ond, it is of special importance
to the because it may dermatologic surgeon herald eventual severe male pattern baldness and it makes very poor donor hair for hair transplant surgery. O'Tar T. Norwood, MD Oklahoma City
Papular Acrodermatitis Associated Hepatitis B Virus Infection
With
To the Editor.\p=m-\Castellano et al, in the October Archives (114:1530-1532, 1978), stated that "papular acrodermatitis associated with HBV [hepatitis B virus] infection has only been described in children." The authors failed to mention a report on papular acrodermatitis (PAC) occurring in three adults' and mimicking the eruption first described by Gianotti in children. The three patients had circulating hepatitis B surface antigen (HBsAg). The eruption was followed by a benign icteric hepatitis that lasted from 30 to 45 days. In two cases, HBsAg disappeared in one month, and in one case, the antigen had been present for more than three months. Direct immunofluorescence staining exhibited C3 deposits in the vessel walls of the dermis without any deposition of immunoglobulins or fibrinogen. The HBsAg and hepatitis e antigen (HBeAg) could not be identified in the skin lesions by fluoresceinconjugated specific antiserum. Serum protein concentration of complement components Clq, C4, C3, and C3PA were within normal limits as measured by radial immunodiffusion. No data supported the hypothesis that PAC may be an immune complex disease. Furthermore, the percent¬ ages of circulating B and T lympho¬
cytes
were normal, as assayed by sheep erythrocyte rosette-forming cells, erythrocyte antibody comple¬ ment rosette-forming cells, and sur¬ face immunoglobulins. Papular acrodermatitis in adults
does exist and
seems
prodrome hepatitis.
benign
to
a
to represent a form of viral
A. L. Claudy, MD J. P. Ortonne, MD C. Trepo, MD B. Bugnon, MD St Etienne, France
1. Claudy AL, Ortonne JP, Trepo C, et al: Acrodermatite papuleuse de l'adulte: A propos de trois cas. Ann Dermatol Venereol 104:190-194, 1977.
Reply.\p=m-\Claudyet al described three adult patients with acute hepatitis B and the presence of skin lesions
In
similar to those described by Gianotti. However, in the experience of two of us at the liver unit at the University of Southern California School of Medicine (M.J.T. and M.O.), we have not encountered this type of skin eruption in adults who were hospitalized for acute type B hepatitis. Also, PAC has not been observed in individuals with acute
type A
or
with
non-A, non-B
hepatitis. Therefore, although PAC
may be seen in adults with acute type B hepatitis, it must be extremely rare. One difference of interest is that the three patients described by Claudy et al were jaundiced, while virtually all of the children described in the literature, including our patient, were anicteric. Their finding of C3 deposits in the vessel walls suggests that activation of the alternative complement pathway, possibly by viral antigens or properdin, may be involved in the pathogenesis of this dermal lesion. Finally, it should be noted that progression to chronic active hepatitis has been reported in children who
initially manifested HBsAg-positive PAC,'2 and the presence of this skin lesion in adult patients with acute hepatitis B infection does not neces¬ sarily imply a benign course. Angela Castellano, MD Richard Schweitzer, MD Myron J. Tong, MD, PhD Masao Omata, MD Pasadena, Calif
1. DeGaspari G, Bardare M, Costantino D: Au antigen in Crosti-Gianotti acrodermatitis. Lan-
cet
1:1116-1117, 1970. 2. Columbo M, Gerber MA, Vernace SJ: Immune response to hepatitis B virus in children with papular acrodermatitis. Gastroenterology 73:1103-1106, 1977.
Skin Markers of X-Linked Dominant Chondrodysplasia Punctata To the Editor.\p=m-\Inthe October 1978 issue of the Archives (114:1479-1483, 1978), Dr Curth reconsidered her interesting case report of 1949' dealing with the association of follicular atrophoderma and chondrodysplasia punctata. With regard to cause, Dr Curth now suggests either an X-linked dominant gene that is lethal in hemizygous males or an autosomal dominant gene with sex-limited expression. The following arguments are advanced to show that the first possibility is more likely. The concept of X-linked dominant chondrodysplasia punctata was proposed because of two facts.2,3 First, some patients with chondrodysplasia punctata show widespread atrophic
Downloaded From: http://archderm.jamanetwork.com/ by a University of Iowa User on 06/17/2015
skin lesions and alopecia, and occasionally also pigmentary disturbances that may easily be confused with incontinentia pigmenti of the BlochSulzberger type. This pattern of skin lesions suggests functional X-chromosome mosaicism. Consequently, in involved areas the X chromosome carrying the mutant gene should be active, whereas in uninvolved skin the normal X chromosome should be the active one. Second, a review of the literature discloses that all patients observed so far are female. At the time of the first report by my colleagues and me,2 the female-tomale ratio was 10:0. At present the sex ratio is 19:0, with the inclusion of four new cases from the Department of Dermatology, University of Münster, West Germany. Both the limitation to the female sex and the mosaic pattern of skin lesions are explicable by postulating an X-linked dominant gene, whereas the assumption of an autosomal domi¬ nant gene with sex-limited expression would only explain the occurrence in women, but not the linear, whorled, or patchy distribution of skin lesions. The cutaneous signs and symptoms allow this new syndrome to be differ¬ entiated from two other types of chon¬ drodysplasia punctata, the rhizomelic type and the Conradi-Hünermann type. For example, the case described by Edidin et al4 should be classified as X-linked dominant chondroplasia punctata. The illustrations of their report show a characteristic linear pattern of hyperkeratotic skin lesions that were present during the first weeks of life. The observations of the family described by Curth1 are compatible with the assumption of an X-linked dominant gene. The woman with the condition had six children; two of her five daughters were affected and her only son was unaffected. The ratio of five females to one male could be due to the X-linked gene that would be present in 50% of male embryos and cause death in utero. However, if the concept of X-linked dominant chon¬ droplasia punctata holds true, it cannot be derived from analysis of this family alone. Rather, the primary evidence comes from the relationship between the mosaic phenotype and the limitation to the female sex. Rudolf Happle, MD Munster, West Germany 1. Curth HO: Follicular atrophoderma and pseudopelade associated with chondrodystrophia calcificans congenita. J Invest Dermatol 13:233\x=req-\
241,1949.
to the because it may dermatologic surgeon herald eventual severe male pattern baldness and it makes very poor donor hair for hair transplant surgery. O'Tar T. Norwood, MD Oklahoma City
Papular Acrodermatitis Associated Hepatitis B Virus Infection
With
To the Editor.\p=m-\Castellano et al, in the October Archives (114:1530-1532, 1978), stated that "papular acrodermatitis associated with HBV [hepatitis B virus] infection has only been described in children." The authors failed to mention a report on papular acrodermatitis (PAC) occurring in three adults' and mimicking the eruption first described by Gianotti in children. The three patients had circulating hepatitis B surface antigen (HBsAg). The eruption was followed by a benign icteric hepatitis that lasted from 30 to 45 days. In two cases, HBsAg disappeared in one month, and in one case, the antigen had been present for more than three months. Direct immunofluorescence staining exhibited C3 deposits in the vessel walls of the dermis without any deposition of immunoglobulins or fibrinogen. The HBsAg and hepatitis e antigen (HBeAg) could not be identified in the skin lesions by fluoresceinconjugated specific antiserum. Serum protein concentration of complement components Clq, C4, C3, and C3PA were within normal limits as measured by radial immunodiffusion. No data supported the hypothesis that PAC may be an immune complex disease. Furthermore, the percent¬ ages of circulating B and T lympho¬
cytes
were normal, as assayed by sheep erythrocyte rosette-forming cells, erythrocyte antibody comple¬ ment rosette-forming cells, and sur¬ face immunoglobulins. Papular acrodermatitis in adults
does exist and
seems
prodrome hepatitis.
benign
to
a
to represent a form of viral
A. L. Claudy, MD J. P. Ortonne, MD C. Trepo, MD B. Bugnon, MD St Etienne, France
1. Claudy AL, Ortonne JP, Trepo C, et al: Acrodermatite papuleuse de l'adulte: A propos de trois cas. Ann Dermatol Venereol 104:190-194, 1977.
Reply.\p=m-\Claudyet al described three adult patients with acute hepatitis B and the presence of skin lesions
In
similar to those described by Gianotti. However, in the experience of two of us at the liver unit at the University of Southern California School of Medicine (M.J.T. and M.O.), we have not encountered this type of skin eruption in adults who were hospitalized for acute type B hepatitis. Also, PAC has not been observed in individuals with acute
type A
or
with
non-A, non-B
hepatitis. Therefore, although PAC
may be seen in adults with acute type B hepatitis, it must be extremely rare. One difference of interest is that the three patients described by Claudy et al were jaundiced, while virtually all of the children described in the literature, including our patient, were anicteric. Their finding of C3 deposits in the vessel walls suggests that activation of the alternative complement pathway, possibly by viral antigens or properdin, may be involved in the pathogenesis of this dermal lesion. Finally, it should be noted that progression to chronic active hepatitis has been reported in children who
initially manifested HBsAg-positive PAC,'2 and the presence of this skin lesion in adult patients with acute hepatitis B infection does not neces¬ sarily imply a benign course. Angela Castellano, MD Richard Schweitzer, MD Myron J. Tong, MD, PhD Masao Omata, MD Pasadena, Calif
1. DeGaspari G, Bardare M, Costantino D: Au antigen in Crosti-Gianotti acrodermatitis. Lan-
cet
1:1116-1117, 1970. 2. Columbo M, Gerber MA, Vernace SJ: Immune response to hepatitis B virus in children with papular acrodermatitis. Gastroenterology 73:1103-1106, 1977.
Skin Markers of X-Linked Dominant Chondrodysplasia Punctata To the Editor.\p=m-\Inthe October 1978 issue of the Archives (114:1479-1483, 1978), Dr Curth reconsidered her interesting case report of 1949' dealing with the association of follicular atrophoderma and chondrodysplasia punctata. With regard to cause, Dr Curth now suggests either an X-linked dominant gene that is lethal in hemizygous males or an autosomal dominant gene with sex-limited expression. The following arguments are advanced to show that the first possibility is more likely. The concept of X-linked dominant chondrodysplasia punctata was proposed because of two facts.2,3 First, some patients with chondrodysplasia punctata show widespread atrophic
Downloaded From: http://archderm.jamanetwork.com/ by a University of Iowa User on 06/17/2015
skin lesions and alopecia, and occasionally also pigmentary disturbances that may easily be confused with incontinentia pigmenti of the BlochSulzberger type. This pattern of skin lesions suggests functional X-chromosome mosaicism. Consequently, in involved areas the X chromosome carrying the mutant gene should be active, whereas in uninvolved skin the normal X chromosome should be the active one. Second, a review of the literature discloses that all patients observed so far are female. At the time of the first report by my colleagues and me,2 the female-tomale ratio was 10:0. At present the sex ratio is 19:0, with the inclusion of four new cases from the Department of Dermatology, University of Münster, West Germany. Both the limitation to the female sex and the mosaic pattern of skin lesions are explicable by postulating an X-linked dominant gene, whereas the assumption of an autosomal domi¬ nant gene with sex-limited expression would only explain the occurrence in women, but not the linear, whorled, or patchy distribution of skin lesions. The cutaneous signs and symptoms allow this new syndrome to be differ¬ entiated from two other types of chon¬ drodysplasia punctata, the rhizomelic type and the Conradi-Hünermann type. For example, the case described by Edidin et al4 should be classified as X-linked dominant chondroplasia punctata. The illustrations of their report show a characteristic linear pattern of hyperkeratotic skin lesions that were present during the first weeks of life. The observations of the family described by Curth1 are compatible with the assumption of an X-linked dominant gene. The woman with the condition had six children; two of her five daughters were affected and her only son was unaffected. The ratio of five females to one male could be due to the X-linked gene that would be present in 50% of male embryos and cause death in utero. However, if the concept of X-linked dominant chon¬ droplasia punctata holds true, it cannot be derived from analysis of this family alone. Rather, the primary evidence comes from the relationship between the mosaic phenotype and the limitation to the female sex. Rudolf Happle, MD Munster, West Germany 1. Curth HO: Follicular atrophoderma and pseudopelade associated with chondrodystrophia calcificans congenita. J Invest Dermatol 13:233\x=req-\
241,1949.
