- Email: [email protected]
Degos' disease is probably a distinct entity: A review of clinical and laboratory evidence
Letters 375
J AM ACAD DERMATOL VOLUME 52, NUMBER 2
cycle. In our view, the resting state of telogen does not, and is often mistakenly imputed to, imply shedding. A graphic example is the fur coat of a weasel, whose follicles experience a prolonged telogen over the winter months. Only many months after the initiation of telogen, in spring, do those follicles receive a unique signal which initiates the shedding process. Moreover, in a recently described mouse model to study exogen it was found that different pelage follicles in telogen shed at different times.3 Moreover, the exogen process cannot simply be a passive event for two reasons: first, the cell structure of the telogen follicle separates the silo of the new anagen shaft from the silo of the resting telogen shafts (isolating shafts of different growth phases),3 and second, prominent shedding does not occur in the trichostatic conditions even though there is extensive dynamic outward growth of the resident shafts. The signals and events of exogen are distinctive and separate from the important stable, structure, and function of telogen. Kurt Stenn, MD Aderans Research Institute, Inc Philadelphia, Pennsylvania REFERENCES 1. Rebora A. Pathogenesis of androgenetic alopecia. J Am Acad Dermatol 2004;50:777-9. 2. Stenn K, Parimoo S, Prouty S. Growth of the Hair Follicle, a Cycling and Regenerating Biological System. In: CM Chuong, editors. Molecular Basis of Epithelial Appendage Morphogenesis. Austin (TX), RG Landes Co, 1998. p. 111-30. 3. Milner Y, Sudnik J, Filippi M, Kashgarian M, Stenn KS. The shedding phase of the hair growth cycle, exogen, is coupled to anagen and generates a unique shaft base. J Invest Dermatol 2002;119:639-44. doi:10.1016/j.jaad.2004.07.040
Linear lichen planus of the face and neck versus amalgam-induced ‘‘isotopic’’ cutaneous lichen planus To the Editor: In the April 2004 issue of the Journal, Al-Mutairi et al1 reported a case of ‘‘isotopic cutaneous lichen planus possibly related to dental amalgam.’’ The authors assumed a causal relationship with an amalgam filling of a molar tooth as well as with a preceding herpes zoster localized in the same region of the face and neck. Such a relationship is hardly possible, for the following reasons. The arrangement of lesions is not ‘‘zosteriform’’ but follows the lines of Blaschko.2,3 In Fig 1, three bands run from the submental region to the lower lip, and in Fig 2, one band runs from the
earlobe along the mandibula to the lower lip, whereas another streak runs in a caudal direction.2 Hence, I propose to categorize this case as a typical example of linear lichen planus of the head and neck showing a unilateral, systematized involvement. For obvious reasons, this disorder cannot be interpreted as an isotopic response to a preceding herpes zoster, nor as a sequela of an amalgam filling. Besides, a positive reaction to patch testing with HgCl2 does not provide proof that amalgam exerted any noxious effect in this patient. If the authors would have performed a patch test with amalgam, the reaction would have certainly been negative.4 Rudolf Happle, MD Department of Dermatology Philipp University Marburg, Germany E-mail: [email protected] REFERENCES 1. Al-Mutairi N, Sharma AK, Osama NE, Joshi A, Ayman H. Isotopic cutaneous lichen planus possibly related to dental amalgam. J Am Acad Dermatol 2004;50:653-4. 2. Happle R, Assim A. The lines of Blaschko on the head and neck. J Am Acad Dermatol 2001;44:612-5. 3. Happle R. ‘‘Zosteriform’’ lichen planus: the bizarre consequences of a misnomer. Acta Derm Venereol 1998;78:300. 4. Kanerva L, Rantanen T, Aalto-Korte K, Estlander T, Hannuksela M, Harvima RJ, et al. A multicenter study of patch test reactions with dental screening series. Am J Contact Dermat 2001;12: 83-7. doi:10.1016/j.jaad.2004.07.039
Degos’ disease is probably a distinct entity: A review of clinical and laboratory evidence To the Editor: I read with interest the article about Degos’ disease (DD) by High et al1 in the June 2004 issue of the Journal and agree that (1) DD likely involves vascular insult and that (2) white atrophic papules are not actually pathognomonic for DD and can be present in a variety of conditions, including lupus. Histology alone, however, cannot define a disease as a non-specific entity. Clinical and laboratory evidence suggest that systemic DD is probably a distinct entity and, at the very least, not a collagen vascular disease.2 The eruption of lupus frequently affects the face, and photosensitivity is a hallmark of collagen vascular disease and lupus specifically.3 Photosensitivity is not associated with DD; moreover, its eruptions rarely, if ever, occur on the face.2 The white atrophic papules that are often referred to as pathognemonic are best described as a sensitive (rather than specific) indication of DD.
376 Letters
J AM ACAD DERMATOL FEBRUARY 2005
In lupus, the gastrointestinal tract is affected but is not a defining disease criteria.3 In systemic DD, the gastrointestinal tract is affected in 50% of cases and intestinal perforation is the most severe complication and cause of death. DD can have peristomal lesions and fistulae, neither of which are mentioned in a Medline search of these terms and lupus.2 Whereas in lupus skin biopsy specimens examined by direct immunofluorescence (DIF) usually demonstrate positive, reproducible results, DIF in DD does not show any particular immunofluorescence pattern. Its biopsies show wedge-shaped necrosis, a finding not commonly described in the biopsies of lupus or other diseases. DD is invariably fatal, usually within 2 to 3 years from the onset of systemic involvement. However, the range of survival time from the time of diagnosis varies from less than 1 year to more than 12 years. Patients with collagen vascular disease and other diseases have more variable survival courses. No specific laboratory test defines DD. Most laboratory tests are normal, with the exception of anemia secondary to intestinal bleeding. Collagen vascular disease is strongly associated with autoantibodies, and most diseases manifest with characteristic laboratory abnormalities. While DD can overlap with antiphospholipid syndrome, it usually does not. In DD, virus-like inclusions are frequently present in endothelial cells and fibroblasts; these are not commonly found in other diseases. This point is mentioned by High et al and deserves careful note. No successful medical therapy for systemic DD exists, which itself separates DD from other diseases. Antiplatelet drugs may reduce the number of new skin papules, but they do not abate systemic disease. Many treatments have been tried without real benefit. Medical therapies will at least usually abate some of the symptoms of lupus and other diseases. In sum, systemic DD is not a collagen vascular disease, and is probably a distinct entity characterized by: (1) an unresponsiveness to therapy; (2) the frequent presence of cellular virus-like inclusions; (3) negative DIF findings; (4) invariable fatal course usually in 2 to 3 years from diagnosis; (5) a lack of photosensitivity; and (6) a lack of facial lesions. White atrophic papules and their histology are a sensitive rather than specific indication of DD. Noah Scheinfeld, MD Department of Dermatology St. Luke’s Roosevelt Hospital Center New York, New York E-mail: [email protected]
REFERENCES 1. High WA, Aranda J, Patel SB, Cockerell CJ, Costner MI. Is Degos’ disease a clinical and histological end point rather than a specific disease? J Am Acad Dermatol 2004;50:895-9. 2. Scheinfeld N. Degos’ disease. In: Emedicine Dermatology Book. Available at: http://www.emedicine.com/derm/topic931.htm. Accessed June 12, 2004. 3. Hildebrand J, Muller D. Systemic lupus erythematosus. In: Emedicine Medicine Book. Available at: http://www. emedicine.com/MED/topic2228.htm. Accessed June 12, 2004. doi:10.1016/j.jaad.2004.06.049
Reply To the Editor: We are pleased to have received correspondence regarding our paper1 which detailed the non-specific clinical and histological findings of Degos’ disease (DD) and alluded to the possibility that this disease may represent, in many cases, a reaction pattern. It is gratifying to learn that our paper has stimulated discussion on the matter. Admittedly, we cannot be certain of the existence of DD as a distinct entity. A plethora of contradictory evidence regarding DD exists. Not only was this summarized within our article, but it has been detailed in the work of other investigators as cited in our paper. Ball et al2 noted also that lupus may in fact represent the most common etiology for such a clinicopathologic constellation of findings. As Dr Scheinfeld notes, there is nothing ‘‘pathognomonic’’ about the clinical presentation of the cutaneous lesions ascribed to DD. While the palms, soles, face, scalp, and genitalia tend to be spared, exceptions have been noted.3-7 Dr Scheinfeld’s observation regarding photosensitivity is indeed a valid concern. One must consider, however, that while many forms of autoimmune connective tissue disease demonstrate photosensitivity, other forms, such as scleroderma, autoimmune vasculitis, or primary antiphospholipid antibody syndrome, do not.8 Additionally, while discoid lupus erythematosus most commonly occurs on photoexposed sites, it may also involve photoprotected areas, such as the concha of the ears, the palms, and the soles of the feet. Similarly, lupus panniculitis, with or without overlying epidermal or dermal change, is certainly an accepted variant of lupus. Up to 70% of lupus panniculitis patients may demonstrate a positive antinuclear antibody (ANA), and a significant number may develop systemic disease.9 With a proclivity for the buttocks, breasts, or upper thighs, lupus panniculitis would not be considered photodistributed.10 While we cannot answer directly these observed incongruities, it is
J AM ACAD DERMATOL VOLUME 52, NUMBER 2
cycle. In our view, the resting state of telogen does not, and is often mistakenly imputed to, imply shedding. A graphic example is the fur coat of a weasel, whose follicles experience a prolonged telogen over the winter months. Only many months after the initiation of telogen, in spring, do those follicles receive a unique signal which initiates the shedding process. Moreover, in a recently described mouse model to study exogen it was found that different pelage follicles in telogen shed at different times.3 Moreover, the exogen process cannot simply be a passive event for two reasons: first, the cell structure of the telogen follicle separates the silo of the new anagen shaft from the silo of the resting telogen shafts (isolating shafts of different growth phases),3 and second, prominent shedding does not occur in the trichostatic conditions even though there is extensive dynamic outward growth of the resident shafts. The signals and events of exogen are distinctive and separate from the important stable, structure, and function of telogen. Kurt Stenn, MD Aderans Research Institute, Inc Philadelphia, Pennsylvania REFERENCES 1. Rebora A. Pathogenesis of androgenetic alopecia. J Am Acad Dermatol 2004;50:777-9. 2. Stenn K, Parimoo S, Prouty S. Growth of the Hair Follicle, a Cycling and Regenerating Biological System. In: CM Chuong, editors. Molecular Basis of Epithelial Appendage Morphogenesis. Austin (TX), RG Landes Co, 1998. p. 111-30. 3. Milner Y, Sudnik J, Filippi M, Kashgarian M, Stenn KS. The shedding phase of the hair growth cycle, exogen, is coupled to anagen and generates a unique shaft base. J Invest Dermatol 2002;119:639-44. doi:10.1016/j.jaad.2004.07.040
Linear lichen planus of the face and neck versus amalgam-induced ‘‘isotopic’’ cutaneous lichen planus To the Editor: In the April 2004 issue of the Journal, Al-Mutairi et al1 reported a case of ‘‘isotopic cutaneous lichen planus possibly related to dental amalgam.’’ The authors assumed a causal relationship with an amalgam filling of a molar tooth as well as with a preceding herpes zoster localized in the same region of the face and neck. Such a relationship is hardly possible, for the following reasons. The arrangement of lesions is not ‘‘zosteriform’’ but follows the lines of Blaschko.2,3 In Fig 1, three bands run from the submental region to the lower lip, and in Fig 2, one band runs from the
earlobe along the mandibula to the lower lip, whereas another streak runs in a caudal direction.2 Hence, I propose to categorize this case as a typical example of linear lichen planus of the head and neck showing a unilateral, systematized involvement. For obvious reasons, this disorder cannot be interpreted as an isotopic response to a preceding herpes zoster, nor as a sequela of an amalgam filling. Besides, a positive reaction to patch testing with HgCl2 does not provide proof that amalgam exerted any noxious effect in this patient. If the authors would have performed a patch test with amalgam, the reaction would have certainly been negative.4 Rudolf Happle, MD Department of Dermatology Philipp University Marburg, Germany E-mail: [email protected] REFERENCES 1. Al-Mutairi N, Sharma AK, Osama NE, Joshi A, Ayman H. Isotopic cutaneous lichen planus possibly related to dental amalgam. J Am Acad Dermatol 2004;50:653-4. 2. Happle R, Assim A. The lines of Blaschko on the head and neck. J Am Acad Dermatol 2001;44:612-5. 3. Happle R. ‘‘Zosteriform’’ lichen planus: the bizarre consequences of a misnomer. Acta Derm Venereol 1998;78:300. 4. Kanerva L, Rantanen T, Aalto-Korte K, Estlander T, Hannuksela M, Harvima RJ, et al. A multicenter study of patch test reactions with dental screening series. Am J Contact Dermat 2001;12: 83-7. doi:10.1016/j.jaad.2004.07.039
Degos’ disease is probably a distinct entity: A review of clinical and laboratory evidence To the Editor: I read with interest the article about Degos’ disease (DD) by High et al1 in the June 2004 issue of the Journal and agree that (1) DD likely involves vascular insult and that (2) white atrophic papules are not actually pathognomonic for DD and can be present in a variety of conditions, including lupus. Histology alone, however, cannot define a disease as a non-specific entity. Clinical and laboratory evidence suggest that systemic DD is probably a distinct entity and, at the very least, not a collagen vascular disease.2 The eruption of lupus frequently affects the face, and photosensitivity is a hallmark of collagen vascular disease and lupus specifically.3 Photosensitivity is not associated with DD; moreover, its eruptions rarely, if ever, occur on the face.2 The white atrophic papules that are often referred to as pathognemonic are best described as a sensitive (rather than specific) indication of DD.
376 Letters
J AM ACAD DERMATOL FEBRUARY 2005
In lupus, the gastrointestinal tract is affected but is not a defining disease criteria.3 In systemic DD, the gastrointestinal tract is affected in 50% of cases and intestinal perforation is the most severe complication and cause of death. DD can have peristomal lesions and fistulae, neither of which are mentioned in a Medline search of these terms and lupus.2 Whereas in lupus skin biopsy specimens examined by direct immunofluorescence (DIF) usually demonstrate positive, reproducible results, DIF in DD does not show any particular immunofluorescence pattern. Its biopsies show wedge-shaped necrosis, a finding not commonly described in the biopsies of lupus or other diseases. DD is invariably fatal, usually within 2 to 3 years from the onset of systemic involvement. However, the range of survival time from the time of diagnosis varies from less than 1 year to more than 12 years. Patients with collagen vascular disease and other diseases have more variable survival courses. No specific laboratory test defines DD. Most laboratory tests are normal, with the exception of anemia secondary to intestinal bleeding. Collagen vascular disease is strongly associated with autoantibodies, and most diseases manifest with characteristic laboratory abnormalities. While DD can overlap with antiphospholipid syndrome, it usually does not. In DD, virus-like inclusions are frequently present in endothelial cells and fibroblasts; these are not commonly found in other diseases. This point is mentioned by High et al and deserves careful note. No successful medical therapy for systemic DD exists, which itself separates DD from other diseases. Antiplatelet drugs may reduce the number of new skin papules, but they do not abate systemic disease. Many treatments have been tried without real benefit. Medical therapies will at least usually abate some of the symptoms of lupus and other diseases. In sum, systemic DD is not a collagen vascular disease, and is probably a distinct entity characterized by: (1) an unresponsiveness to therapy; (2) the frequent presence of cellular virus-like inclusions; (3) negative DIF findings; (4) invariable fatal course usually in 2 to 3 years from diagnosis; (5) a lack of photosensitivity; and (6) a lack of facial lesions. White atrophic papules and their histology are a sensitive rather than specific indication of DD. Noah Scheinfeld, MD Department of Dermatology St. Luke’s Roosevelt Hospital Center New York, New York E-mail: [email protected]
REFERENCES 1. High WA, Aranda J, Patel SB, Cockerell CJ, Costner MI. Is Degos’ disease a clinical and histological end point rather than a specific disease? J Am Acad Dermatol 2004;50:895-9. 2. Scheinfeld N. Degos’ disease. In: Emedicine Dermatology Book. Available at: http://www.emedicine.com/derm/topic931.htm. Accessed June 12, 2004. 3. Hildebrand J, Muller D. Systemic lupus erythematosus. In: Emedicine Medicine Book. Available at: http://www. emedicine.com/MED/topic2228.htm. Accessed June 12, 2004. doi:10.1016/j.jaad.2004.06.049
Reply To the Editor: We are pleased to have received correspondence regarding our paper1 which detailed the non-specific clinical and histological findings of Degos’ disease (DD) and alluded to the possibility that this disease may represent, in many cases, a reaction pattern. It is gratifying to learn that our paper has stimulated discussion on the matter. Admittedly, we cannot be certain of the existence of DD as a distinct entity. A plethora of contradictory evidence regarding DD exists. Not only was this summarized within our article, but it has been detailed in the work of other investigators as cited in our paper. Ball et al2 noted also that lupus may in fact represent the most common etiology for such a clinicopathologic constellation of findings. As Dr Scheinfeld notes, there is nothing ‘‘pathognomonic’’ about the clinical presentation of the cutaneous lesions ascribed to DD. While the palms, soles, face, scalp, and genitalia tend to be spared, exceptions have been noted.3-7 Dr Scheinfeld’s observation regarding photosensitivity is indeed a valid concern. One must consider, however, that while many forms of autoimmune connective tissue disease demonstrate photosensitivity, other forms, such as scleroderma, autoimmune vasculitis, or primary antiphospholipid antibody syndrome, do not.8 Additionally, while discoid lupus erythematosus most commonly occurs on photoexposed sites, it may also involve photoprotected areas, such as the concha of the ears, the palms, and the soles of the feet. Similarly, lupus panniculitis, with or without overlying epidermal or dermal change, is certainly an accepted variant of lupus. Up to 70% of lupus panniculitis patients may demonstrate a positive antinuclear antibody (ANA), and a significant number may develop systemic disease.9 With a proclivity for the buttocks, breasts, or upper thighs, lupus panniculitis would not be considered photodistributed.10 While we cannot answer directly these observed incongruities, it is