Clinical and J'.xperimental
Dermatology
1^92; 1/: 305-306.
Review Article
What is pseudopelade? yi.^:>.\\^m-.K Slade Hospital. Ueadtngton, Oxford. UK Accepted for puhltcation 3 October 1991
In the two detailed reference books in the field of hair and scalp diseases, there is comprehensi\e cover ol' the c^inicaWy diffiicuh area of cicatricial alopecia. With refjard to pseudopelade, Orfanos et al.^ state that 'today Brocq's pseudopelade must be considered a polyetiologic disorder. The pathomorphologic changes which finally lead to this cicatricial end-stage have not >et been determined. Treatment is determined by finding the underlying basic defect.' Rook et al.- state that 'recent studies strongly support the fact that pseudopelade is a distinct entity.' In view of this polarity of opinion and the fact that most experienced clinicians have difficulty in diagnosing cicatricial alopecias, it is perhaps useful at rhis time to consider the history and current status of pseudopelade in relation to other chronic inilammatory diseases which may lead to differential diagnostic confusion. The term pseudopelade is attributable to Brocq,' differentiating this chronic, slowK progressing, permanent tvpe of alopecia from the 'peladc' of alopecia areata; this \attcT term had ahcady existed ior more than 201) \ears.' Before entering into the puzzle created by the pseudopelade literature of the last 106 years, it is apposite to note that within 22 years of the initial paper of 1885,' Brocq himself admitted that the entity and its title continued to cause confusion!'^ Photinos (1930),'' in a detailed clinical and pathological treatise, presented some of the earliest literature implying that pseudopelade is a distinct entity. He made the general point that it is a syndrome in which destruction of follicles occurs leading to permanent patchy biildness that is not accompanied by any clinicall> evident inflammatory pathology. Despite this major finding, opinions remained equivocal until the entry of Degos et al!' '' These papers introduced the term Etat pseudopeladtque showing that in 70% of cases of this 'state' a preceding cause could be found discoid lupus erythematosus, lichen planus, sclerodevma, disturbai^ccs of ktYatrnv/ation, carcinornatous rnetasta.ses, benign lymphocytic infiltrates' and others. Thus etat pseudopeladique signified an end stage and not a nosological entity. Laymon and Murphy^'^' and Ludwig^' both detailed studies from tnajor authoritative centres, also contributed to this 'end stage' conclusion in the same era.
The major academic paper which began to change thinking came from Pinkus in 1978'" in a comprehensive hi,stopatho)ogical study of scarring and nun-scarring alopecias, including 180 cases which satisfied the criteria for pseudopelade. Using acid alcoholic orcein stain he studied the distribution of elastic fibres around the hair follicles in many sections from biopsies of cicatricial alopecia of a variety of types. He found that the fibrous strands which replaced destroyed lbilicles in lichen planus and lupus erythematosus consist of collagen without elastic-like bodies of clastic fibres. In lupus erythematosus there is widespread destruction of elastic fibres in the interfoilicular dermis. Of the 180 cases of pseudopelade, 106 showed additional features for which Pinkus coined the term 'fibrosing alopecia'. The most striking of these features were the development of elastic fibres around the lower part of the follicle and less peri follicular cellular infiltrate than in other 'pseudopelade' cases. The age and sex incidence of the two subgroups did not differ significantly. A^'hatever ones opinion was of this as a clinician, here was a major pathology expert separating lupus erytfiematosus and lichen planus from pseudopelade and even more importantly, recognizing this 'fibrosing alopecia' in which there was very little infiammation. However, no big clinical studies appeared to substantiate this and the Dego etat pseudopeladique 'gold standard' continued to prevail. Braun-Falco et al.^^ have now changed the balance towards pseudopelade being a distinct entity. Their clinical and histopathological observations in a large series proposed the following as diagnostic criteria. (a) Clinical criteria irregularly defined and coalescing patches of alopecia, moderate atrophy—late stage, mi\d perifolVicular erythema -early stage, female :male = 3:1, long course— > 2 years, slots progression with spontaneous termination possible (b) Direct immunofluorescence negative or only IgM 305
306
R.DAWBER
(c) Ilistological criteria absence of marked inflammation, absence of widespread scarring, absence of significant follicular plugging, absence, or marked decrease of sebaceous glands, presence of normal epidermis, fibrotic streams into subcutis (follicular 'ghosts').^ This is the clearest evidence yet that within the pseudopelade group there is a specific entity. One is moved to conclude that the name has outlived its usefulness. With this in mind it is attractive to consider the question recently posed by Pincelli et al.^^ {Pseudopelade of Brocq: An immunologically mediated disease?) Much of the literature of the fast 106 years (post Brocq) could support the concept of this being a primary scalp follicular (Pautoimmune) atrophy which can be separated from the scarring states induced by L|'.''''- and lichen planus."''''^ Indeed, if this primary scalp follicular atrophy is a specific autoimmune disease, then one might occasionally logically expect it to occur in individuals with other autoimmune diseases even lichen planus and lupus erythematosus?! Removing the name pseudopelade and much of the enigmatic literature of the last century, might at least lead to more logical research and perhaps eventually to specific therapy. References 1. Orfanos CK, Happlc R, tds. Hair and Itair Diseau-s, 1st edn. Kcr^m: Springur-t'erlag, J9W; p. (t2S. 2. Rook AJ, Dawber RPR, ciis. thseases of ihe Hair and ualp, 2nd edn. Oxford: Hlackwell Scientific Publicitions, 1991: p- 339.
3. Brocq I-. Alopecia. Journal of Cutaneous and Venereal Diseases 1885; 3: 49. 4. Brocq L. Pseudopelade. In: Traile f.lementaire de Dermatologie Fractique, Vol. 2. Parism-Doin Publishers, 1907: p. 648. 5. Photinos P. l.a pseudopelade de Brocq. Paris: Maloirc Publishers, 1930. 6. Degos R, Rabut R, Duperrat H, Leclercq R. Apropos de III cas d'alopecies cicatrkielles en petit aires (teignes exckies). L ' m t pseudopeladique. Bulletin de la Sociele Franfais DermatologieSyphilige 1951; 58: 451-452. 7. Degos R, Rabut R, Duperrat H, Leclercq R. L'ctat pscudopelacHque. Annales de Dermalologie et de Syphilitic 1954; 81, 5. 8. Degos R, Rabut R, Ilcwia J, Ktat pseudopeladique du a des metastases cacinomateuscs alopeciantes. Hiiltetui de la Societf Franfais Dermulologie-Syphiltge 1954; 61: 5(19 510. 9. Degos R, Rabut R, Duperrat B. Alopecie en pelil aires. Reticulose hmphocvrnire bcnigiic. Balkim de In S&aetc Franfi/i.'i Di'rmal«h/gie-SyphUtge 1955; 62: 134 135. 10. Laymon (^VV, Murphy RJ. The cicatricial alopecias. .7ww;'Wi// oJ Investigative Dermatology 1947; 8: 99 122. 11. Ludwig F. (1961) Uber die narbig-atrophischcn alopecien vom typ der pseudopelade de IJrocq. Berujs Dermatnsen 1961; 9: 257 270. 12. Pinkus H. Differential patterns of elastic fibres in scarrin|j,& nonscarring iiiopecias. Journal oj Culaiieous Faihotogy 1'I78; 5: 93. 13. Br^Hin-Falco O, Imci S, Schmoeckel C. Pseudopeiade oi Brocq, Dcrmalohfura 1986; 172: 18. 14. Pincclli C, Girolomoni G, Beiiassi L. Pscutlopelatlc of Brocq: an immunologically mediated disease? Dermalologica 1987; 176: 49. 15. Wilson CL, Burge SM, Dean H, Dawhcwr RPR, Wojnarowska F. A clinicopathological stud\ of scarring alopecia in chronic cutaneous lupus erythemaiosus. British Journal oj Dermatology 1990; 123: (Suppl. 37), 13. 16. Kaminsky A, Kaminskv C'\, dc Kaminsky AR, Ahukfia J. Liquen folicuhir akipeeiantc. Meduina Cutanea 1967; 2: 135. 17. Gray Prieto J. Pscudopelade of Brocq: its relationship to some forms of cicatricial alopecia and to lichen planus. Journal of InvcstifijiUve Dermalolugy 1955: 24: 322.
Dermatology
1^92; 1/: 305-306.
Review Article
What is pseudopelade? yi.^:>.\\^m-.K Slade Hospital. Ueadtngton, Oxford. UK Accepted for puhltcation 3 October 1991
In the two detailed reference books in the field of hair and scalp diseases, there is comprehensi\e cover ol' the c^inicaWy diffiicuh area of cicatricial alopecia. With refjard to pseudopelade, Orfanos et al.^ state that 'today Brocq's pseudopelade must be considered a polyetiologic disorder. The pathomorphologic changes which finally lead to this cicatricial end-stage have not >et been determined. Treatment is determined by finding the underlying basic defect.' Rook et al.- state that 'recent studies strongly support the fact that pseudopelade is a distinct entity.' In view of this polarity of opinion and the fact that most experienced clinicians have difficulty in diagnosing cicatricial alopecias, it is perhaps useful at rhis time to consider the history and current status of pseudopelade in relation to other chronic inilammatory diseases which may lead to differential diagnostic confusion. The term pseudopelade is attributable to Brocq,' differentiating this chronic, slowK progressing, permanent tvpe of alopecia from the 'peladc' of alopecia areata; this \attcT term had ahcady existed ior more than 201) \ears.' Before entering into the puzzle created by the pseudopelade literature of the last 106 years, it is apposite to note that within 22 years of the initial paper of 1885,' Brocq himself admitted that the entity and its title continued to cause confusion!'^ Photinos (1930),'' in a detailed clinical and pathological treatise, presented some of the earliest literature implying that pseudopelade is a distinct entity. He made the general point that it is a syndrome in which destruction of follicles occurs leading to permanent patchy biildness that is not accompanied by any clinicall> evident inflammatory pathology. Despite this major finding, opinions remained equivocal until the entry of Degos et al!' '' These papers introduced the term Etat pseudopeladtque showing that in 70% of cases of this 'state' a preceding cause could be found discoid lupus erythematosus, lichen planus, sclerodevma, disturbai^ccs of ktYatrnv/ation, carcinornatous rnetasta.ses, benign lymphocytic infiltrates' and others. Thus etat pseudopeladique signified an end stage and not a nosological entity. Laymon and Murphy^'^' and Ludwig^' both detailed studies from tnajor authoritative centres, also contributed to this 'end stage' conclusion in the same era.
The major academic paper which began to change thinking came from Pinkus in 1978'" in a comprehensive hi,stopatho)ogical study of scarring and nun-scarring alopecias, including 180 cases which satisfied the criteria for pseudopelade. Using acid alcoholic orcein stain he studied the distribution of elastic fibres around the hair follicles in many sections from biopsies of cicatricial alopecia of a variety of types. He found that the fibrous strands which replaced destroyed lbilicles in lichen planus and lupus erythematosus consist of collagen without elastic-like bodies of clastic fibres. In lupus erythematosus there is widespread destruction of elastic fibres in the interfoilicular dermis. Of the 180 cases of pseudopelade, 106 showed additional features for which Pinkus coined the term 'fibrosing alopecia'. The most striking of these features were the development of elastic fibres around the lower part of the follicle and less peri follicular cellular infiltrate than in other 'pseudopelade' cases. The age and sex incidence of the two subgroups did not differ significantly. A^'hatever ones opinion was of this as a clinician, here was a major pathology expert separating lupus erytfiematosus and lichen planus from pseudopelade and even more importantly, recognizing this 'fibrosing alopecia' in which there was very little infiammation. However, no big clinical studies appeared to substantiate this and the Dego etat pseudopeladique 'gold standard' continued to prevail. Braun-Falco et al.^^ have now changed the balance towards pseudopelade being a distinct entity. Their clinical and histopathological observations in a large series proposed the following as diagnostic criteria. (a) Clinical criteria irregularly defined and coalescing patches of alopecia, moderate atrophy—late stage, mi\d perifolVicular erythema -early stage, female :male = 3:1, long course— > 2 years, slots progression with spontaneous termination possible (b) Direct immunofluorescence negative or only IgM 305
306
R.DAWBER
(c) Ilistological criteria absence of marked inflammation, absence of widespread scarring, absence of significant follicular plugging, absence, or marked decrease of sebaceous glands, presence of normal epidermis, fibrotic streams into subcutis (follicular 'ghosts').^ This is the clearest evidence yet that within the pseudopelade group there is a specific entity. One is moved to conclude that the name has outlived its usefulness. With this in mind it is attractive to consider the question recently posed by Pincelli et al.^^ {Pseudopelade of Brocq: An immunologically mediated disease?) Much of the literature of the fast 106 years (post Brocq) could support the concept of this being a primary scalp follicular (Pautoimmune) atrophy which can be separated from the scarring states induced by L|'.''''- and lichen planus."''''^ Indeed, if this primary scalp follicular atrophy is a specific autoimmune disease, then one might occasionally logically expect it to occur in individuals with other autoimmune diseases even lichen planus and lupus erythematosus?! Removing the name pseudopelade and much of the enigmatic literature of the last century, might at least lead to more logical research and perhaps eventually to specific therapy. References 1. Orfanos CK, Happlc R, tds. Hair and Itair Diseau-s, 1st edn. Kcr^m: Springur-t'erlag, J9W; p. (t2S. 2. Rook AJ, Dawber RPR, ciis. thseases of ihe Hair and ualp, 2nd edn. Oxford: Hlackwell Scientific Publicitions, 1991: p- 339.
3. Brocq I-. Alopecia. Journal of Cutaneous and Venereal Diseases 1885; 3: 49. 4. Brocq L. Pseudopelade. In: Traile f.lementaire de Dermatologie Fractique, Vol. 2. Parism-Doin Publishers, 1907: p. 648. 5. Photinos P. l.a pseudopelade de Brocq. Paris: Maloirc Publishers, 1930. 6. Degos R, Rabut R, Duperrat H, Leclercq R. Apropos de III cas d'alopecies cicatrkielles en petit aires (teignes exckies). L ' m t pseudopeladique. Bulletin de la Sociele Franfais DermatologieSyphilige 1951; 58: 451-452. 7. Degos R, Rabut R, Duperrat H, Leclercq R. L'ctat pscudopelacHque. Annales de Dermalologie et de Syphilitic 1954; 81, 5. 8. Degos R, Rabut R, Ilcwia J, Ktat pseudopeladique du a des metastases cacinomateuscs alopeciantes. Hiiltetui de la Societf Franfais Dermulologie-Syphiltge 1954; 61: 5(19 510. 9. Degos R, Rabut R, Duperrat B. Alopecie en pelil aires. Reticulose hmphocvrnire bcnigiic. Balkim de In S&aetc Franfi/i.'i Di'rmal«h/gie-SyphUtge 1955; 62: 134 135. 10. Laymon (^VV, Murphy RJ. The cicatricial alopecias. .7ww;'Wi// oJ Investigative Dermatology 1947; 8: 99 122. 11. Ludwig F. (1961) Uber die narbig-atrophischcn alopecien vom typ der pseudopelade de IJrocq. Berujs Dermatnsen 1961; 9: 257 270. 12. Pinkus H. Differential patterns of elastic fibres in scarrin|j,& nonscarring iiiopecias. Journal oj Culaiieous Faihotogy 1'I78; 5: 93. 13. Br^Hin-Falco O, Imci S, Schmoeckel C. Pseudopeiade oi Brocq, Dcrmalohfura 1986; 172: 18. 14. Pincclli C, Girolomoni G, Beiiassi L. Pscutlopelatlc of Brocq: an immunologically mediated disease? Dermalologica 1987; 176: 49. 15. Wilson CL, Burge SM, Dean H, Dawhcwr RPR, Wojnarowska F. A clinicopathological stud\ of scarring alopecia in chronic cutaneous lupus erythemaiosus. British Journal oj Dermatology 1990; 123: (Suppl. 37), 13. 16. Kaminsky A, Kaminskv C'\, dc Kaminsky AR, Ahukfia J. Liquen folicuhir akipeeiantc. Meduina Cutanea 1967; 2: 135. 17. Gray Prieto J. Pscudopelade of Brocq: its relationship to some forms of cicatricial alopecia and to lichen planus. Journal of InvcstifijiUve Dermalolugy 1955: 24: 322.
