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Mid dermal elastolysis
Volume 22 Number 2, Part I February 1990 with treatment. Fig. I demonstrates a pretreatment lesion of seborrheic keratosis. The lesion appears to have a polypoid appearance (Fig. 2). Note the presence of large globular exudations from the surface (Fig. 3); these appear to be oleaginous globules, probably sebum. In Fig. 4 the lesion has cleared and the skin appearance is normal. CONCLUSION
Despite many anecdotal reports describing effective treatment (disappearance of lesions), in our vehicle-controlled, double-blind clinical study, we did not observe a significant difference between results of the active drug versus those of the vehicle except for the height (elevation) of the lesions, which was reduced by Lac-Hydrin lotion.
Ammonium lactate in seborrheic keratoses REFERENCES 1. Tindall JP. Skin changes and lesions in our senior citizens:
incidences. Cutis 1976;18:359-62. 2. Van Scott EJ, Yu RJ. Hyperkeratinization, comeocyte c0hesion, and alpha hydroxy acids. J AM ACAD DERMATOL 1984;11:867-79.
Russell T. Bagatell F, Wehr R, et al. Comparative efficacy of 12% ammonium lactate lotion vs a petrolatum based crcam in acute and extended treatment of moderate to severe xerosis. Presented at Forty-Ninth Annual Meeting of the American Academy of Dermatology, Dec. 5-10, 1987; San Antonio, Tex. 4. Ryan RL, Hing SAD, Theiler RF. A replica technique for the evaluation of human skin by scanning electron microscopy. J Cutan Pathol 1983;10:262-76.
3.
Mid dermal elastolysis Robert I. Rudolph, MD* Wyomissing, Pennsylvania Mid dermal elastolysis is an exceedingly rare condition. Only three cases have been reported previously. The disease manifests as sharply marginated plaques of fine wrinkles of various size that give the skin an aged appearance. It is characterized histologically by a complete absence of mid dermal elastic fibers and lack of any inflammatory infiltrate. Two new cases are reported, and the other cases are reported. (J AM ACAD DERMATOL 1990;22:203-6.) Two cases of a rare acquired noninflammatory dermal elastolysis are reported in which patches of fine wrinkles were the only clinical manifestations. CASE REPORTS
Case It A 40-year-old white man noted "patches of wrinkles" on his arms for about 3 or 4 years. At no time had any inflammation or pruritus occurred, nor were the patches
From the Department of Dermatology, University of Pennsylvania. Accepted for publication March 23, 1989. No reprints available. -In private practice in Wyomissing, Pennsylvania. tReportcd at the American Academy of Dermatology meeting, Washington, DC, Dec. 3, 1988.
enlarging. He was in excel1ent general health and was not an avid "sun worshipper." Symmetric and sharply circumscribed plaques of fine wrinkles were noted on the deltoid and acromial regions, with smaller patches located on the scapular and pectoral regions (Figs. 1 and 2). No follicular prominences were noted, but the wrinkles did appear to follow lines of cleavage. The affected area were normal in pigmentation, and no erythema, scaling, induration, atrophy, or herniation was present. When the skin was pulled up between the thumb and index finger both normal and abnormal skin rebounded the same. Lateral tension obliterated the wrinkling. The rest of the skin appeared normal.
Case 2 A 57-year-old white woman noted "patches of wrinkles" on her breasts, abdomen, back, and sides of the trunk for about 1 year. At no time had any redness or 203
Journal of the American Academy of Dermatology
204 Rudolph
Fig. 1. Case 1. Finely wrinkled skin on deltoid area.
itching occurred. She had chronic rheumatoid arthritis for which shewas taking aspirin. She had avoided sunlight exposure for many years. Examination revealed circumscribed plaques of fine wrinkles on the breasts, upper aspect of the back, abdomen, and flanks. No follicular prominences were seen, and the wrinkles appeared to follow lines of cleavage. No erythema, scale, inflammation, herniation, or atrophy was present. Affected areas were normal in color and exhibited the same rebound as unaffected skin. The remainder of the skin appeared normal.
Histopathologic and immunofluorescence study findings Punch biopsy specimens revealed a somewhat thinned epidermis, with a well-defined basal cell layer. Small vessels of the papillary dermis were not increased in number, nor did cellular infiltration occur about them. Elastic tissue stain reactions showed a complete absence of elastic fibers throughout the upper mid dermis, although a fcw fibers were seen around some of the blood vessels (Fig. 3). Elastic tissues in the papillary and reticular dermis appeared normal. A cellular inflammatory infiltrate was absent. Immunofluorescence studies were negative.
Fig. 2. Case 1. Close-up showing sharply marginated, fine wrinkles that follow cleavage lines. DISCUSSION
Only three other patients with similar skin findings have been described. The fust case of "wrinkles due to idiopathic loss of mid dermal elastic tissue" was described by Shelley and Wood in 1977. 1 Their patient, a 42-year-old white woman, had areas of "crinkling of her skin" for about 2 years. The areas were normal in color and asymptomatic and exhibited no atrophy or herniation. The patches on the arms, antecubital fossae, and trunk were circumscribed areas of fine wrinkles that gave the patient an "inappropriate aged look." This woman had a I-year history of recurrent urticaria about 7 years before the onset of the wrinkling. A second case, now entitled "noninflammatory dermal elastolysis" was reported in 1978. 2 The patient, a 32-year-old woman, had plaquelike, weJlmarginated area of wrinkling on her trunk for about 4 years, which led to a "misfitting aged appearance of her skin." There was no history of preceding urticaria nor of any other skin disorder, and no family history of such a condition. Clinically, two morphologic patterns were noted: small wrinkles arranged
Volume 22 Number 2, Parl 1 February 1990
Mid dermal elastolysis 205
Fig. 3. Case I. Absence of elastic tissues in upper mid dermis, with normal elastic network in papillary and reticular dermis. (Verhoeff's elastic tissue stain; Xl 02.)
parallel to the skin cleavage lines (type I) and very small soft papular lesions composed of perifollicular protrusions that left an indented center at the follicular orifice (type II). A third case was recently reported under the title "disseminated nevus anelasticus."3 The patient, a 23-year-old white man, had diffuse fine wrinkling of the skin of his back and trunk arranged along lines of cleavage. In some areas (especially the upper aspect of the chest and interscapular areas) the skin was stippled by multiple small follicular lesions, such that "one could have the impression of a hammered iron sheet." All five reported cases seemed to represent a distinct clinicopathologic entity. Except for one patient,
no preceding history of inflammation of any kind existed. In no case was there a family history of similar cutaneous findings, nor a history of other associated cutaneous or medical conditions or drug ingestion. Once the plaques of wrinkling had appeared they appeared to be stationary. A relation to sunlight exposure does not appear to exist; patches of wrinkles have been noted on exposed and nonexposed areas. The clinical appearance was the same in all cases, that is, patches of finely wrinkled skin with tiny wrinkles following lines of cleavage. Affected skin was normal in color, and no patient exhibited any sign of inflammation, scaling, atrophy, or herniation. Two patients had a second type of pattern within the
Journal of the American Academy of Dermatology
206 Rudolph plaques, namely, perifollicular lesions corresponding to some preservation of elastic tissue around hair follicles. The histologic findings in all reported cases, including the two cases I report, appear to be identical. Various elastic tissue stain reactions revealed a complete absence of elastic fibers in the mid to upper mid dermis. The elastic fiber network in the papillary and reticular dermis appeared to he intact and completely normal. Elastic fibers at the borders of the lesions did not seem to be in disarray, and unaffected skin exhibited a normal elastic fiber network. Electron microscopy showed a "marked reduction of elastic fibers in the mid dermal zone," whereas other constituents of the dermis, including collagen fibers, vessels, and fibroblasts, showed no alterations. 2 Shelley and Wood I believed that the process most likely represented a "post inflammatory elastolysis" related to the urticaria noted years before by their patient, akin to the changes found in young African patients in which a complete absence of elastic tissue in the upper mid dennis manifest as large patches of wrinkling. 4 In these children, however,
there was a preceding acute phase of firm and erythematous lesions, which were thought to be possibly related to arthropod bites. It is certainly conceivable that some type of inflammatory elastolysis occurred in these patients that was asymptomatic and clinically nonevident, but sufficient in intensity to cause destruction and disappearance of elastic fibers. Since this article was accepted for publication, another case of mid dermal elastolysis has been reported by Virginia Rae and Vincent Falanga (Arch Dermato1 1989;125:950-1). The patient was a 33-year-old white woman with fine wrinkling on the abdomen, back, and upper arms, which was not preceded by trauma or urticaria. Pathologic findings were identical to my cases and the other reports in the literature. REFERENCES 1. Shelley WB, Wood MG. Wrinkles due to idiopathic loss of mid-dermal clastic tissue. Br J Dcrmatol 1977;97:441-5. 2. Brenner W, Gsehnait F, Konrad H, et al. Non-inflammatory dermal elastosis. Br J Dermatol 1978;98:335-8. 3. Crivellato E. Disseminated nevus anelasticus. tnt J Dermatol 1986;25: 171-3. 4. Verhagen AR, Woerdernan MJ. Post-inflammatory elastolysis and cutis laxa. Br.l Dcrmatol 1975;95:183-90.
Despite many anecdotal reports describing effective treatment (disappearance of lesions), in our vehicle-controlled, double-blind clinical study, we did not observe a significant difference between results of the active drug versus those of the vehicle except for the height (elevation) of the lesions, which was reduced by Lac-Hydrin lotion.
Ammonium lactate in seborrheic keratoses REFERENCES 1. Tindall JP. Skin changes and lesions in our senior citizens:
incidences. Cutis 1976;18:359-62. 2. Van Scott EJ, Yu RJ. Hyperkeratinization, comeocyte c0hesion, and alpha hydroxy acids. J AM ACAD DERMATOL 1984;11:867-79.
Russell T. Bagatell F, Wehr R, et al. Comparative efficacy of 12% ammonium lactate lotion vs a petrolatum based crcam in acute and extended treatment of moderate to severe xerosis. Presented at Forty-Ninth Annual Meeting of the American Academy of Dermatology, Dec. 5-10, 1987; San Antonio, Tex. 4. Ryan RL, Hing SAD, Theiler RF. A replica technique for the evaluation of human skin by scanning electron microscopy. J Cutan Pathol 1983;10:262-76.
3.
Mid dermal elastolysis Robert I. Rudolph, MD* Wyomissing, Pennsylvania Mid dermal elastolysis is an exceedingly rare condition. Only three cases have been reported previously. The disease manifests as sharply marginated plaques of fine wrinkles of various size that give the skin an aged appearance. It is characterized histologically by a complete absence of mid dermal elastic fibers and lack of any inflammatory infiltrate. Two new cases are reported, and the other cases are reported. (J AM ACAD DERMATOL 1990;22:203-6.) Two cases of a rare acquired noninflammatory dermal elastolysis are reported in which patches of fine wrinkles were the only clinical manifestations. CASE REPORTS
Case It A 40-year-old white man noted "patches of wrinkles" on his arms for about 3 or 4 years. At no time had any inflammation or pruritus occurred, nor were the patches
From the Department of Dermatology, University of Pennsylvania. Accepted for publication March 23, 1989. No reprints available. -In private practice in Wyomissing, Pennsylvania. tReportcd at the American Academy of Dermatology meeting, Washington, DC, Dec. 3, 1988.
enlarging. He was in excel1ent general health and was not an avid "sun worshipper." Symmetric and sharply circumscribed plaques of fine wrinkles were noted on the deltoid and acromial regions, with smaller patches located on the scapular and pectoral regions (Figs. 1 and 2). No follicular prominences were noted, but the wrinkles did appear to follow lines of cleavage. The affected area were normal in pigmentation, and no erythema, scaling, induration, atrophy, or herniation was present. When the skin was pulled up between the thumb and index finger both normal and abnormal skin rebounded the same. Lateral tension obliterated the wrinkling. The rest of the skin appeared normal.
Case 2 A 57-year-old white woman noted "patches of wrinkles" on her breasts, abdomen, back, and sides of the trunk for about 1 year. At no time had any redness or 203
Journal of the American Academy of Dermatology
204 Rudolph
Fig. 1. Case 1. Finely wrinkled skin on deltoid area.
itching occurred. She had chronic rheumatoid arthritis for which shewas taking aspirin. She had avoided sunlight exposure for many years. Examination revealed circumscribed plaques of fine wrinkles on the breasts, upper aspect of the back, abdomen, and flanks. No follicular prominences were seen, and the wrinkles appeared to follow lines of cleavage. No erythema, scale, inflammation, herniation, or atrophy was present. Affected areas were normal in color and exhibited the same rebound as unaffected skin. The remainder of the skin appeared normal.
Histopathologic and immunofluorescence study findings Punch biopsy specimens revealed a somewhat thinned epidermis, with a well-defined basal cell layer. Small vessels of the papillary dermis were not increased in number, nor did cellular infiltration occur about them. Elastic tissue stain reactions showed a complete absence of elastic fibers throughout the upper mid dermis, although a fcw fibers were seen around some of the blood vessels (Fig. 3). Elastic tissues in the papillary and reticular dermis appeared normal. A cellular inflammatory infiltrate was absent. Immunofluorescence studies were negative.
Fig. 2. Case 1. Close-up showing sharply marginated, fine wrinkles that follow cleavage lines. DISCUSSION
Only three other patients with similar skin findings have been described. The fust case of "wrinkles due to idiopathic loss of mid dermal elastic tissue" was described by Shelley and Wood in 1977. 1 Their patient, a 42-year-old white woman, had areas of "crinkling of her skin" for about 2 years. The areas were normal in color and asymptomatic and exhibited no atrophy or herniation. The patches on the arms, antecubital fossae, and trunk were circumscribed areas of fine wrinkles that gave the patient an "inappropriate aged look." This woman had a I-year history of recurrent urticaria about 7 years before the onset of the wrinkling. A second case, now entitled "noninflammatory dermal elastolysis" was reported in 1978. 2 The patient, a 32-year-old woman, had plaquelike, weJlmarginated area of wrinkling on her trunk for about 4 years, which led to a "misfitting aged appearance of her skin." There was no history of preceding urticaria nor of any other skin disorder, and no family history of such a condition. Clinically, two morphologic patterns were noted: small wrinkles arranged
Volume 22 Number 2, Parl 1 February 1990
Mid dermal elastolysis 205
Fig. 3. Case I. Absence of elastic tissues in upper mid dermis, with normal elastic network in papillary and reticular dermis. (Verhoeff's elastic tissue stain; Xl 02.)
parallel to the skin cleavage lines (type I) and very small soft papular lesions composed of perifollicular protrusions that left an indented center at the follicular orifice (type II). A third case was recently reported under the title "disseminated nevus anelasticus."3 The patient, a 23-year-old white man, had diffuse fine wrinkling of the skin of his back and trunk arranged along lines of cleavage. In some areas (especially the upper aspect of the chest and interscapular areas) the skin was stippled by multiple small follicular lesions, such that "one could have the impression of a hammered iron sheet." All five reported cases seemed to represent a distinct clinicopathologic entity. Except for one patient,
no preceding history of inflammation of any kind existed. In no case was there a family history of similar cutaneous findings, nor a history of other associated cutaneous or medical conditions or drug ingestion. Once the plaques of wrinkling had appeared they appeared to be stationary. A relation to sunlight exposure does not appear to exist; patches of wrinkles have been noted on exposed and nonexposed areas. The clinical appearance was the same in all cases, that is, patches of finely wrinkled skin with tiny wrinkles following lines of cleavage. Affected skin was normal in color, and no patient exhibited any sign of inflammation, scaling, atrophy, or herniation. Two patients had a second type of pattern within the
Journal of the American Academy of Dermatology
206 Rudolph plaques, namely, perifollicular lesions corresponding to some preservation of elastic tissue around hair follicles. The histologic findings in all reported cases, including the two cases I report, appear to be identical. Various elastic tissue stain reactions revealed a complete absence of elastic fibers in the mid to upper mid dermis. The elastic fiber network in the papillary and reticular dermis appeared to he intact and completely normal. Elastic fibers at the borders of the lesions did not seem to be in disarray, and unaffected skin exhibited a normal elastic fiber network. Electron microscopy showed a "marked reduction of elastic fibers in the mid dermal zone," whereas other constituents of the dermis, including collagen fibers, vessels, and fibroblasts, showed no alterations. 2 Shelley and Wood I believed that the process most likely represented a "post inflammatory elastolysis" related to the urticaria noted years before by their patient, akin to the changes found in young African patients in which a complete absence of elastic tissue in the upper mid dennis manifest as large patches of wrinkling. 4 In these children, however,
there was a preceding acute phase of firm and erythematous lesions, which were thought to be possibly related to arthropod bites. It is certainly conceivable that some type of inflammatory elastolysis occurred in these patients that was asymptomatic and clinically nonevident, but sufficient in intensity to cause destruction and disappearance of elastic fibers. Since this article was accepted for publication, another case of mid dermal elastolysis has been reported by Virginia Rae and Vincent Falanga (Arch Dermato1 1989;125:950-1). The patient was a 33-year-old white woman with fine wrinkling on the abdomen, back, and upper arms, which was not preceded by trauma or urticaria. Pathologic findings were identical to my cases and the other reports in the literature. REFERENCES 1. Shelley WB, Wood MG. Wrinkles due to idiopathic loss of mid-dermal clastic tissue. Br J Dcrmatol 1977;97:441-5. 2. Brenner W, Gsehnait F, Konrad H, et al. Non-inflammatory dermal elastosis. Br J Dermatol 1978;98:335-8. 3. Crivellato E. Disseminated nevus anelasticus. tnt J Dermatol 1986;25: 171-3. 4. Verhagen AR, Woerdernan MJ. Post-inflammatory elastolysis and cutis laxa. Br.l Dcrmatol 1975;95:183-90.