1
506 Correspondence Reply To the Editor: We thank Drs. Trileb, Bruckner-Tuder- man, and Burg for their report of a patient withthe EEC syndrome and scalp dermatitis. In our article we stated that scalp dermatitis can be seen in patients with the Rapp-Hodgkin,Bowen-Armstrong,andAECsyndromes. Thisstatement wasbasedonan analysisofcasesreported in the literature. We also noted that patients with the ABC syndrome were 6.6 times more likely to have scalp dermatitis than patients with the Rapp-Hodgkin syn- drome.With inclusionofthecasereportedbyTriiebetal., patients with the AEC syndrome would also be more likelyto havescalp dermatitisthan patients withthe EEC syndrome (7 of 12 vs 1 of 148). Scott w: Fosko , MIJP Kurt S. Stenn, MIY Jean L. Bolognia, MY Department of Dermatology, University of Pennsylvania School of Medicine, Philadelphia" Skin Biology Unit, R. W. Johnson Pharmaceutical Research Institute, Raritan, New Jersey' Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut" The spectrum of dermal elastolysis To the Editor: We haveread with great interest the clin- ical casesofdermal elastolysis reported by Brodet al. 1and by Betti et al. 2 We recently reported a caseof mid-der- mal elastolysis' and wewant to defineprecisely the clini- copathologicfeatures of the differentvarieties of elastol- ysis. Mid-dermal elastolysis was first reported by Shelley and Wood" as an acquired, noninflammatory disease in adults, witha pathognomonic histologic pattern: bandlike mid-dermal elastolysis without any other morphologic lesion. The case of Betti et aP has the same clinical pattern as type II mid-dermal elastolysis reported by Brenner et al. s with papular lesions and perifollicular protrusions. Histologically, elastic fibers were absent in the papillary dermis and focally in mid dermis, without a mid-dermal bandlike pattern . Instead of a clinical pattern similar to mid-dermal elastol ysis , Betti et al. described the case as noninflammatory dermal elastolysis. We agree with them and postulate that there are two groups of elastolysis. The first is represented by nonin- flammatoryelastolysis as in the caseof Betti et al. and the cases of typical mid-dermal elastolysis first reported by Shelley and Wood 4 and by other authors more recently. The second group is represented by postinf'lammatory Journal of the American Academy of Dermatology September 1993 elastolysis. Some cases of postinfiammatory elastolysis are associated with cutis laxa 6 , 7; they were observed in children of African origin, except the one case of Lewis et a1. 8 in a Caucasian girl. Other postinflammatory elastolysis without cutis laxa may resemble mid-dermal elastolysis clinically, but the histologic feature is the absenceof a bandlikepattern of elastolysis in the mid dermis.Elastic fibersare absent in the papillary and mid dermis and persistent elastic fibers are dystrophic; some inflammatory cells are present. We believe that the caseof Brad et al. can be classified in this second group because the patient noticed inflam- matory lesions and because the histologic features were not those of typical mid-dermal elastolysis. Coincidentally, Shelley and Wood 4 have reported a preceding inflammatory dermatosis in one case of mid- dermal elastolysis, and it is possible that the differences betweennoninflammatoryelastolysis and postinflamma- tory elastolysis are not so meaningful. In addition, common features exist in all cases of elas- tolysis: a noninheriteddisease, female predominance, ab- sence of herniation clinically, and chronicity, without systemic involvment. M. Grossin" S. Maghraouii B. Crickx" and S. Belaich'' Departments of Pathology" and Dermatology' Hospital Bichat, 46, rue Henri Huchard 75877 Paris Cedex 18, France REFERENCES I . Brod BA, Rabkin M, Rhodes AR, et aJ. Mid-dermal elastolysis with inflammation. J AM ACAD DERMATOL 1992;26:882-4. 2. Betti R, Urbani CE, Lodi A, et al. Noninflammatory focal dermal elastolysis. J AM ACAD DERMATOL 1992;27:113-5. 3. Maghraoui S, Grossin M, Crickx B, et aJ.Mid dermal elas- tolysis. J AM ACAD DERMATOL 1992;26:490-2. 4. Shelley WB, Wood MG. Wrinkles due to idiopathic loss of midderrna l elastic tissue. Br J Dermatol 1977;97:441-5. 5. Brenner W, Gschnait F, Konrad K, et al. Noninflammatory dermal elastolysis. Br J Dermatol 1978;99:335-8. 6. Marshall J, Heyl T, Weber H. Post-inflammatory elastoly- sis and cutis laxa. South Med J 1966;40:1016-22. 7. Verhagen AR , Woerdeman MJ. Post-inflammatory elastol- ysis and cutis laxa. Br J Derrnatol 1975;92:183-90. 8. Lewis PG, Hood AF, Barnett NK, et al. Postinflamm ator y elastolysis and cutis laxa. J AM ACAD DERMATOL 1990;22:40-8. Reply To the Editor: I appreciate the interest in our report shown by Grossin, Maghraoui, Crickx,and Belaich. It is

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506 Correspondence

Reply

To the Editor: We thank Drs. Trileb, Bruckner-Tuder­man, and Burg for their reportof a patient withthe EECsyndrome and scalp dermatitis. In our article we statedthat scalp dermatitis can be seen in patients with theRapp-Hodgkin,Bowen-Armstrong, andAECsyndromes.Thisstatement wasbasedonan analysisofcasesreportedin the literature. We also noted that patients with theABC syndrome were 6.6 timesmore likely to have scalpdermatitis than patients with the Rapp-Hodgkin syn­drome.With inclusionofthecasereportedbyTriiebetal.,patients with the AEC syndrome would also be morelikelyto havescalpdermatitisthan patients withthe EECsyndrome (7 of 12 vs 1 of 148).

Scott w: Fosko , MIJPKurt S. Stenn, MIY

Jean L. Bolognia, MYDepartment ofDermatology,

University ofPennsylvaniaSchool ofMedicine, Philadelphia"

Skin Biology Unit, R. W. Johnson PharmaceuticalResearch Institute, Raritan, New Jersey'

Department ofDermatology, Yale University Schoolof Medicine, New Haven, Connecticut"

The spectrum of dermal elastolysis

To the Editor: We haveread withgreat interest the clin­icalcasesofdermal elastolysis reportedbyBrodet al.1andby Betti et al.2 We recently reported a case of mid-der­mal elastolysis' and wewant to defineprecisely the clini­copathologicfeatures of the differentvarieties of elastol­ysis.

Mid-dermal elastolysis was first reported by Shelleyand Wood" as an acquired, noninflammatory disease inadults, witha pathognomonic histologic pattern: bandlikemid-dermal elastolysis without any other morphologiclesion.

The case of Betti et aP has the same clinical patternas type II mid-dermal elastolysis reported by Brenner etal.s with papular lesions and perifollicular protrusions.Histologically, elastic fibers were absent in the papillarydermis and focally in mid dermis, without a mid-dermalbandlike pattern . Instead of a clinical pattern similar tomid-dermal elastolysis, Betti et al. describedthe case asnoninflammatory dermal elastolysis.

We agree with them and postulate that there are twogroups of elastolysis. The first is represented by nonin­flammatoryelastolysis as in the caseof Betti et al. and thecases of typical mid-dermal elastolysis first reported byShelley and Wood4 and by other authors more recently.

The secondgroup is represented by postinf'lammatory

Journal of the American Academy of DermatologySeptember 1993

elastolysis. Some cases of postinfiammatory elastolysisare associated with cutis laxa6, 7; they were observed inchildren of African origin, except the one case of Lewiset a1.8 in a Caucasian girl.

Other postinflammatory elastolysis without cutis laxamay resemble mid-dermal elastolysis clinically, but thehistologic feature is the absenceof a bandlike pattern ofelastolysis in the mid dermis. Elastic fibers are absent inthe papillary and mid dermis and persistent elastic fibersare dystrophic;some inflammatory cells are present.

We believe that the caseof Brad et al. can be classifiedin this second group because the patient noticed inflam­matory lesions and because the histologic features werenot those of typical mid-dermal elastolysis.

Coincidentally, Shelley and Wood4 have reported apreceding inflammatory dermatosis in one case of mid­dermal elastolysis, and it is possible that the differencesbetweennoninflammatoryelastolysis and postinflamma­tory elastolysis are not so meaningful.

In addition, common features exist in all casesof elas­tolysis: a noninheriteddisease, female predominance, ab­sence of herniation clinically, and chronicity, withoutsystemic involvment.

M. Grossin"S. MaghraouiiB. Crickx" and

S. Belaich''Departments of Pathology" and Dermatology'

Hospital Bichat, 46, rue Henri Huchard75877 Paris Cedex 18, France

REFERENCES

I . Brod BA, Rabkin M, Rhodes AR, et aJ. Mid-dermalelastolysis with inflammation. J AM ACAD DERMATOL

1992;26:882-4.2. Betti R, Urbani CE, Lodi A, et al. Noninflammatory focal

dermal elastolysis. J AM ACAD DERMATOL 1992;27:113-5.3. Maghraoui S, Grossin M, Crickx B, et aJ.Mid dermal elas­

tolysis. J AM ACAD DERMATOL 1992;26:490-2.4. Shelley WB, Wood MG. Wrinkles due to idiopathic loss of

midderrna l elastic tissue. Br J Dermatol 1977;97:441-5.5. Brenner W, Gschnait F, Konrad K, et al. Noninflammatory

dermal elastolysis. Br J Dermatol 1978;99:335-8.6. Marshall J, Heyl T, Weber H . Post-inflammatory elastoly­

sis and cutis laxa. South Med J 1966;40:1016-22.7. Verhagen AR , Woerdeman MJ. Post-inflammatory elastol­

ysis and cutis laxa. Br J Derrnatol 1975;92:183-90.8. Lewis PG, Hood AF, Barnett NK, et al. Postinflammator y

elastolysis and cutis laxa. J AM ACAD DERMATOL1990;22:40-8.

Reply

To the Editor: I appreciate the interest in our reportshown by Grossin, Maghraoui, Crickx, and Belaich.It is