help to direct patient care. This simple glossary is now available for further testing.
British Journal of Dermatology (2015) 173, pp1298–1300
1
Clinique Dermatologique, Hoˆpitaux Civils de Strasbourg, 1 Place de l’Hoˆpital, 67000 Strasbourg, France 2 Department of Dermatology, Roskilde Hospital, University of Copenhagen, Copenhagen, Denmark Correspondence: Dan Lipsker. E-mail: [email protected]
M. FREYSZ1 G.B.E. JEMEC2 D. LIPSKER1
References 1 Poli F, Wolkenstein P, Revuz J. Back and face involvement in hidradenitis suppurativa. Dermatology 2010; 221:137–41. 2 Chicarilli ZN. Follicular occlusion triad: hidradenitis suppurativa, acne conglobata, and dissecting cellulitis of the scalp. Ann Plast Surg 1987; 18:230–7. 3 Jansen T, Plewig G. Acne inversa. Int J Dermatol 1998; 37:96–100. 4 Domenech C, Matarredona J, Escribano-Stable JC et al. Facial hidradenitis suppurativa in a 28-year-old male responding to finasteride. Dermatology 2012; 224:307–8. 5 Culp CE. Chronic hidradenitis suppurativa of the anal canal. A surgical skin disease. Dis Colon Rectum 1983; 26:669–76.
Funding sources: none. Conflicts of interest: none declared.
© 2015 British Association of Dermatologists
A systematic review of terms used to describe hidradenitis suppurativa DOI: 10.1111/bjd.13940 DEAR EDITOR, The diagnosis of hidradenitis suppurativa (HS) is clinical and relatively straightforward for an experienced clinician. However, the terminology needed for a detailed and precise description of the lesions is more problematic. The aim of this study was to identify, enumerate and clearly define the primary lesions described in HS so as to compile a glossary that can be used to define clinical patient profiles. A review of the literature in English was performed on Medline (PubMed), extending from 1949 to October 2013. This review targeted semantic aspects. The keywords used were ‘hidradenitis suppurativa’, ‘Verneuil disease’ and ‘acne inversa’. We systematically perused all of the titles of the articles and abstracts retrieved from the database using these keywords. Titles relating to clinical description, semiology and HS morphology, and titles referring to the association between HS and other pathologies were selected. In the selection, only articles in English were retained. The selected articles were then all extracted and perused. A similar search was performed in several textbooks. Following this exploration, the terms used for clinical description, semiology and morphology were systematically noted. The descriptive terms collected in this way were then studied, defined and critically appraised so as to produce a glossary of terms specifically describing HS. In total 249 articles were studied, identifying 65 different English descriptive terms used. Of these, 40 terms had a consensus definition in the medical community. The other terms were less well established. Among these figurative terms, some were nevertheless unambiguous and easy to understand (e.g. blind boil, bump, ropelike lesion, pit, tunnel, ridge etc.). However, several of these terms were synonyms, so that only the most relevant were retained. A number of terms were rather ambiguous, often not very meaningful and had no apparent bearing on the object of the description, and hence were not liable to be reused. For example, we found the terms ‘box-car’,1 ‘scar entrapped lesion’,2 ‘toothbrush sign’,3 ‘worm-eaten lesion’1,4 and ‘collar button lesion’.5 These terms, whose link with a clinical lesion was hard to establish, were excluded from the glossary after critical analysis. The nouns were sorted from the adjectives, as adjectives can enable nouns to be more finely described. These adjectives, for the most part widely used in medical practice, 1298
British Journal of Dermatology (2015) 173, pp1298–1300
although not necessarily in dermatology, were not all included in the final glossary, but in some instances were used to refine the meaning of another term, when they complied with a generally accepted definition. The adjectives appearing the most useful to describe HS were included. Words relating solely to nosography rather than morphology were not used or retained in this purely descriptive approach. For example, we rejected these nosographic terms: abscess, (blind) boil, carbuncle-like lesion, epidermal cyst, fibrosis, scar and tumour. The terms derived from the literature that were retained and defined in this glossary were classified by way of a morphological approach, namely palpable lesions, either raised or depressed, with solid or liquid content; alterations of the skin surface – thinned, broken, or absent; and finally complex lesions combining several of the above (Table 1). The suggested glossary includes 13 nouns and 13 adjectives that are defined and classified. For clinically defined diseases, nosology and physician assessment of severity both depend on semiology. The establishment of a well-defined glossary is therefore a prerequisite for scientific development. In the absence of a pathognomonic test, these descriptive terms form the basis of our understanding and communication regarding the disease. Therefore we next performed a critical appraisal of the terms so as to form a glossary comprising solely nouns and adjectives that were clear in meaning and useful for the description of this condition. Among these terms derived from the literature we distinguished those relating to semiology alone, and those relating to nosography. Indeed nosography (the systematic description of diseases) integrates semiology and aetiology, and suggests a physiopathological mechanism, unlike semiology, which, strictly, is the study of signs. These are two different concepts, often confused, as was noted in this review of the literature. Yet for purely descriptive purposes, only the semiological terms should be used, which is the reason why we excluded all nosographic terms from the final glossary. We thus excluded the words abscess and scar, despite the fact that they are the most frequently used terms, because they assume a pathogenic process. Indeed, it may be speculated that what is described as an abscess generally corresponds clinically to an inflammatory nodule. But an inflammatory nodule is not necessarily synonymous with an abscess, as it can relate to an inflammatory flare of an epidermoid or trichilemmal cyst, in which case the pathology is very different. In fact, the description of an abscess is difficult because a ‘nodule’ would imply © 2015 British Association of Dermatologists
Research letter 1299 Table 1 Glossary of terms to be retained and used (in italics), and their definitions when required (A) Palpable lesions (1) Lesions that are generally raised Solid content Papule: raised lesion clearly outlined measuring ≤ 10 mm Plaque: raised lesion measuring > 10 mm, wider than high Nodule: raised lesion, round, diameter > 10 mm, three dimensional Ridge: palpable elongated fine lesion Cord: linear lesion suggesting a rope or cord on palpation Morphological variations on cord Bridle: a strip of skin that may or may not be a cord, linking two anatomical zones and causing adherences Strand: fine, narrow cord Adjectives enabling finer description of the morphology of solid palpable lesions Puckered Tender (for lesions with pain elicited on palpation) Liquid content Pustule: lesion < 5 mm containing cloudy fluid (pus) (2) Depressed lesions Adjectives enabling description of the morphology of depressed skin lesions Shallow Deep Pitted Punctate Cribriform: with several small, superficial, circular, cutaneous depressions dotted across the skin surface Honeycomb: with several, uniform depressions in honeycomb formation – a variant of cribriform Crypt: with irregular skin depressions with cracks or crevices (B) Alterations of the skin surface (1) Thinned surface Atrophy: thinning of the tegument, which retains a pinch mark; loss of elasticity; smooth pearly appearance (2) Broken surface Tunnel: opening of variable length and depth, ending at the skin surface, and sometimes oozing a fluid. Term to be preferred if the lesion extends beyond the skin, and if not related to a skin structure such as a pilosebaceous or sweat gland duct Adjectives enabling finer description of tunnel Draining Discharging Pore: opening not extending beyond the skin and usually related to a skin structure such as a pilosebaceous or sweat gland duct; for example, a comedo is a special type of pore Adjectives enabling finer description of pores and comedones Unipore Interconnected (3) Loss of skin surface Ulceration: complete loss of deep substance (C) Interconnecting lesions Bridge: a cord ‘bridging’ a cutaneous depression, linking the two edges of the depression
a raised rather than a subcutaneous lesion. It should be underlined that the terms fistula, sinus and comedo belong more to the area of nosography than semiology. However, there is no semiological terminology enabling a description of this type of break in the skin surface. Consequently, we suggest using the terms tunnel or pore. Tunnel should be used in case of a deep break, extending beyond the skin, usually not related to a skin structure such as a pilosebaceous or sweat gland duct, especially if it drains pus. Pore should be used in case of a superficial break restricted to the skin, usually in connection with a pilosebaceous or sweat gland duct. The comedo is a special type of pore. Consensus on semiotics is a prerequisite for precise communication. An analysis and clarification of the semiotics of HS is © 2015 British Association of Dermatologists
necessary in order to produce a detailed, precise description of the lesions, which, in turn, is a prerequisite for classification of patients into homogeneous groups and assessment of disease severity. Both of these goals are important, as careful clinical subclassification may identify groups with significant differences in prognosis or in which the therapeutic response may vary widely. A careful, well-defined description of the disease is the classical clinical approach to personalized medicine. This is all the more important because there are recognized monogenic and complex forms of HS, syndromic forms (e.g. PAPA syndrome: pyogenic arthritis, pyoderma gangrenosum and acne), forms associated with other diseases (e.g. Crohn disease) and isolated forms. It is not known whether there are significant clinical differences between these variants, for lack of British Journal of Dermatology (2015) 173, pp1298–1300
1300 Research letter
means to describe them adequately. In addition, our clinical experience has shown that there is marked clinical heterogeneity, and highly varied treatment response profiles among patients with HS. For example, some patients respond well to tumour necrosis factor-a antibodies, while others, among whom these treatments have failed, will respond to treatments inhibiting interleukin-1. HS most probably also presents a heterogeneous spectrum, where clinical classification is possible by the association of the lesions present and their distribution. To the best of our knowledge, no articles exploring the straightforward clinical description of HS lesions in such detail are available in the literature. There are a number of articles on the subject of physiopathology or treatment of the disease, but none that specifically addresses the morphological terminology. The restriction to the English language is a limitation of this work. However, it is suggested that the inclusion of other languages would not strengthen the findings. If the need for clarification of the glossary is apparent in the most commonly used scientific language, English, it may only be expected to be even greater if additional languages were included in the analysis. The main advantage of this study is thus the development of an inventory of lesions specific to HS so as to enable the definition of homogeneous patient groups in the future, on the basis of a classification of semiological terms that could help to direct patient care. This simple glossary is now available for further testing.
British Journal of Dermatology (2015) 173, pp1298–1300
1
Clinique Dermatologique, Hoˆpitaux Civils de Strasbourg, 1 Place de l’Hoˆpital, 67000 Strasbourg, France 2 Department of Dermatology, Roskilde Hospital, University of Copenhagen, Copenhagen, Denmark Correspondence: Dan Lipsker. E-mail: [email protected]
M. FREYSZ1 G.B.E. JEMEC2 D. LIPSKER1
References 1 Poli F, Wolkenstein P, Revuz J. Back and face involvement in hidradenitis suppurativa. Dermatology 2010; 221:137–41. 2 Chicarilli ZN. Follicular occlusion triad: hidradenitis suppurativa, acne conglobata, and dissecting cellulitis of the scalp. Ann Plast Surg 1987; 18:230–7. 3 Jansen T, Plewig G. Acne inversa. Int J Dermatol 1998; 37:96–100. 4 Domenech C, Matarredona J, Escribano-Stable JC et al. Facial hidradenitis suppurativa in a 28-year-old male responding to finasteride. Dermatology 2012; 224:307–8. 5 Culp CE. Chronic hidradenitis suppurativa of the anal canal. A surgical skin disease. Dis Colon Rectum 1983; 26:669–76.
Funding sources: none. Conflicts of interest: none declared.
© 2015 British Association of Dermatologists
