Report
ERYTHEMA MULTIEORME INFANTUM ATROPHICANS R. D. AZULAY, M.D., L. p. QUEVEDO, M.D., > R. DAL BELLO GIFUEIRA, M.D., AND L. E. MANHAES, M.D. From the Postgraduation Course of Antonio Pedro University Hospital, State ol Rio de Janeiro, Brasil.
Dermatology, Niteroi,
taneous elastolysis (cutis laxa), circumscribed cutaneous elastolysis and cystenic elastolysis. The last is an open category to cover the possibility that elastolysis may not always involve the ABSTRACT: A new case of erythema mul- skin. tiforme is described, characterized by mulIn addition, Marshall et al. called attentiforme eruption of erythematous papules, plaques and occasional bullous lesions. The tion to a new condition based on their acute stage lasts several weeks, then atrophy observation of 5 cases "in which elastois noted. The face appears senile. Histolysis and cutis laxa followed a chronic pathologically, the lesions show erythema annular erythematous eruption." multiforme in the acute phase; in the Ramos e Silva"* has proposed the name atrophic stage, a disappearance of the elastic James Marshall's disease for this condifibers was observed. tion. In 1964 Azulay and Figueiras' described We wish to describe another patient with this condition. the case of an 8-year-old girl with inflammatory papules and nodules, which had atrophied. The child had a senile Case Report facies. The condition was classified as A 16-month-old girl presented with red atrophodermia affecting extensive areas. patches, bullous lesions, pruritus and fever. Tv\'o years later Marshall et al.^ pubLaboratory findings are listed in Table 2. She was treated with antibiotics and corticolished a report entitled "Postinflammasteroids. The acute stage lasted for several tory Elastolysis and Cutis Laxa." This was weeks. The lesions eventually atrophied, a report of a newly discovered variety developing the characteristics of cutis lata. of the previously described condition. After a year's observation, her face had a In a discussion of some syndromes charsenile appearance. acterized by elastolysis, they presented Histopathologically in the acute phase, there was mild acanthosis and discrete inthe classification shown in Table 1. Their tercellular edema. The dermis showed inthree main types were generalized cutense polymorphonuclear infiltration, mainly around the vessels, and mild vasculitis. In Presented at the XIV International Gongress of the atrophic stage, the only change is the Dermatology, May 22-27, 1972, Venice, Italy. absence of elastic tissue in certain areas of Financial support of GOMPEG, UFF. the dermis.
56
No.
1
ERYTHEMA MULTIFORME INFANTUM ATROPHIGANS • Azulay, et al.
57
Fig. 1—Lesions characteristic multiforme in the acute phase.
Discussion In the clinical conditions called cutis laxa and anetoderma there is a basic histopathologic alteration called "elastolysis," which is represented by destruction and disappearance of the elastic fibers. Marshall used the term elastoiysis with a clinical meaning. Our case may be included in Marshall's classification as a Table 1.
Fig. 2—Lesions in the acute phase.
Marshall's Classification of Elastolysis
Generalized cutaneous elastolysis (cutis laxa) Gongenital ' Apparently restricted to skin With systemic lesions
•
Acquired Insidious onset apparently restricted to skin Postinflammatory systemic lesions Gircumscribed cutaneous elastolysis Blepharochalasis Anetoderma (cryptogenic) Systemic elastolysis
'
"
'
•
Fig. 3—Atropl-iic residual lesions (senile) appearance.
"postinflammatory acquired generalized cutaneous elastolysis." Nine other patients are known thus far. The one of Ramos e Silva,'* the 5 cases of Marshall, one of Azulay or Filgueiras (1964), one of Ramos e Silva (1958-1971), one of Jablonska' and the case of Reed
INTERNATIONAL JOURNAL OF DERMATOLOGY Jan./Feb. 1975
58 Table 2.
Comparison of Laboratory Findings in Acute and Atrophic Stages
Atrophic
Acute Oct 2, 1970
Jan. 6, 1971
RBG
3.3
3.7
Hgb
7.23
9.52
Hct
26
33
WBG
16.3
20.0
Segs Bands
22 3
59 14
Eosin
54
Metany
Lymph
21
0 19
0
3
Mon Platelets
Normal
Normal
et al.'' All have several interesting points in common: the condition started in childhood as erythema multiforme exudativum and atrophied, giving the appearance of cutis laxa and a senile facies, without systemic lesions.
Vol. 14
We suggest that this condition is typical erythema exudative multiforme which atrophies due to a special enzymatic defect. We believe that the most descriptive name is erythema multiforme infantum atrophicans. References 1. Azulay, R. D., and Filgueiras, A. L., Gaso de Atrophoderma em grandes placas. An. Bras. Dermatol. 39:69, 1964. 2. Marshall, L., Heyl, T., and Weber, H. W., Postinflammatory Elastolysis and Gutis Laxa. S. Afr. Med. J. 40:1016, 1966. 3. Ramos e Silva, J., Eritema multiforme infantum sui generis. Presented to Sociedade Brasileira de Dermatologia, Meeting of March 31st, 1971. 4. Ramos e Silva, J., Erythema multiforme infantum sui generis seguido de alastolise e de cutis laxa, com blefarocalase. Dermatologia Ibero-Lat. Am. 14:39, 1972. 5. Jablonska, St., Inflammatorische Hautver anderung, die einer erworbener Gutis Laxa vorausgehen. Der Hautarzt 17:341, 1966. 6. Reed, W. B., Horowitz, R. E., and Beighton, P., Acquired cutis laxa. Arch. Dermatol. 103: 661, 1971.
Notes from Malcolm Morris. Antimony in Skin Diseases: Mr. Morns is a strong advocate of the use of Antimony. According to his claim it subdues irritation, pain and inflammation in the skin, and checks weeping to a remarkable degree. He therefore advises it in acute inflammatory conditions of the skin, especially in acute weeping eczemas. He gives it in the form of wine of antimony 2-15 qtt. t.i.d. or oftener.--Recordec/ by William Allen Pusey in his notebook from London 1892 and deposited in the American Academy of Dermatology Collection at the Library of the College of Physicians of Philadelphia.
ERYTHEMA MULTIEORME INFANTUM ATROPHICANS R. D. AZULAY, M.D., L. p. QUEVEDO, M.D., > R. DAL BELLO GIFUEIRA, M.D., AND L. E. MANHAES, M.D. From the Postgraduation Course of Antonio Pedro University Hospital, State ol Rio de Janeiro, Brasil.
Dermatology, Niteroi,
taneous elastolysis (cutis laxa), circumscribed cutaneous elastolysis and cystenic elastolysis. The last is an open category to cover the possibility that elastolysis may not always involve the ABSTRACT: A new case of erythema mul- skin. tiforme is described, characterized by mulIn addition, Marshall et al. called attentiforme eruption of erythematous papules, plaques and occasional bullous lesions. The tion to a new condition based on their acute stage lasts several weeks, then atrophy observation of 5 cases "in which elastois noted. The face appears senile. Histolysis and cutis laxa followed a chronic pathologically, the lesions show erythema annular erythematous eruption." multiforme in the acute phase; in the Ramos e Silva"* has proposed the name atrophic stage, a disappearance of the elastic James Marshall's disease for this condifibers was observed. tion. In 1964 Azulay and Figueiras' described We wish to describe another patient with this condition. the case of an 8-year-old girl with inflammatory papules and nodules, which had atrophied. The child had a senile Case Report facies. The condition was classified as A 16-month-old girl presented with red atrophodermia affecting extensive areas. patches, bullous lesions, pruritus and fever. Tv\'o years later Marshall et al.^ pubLaboratory findings are listed in Table 2. She was treated with antibiotics and corticolished a report entitled "Postinflammasteroids. The acute stage lasted for several tory Elastolysis and Cutis Laxa." This was weeks. The lesions eventually atrophied, a report of a newly discovered variety developing the characteristics of cutis lata. of the previously described condition. After a year's observation, her face had a In a discussion of some syndromes charsenile appearance. acterized by elastolysis, they presented Histopathologically in the acute phase, there was mild acanthosis and discrete inthe classification shown in Table 1. Their tercellular edema. The dermis showed inthree main types were generalized cutense polymorphonuclear infiltration, mainly around the vessels, and mild vasculitis. In Presented at the XIV International Gongress of the atrophic stage, the only change is the Dermatology, May 22-27, 1972, Venice, Italy. absence of elastic tissue in certain areas of Financial support of GOMPEG, UFF. the dermis.
56
No.
1
ERYTHEMA MULTIFORME INFANTUM ATROPHIGANS • Azulay, et al.
57
Fig. 1—Lesions characteristic multiforme in the acute phase.
Discussion In the clinical conditions called cutis laxa and anetoderma there is a basic histopathologic alteration called "elastolysis," which is represented by destruction and disappearance of the elastic fibers. Marshall used the term elastoiysis with a clinical meaning. Our case may be included in Marshall's classification as a Table 1.
Fig. 2—Lesions in the acute phase.
Marshall's Classification of Elastolysis
Generalized cutaneous elastolysis (cutis laxa) Gongenital ' Apparently restricted to skin With systemic lesions
•
Acquired Insidious onset apparently restricted to skin Postinflammatory systemic lesions Gircumscribed cutaneous elastolysis Blepharochalasis Anetoderma (cryptogenic) Systemic elastolysis
'
"
'
•
Fig. 3—Atropl-iic residual lesions (senile) appearance.
"postinflammatory acquired generalized cutaneous elastolysis." Nine other patients are known thus far. The one of Ramos e Silva,'* the 5 cases of Marshall, one of Azulay or Filgueiras (1964), one of Ramos e Silva (1958-1971), one of Jablonska' and the case of Reed
INTERNATIONAL JOURNAL OF DERMATOLOGY Jan./Feb. 1975
58 Table 2.
Comparison of Laboratory Findings in Acute and Atrophic Stages
Atrophic
Acute Oct 2, 1970
Jan. 6, 1971
RBG
3.3
3.7
Hgb
7.23
9.52
Hct
26
33
WBG
16.3
20.0
Segs Bands
22 3
59 14
Eosin
54
Metany
Lymph
21
0 19
0
3
Mon Platelets
Normal
Normal
et al.'' All have several interesting points in common: the condition started in childhood as erythema multiforme exudativum and atrophied, giving the appearance of cutis laxa and a senile facies, without systemic lesions.
Vol. 14
We suggest that this condition is typical erythema exudative multiforme which atrophies due to a special enzymatic defect. We believe that the most descriptive name is erythema multiforme infantum atrophicans. References 1. Azulay, R. D., and Filgueiras, A. L., Gaso de Atrophoderma em grandes placas. An. Bras. Dermatol. 39:69, 1964. 2. Marshall, L., Heyl, T., and Weber, H. W., Postinflammatory Elastolysis and Gutis Laxa. S. Afr. Med. J. 40:1016, 1966. 3. Ramos e Silva, J., Eritema multiforme infantum sui generis. Presented to Sociedade Brasileira de Dermatologia, Meeting of March 31st, 1971. 4. Ramos e Silva, J., Erythema multiforme infantum sui generis seguido de alastolise e de cutis laxa, com blefarocalase. Dermatologia Ibero-Lat. Am. 14:39, 1972. 5. Jablonska, St., Inflammatorische Hautver anderung, die einer erworbener Gutis Laxa vorausgehen. Der Hautarzt 17:341, 1966. 6. Reed, W. B., Horowitz, R. E., and Beighton, P., Acquired cutis laxa. Arch. Dermatol. 103: 661, 1971.
Notes from Malcolm Morris. Antimony in Skin Diseases: Mr. Morns is a strong advocate of the use of Antimony. According to his claim it subdues irritation, pain and inflammation in the skin, and checks weeping to a remarkable degree. He therefore advises it in acute inflammatory conditions of the skin, especially in acute weeping eczemas. He gives it in the form of wine of antimony 2-15 qtt. t.i.d. or oftener.--Recordec/ by William Allen Pusey in his notebook from London 1892 and deposited in the American Academy of Dermatology Collection at the Library of the College of Physicians of Philadelphia.
