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Periumbilical perforating pseudoxanthoma elasticum
642
Brief communications
primary manufacturer of DINP), there are no severe hazardous side effects from DINP unless it is burned. In this case, it releases toxic fumes. The greatest hazards from contact are mild eye and skin irritation. The Federal Department of Transportation does not consider DINP hazardous (Aristech Chemical Co., personalcommunication, June 1991). The Consumer Protection Agency had no reports of problems relating to DINP or Sqwish Balls. The other possible irritant to which the patient was exposed is the cleanser. The manufacturer of Soft Scrub informed us that the primary ingredient is calcium carbonate and that there have been few reports of dermatitis from its use (personal communication, August 1991). Because of the detergent
Journal of the American Academy of Dermatology
content of Soft Scrub and its pH of 10.5, irritation is possible. In this case, the clinical appearance of the eruption was that of an irritant dermatitis rather than an allergic contact dermatitis. This may have been caused by either the material within the "Sqwish Ball" (perhaps DINP) or from efforts used to remove this sticky material, or both. It should be emphasized that the adverse reaction occurred only after misuse of the toy. c.A.P. Toys, Inc. recommends the use of strong cleansers advertising degreasing action to remove this material from walls and other objects. A similar approach will remove the material from hands, although irritation is then likely to occur, especially in sensitive persons.
Periumbilical perforating pseudoxanthoma elasticum Debra Pruzan, MD, Phoebe E. Rabbin, MD, and Edward R. Heilman, MD
Brooklyn, New York Periumbilical perforating pseudoxanthoma elasticum represents a distinct clinicopathologic entity that occurs in obese, middle-aged, multiparous, black women. Patients typically present with a slowly enlarging, hyperpigmented, periumbilical plaque. The lesion is well demarcated with serpiginous borders. It is often flat centrally with discrete and coalescent keratotic papules scattered at the periphery to give a verrucous appearance. 1-6 We report the case of a patient with periumbilical perforating pseudoxanthoma elasticum and review previously reported cases and theories of etiology. CASE REPORT
A 61-year-old, multiparous, gravida 6, black woman had a 3-month history of a gradually enlarging, hyper-
From the Department of Dermatology, State University of New York-Health Science Center at Brooklyn. Reprint requests:Phoebe E. Rabbin, MD, Dept. of Dermatology, State Universityof New York-Health Science Center at Brooklyn,450 Clarkson Ave., Box 46, Brooklyn, NY 11203. 16/54/33900
pigmented plaque on her abdomen (Fig. 1). The lesion was asymptomatic. She had hypertension and congestive heart disease of severalyearsduration. She alsoreported a long history of diet-controlled diabetes mellitus. Previous surgery included an appendectomy and umbilical hernia repair at age 32,and a hysterectomy 10 yearslater. Physicalexaminationrevealed an obesewoman with a well-demarcated, hyperpigmented, 3 em plaque surrounding a small umbilicus. The right side of the lesion was flat and slightly atrophic, whilethe left portion was covered with discrete and confluent keratotic papules. Extending vertically downward from the plaque was a well-healed 7 em scar from her previous surgeries. Ophthalmologic examination showed no evidence of angioid streaks. The biopsyspecimen revealed pseudoepitheliomatous epidermalhyperplasia and increased bundlesofabnormal elastic fibers throughout the dermis (Fig. 2). There was transepidermal elimination of altered elastic fibers through channelsformed by hyperplastic epidermis that communicated with the surface. Within these channels there was basophilic degeneration and cornified debris (Fig. 2). The abnormal elasticfibers were arrayed in irregular bundleswith basophilic stipplingtypicalof calcification of the fiber (Fig. 3).
Volume 26 Number 4 April 1992
Briefcommunications
643
Fig. 1. Hyperpigmented periumbilical plaque.
Fig. 2. Photomicrograph of biopsy specimen of periumbilical plaque. Fig. 3. Calcification of elastic fibers in mid reticular dermis.
DISCUSSION
Eleven cases of perforating pseudoxantboma elasticum limited to the periumbilical area have been reported since 1979.2-6 These patients lack the cutaneous lesions of classic heritable pseudoxanthoma elasticum and retinal and vascular complications are generally not seen. Two patients with periumbilical pseudoxanthoma elasticum, however, were noted to have angioid streaks. I, 2 The patients share a similar medical history.2.6 All but two had hypertension. Other cardiovascular findings included cardiomegaly, left ventricular hypertrophy, congestive heart failure, and coronary artery disease. Diabetes mellitus was present in 27%. Although vascular abnormalities may occur in systemic pseudoxanthoma elasticum,? in perforating pseudoxanthoma elasticum these findings are more
likely attributable to the patient's atherosclerotic disease. A biopsy specimen of perforating pseudoxanthoma e1asticum stained with hematoxylin and eosin reveals short, gnarled, basophilic elastic fibers beneath an acanthotic epidermis. The fibers stain positively for calcium. Transepidermal elimination of these altered fibers is seen." In the past the perforation was attributed to coexistent elastosis perforans serpiginosa and pseudoxanthoma elasticum, but in 1976 Lund and Gilberti proved this was not true. After evaluation of nine cases they determined there was no histologic evidence of elastosis perforans serpiginosa. A definitive term to describe this entity has not been universally accepted. Lever and SchaumburgLever? use the term perforating calcific elastosis;
644
Brief communications
Neldnerand Martinez-Hernandez'' preferlocalized acquired cutaneous pseudoxanthoma elasticum. Lund coined the term perforatingpseudoxanthoma elasticum. Perforation is not unique to patients with periumbilical pseudoxanthoma elasticum, it has also been noted in lesions of classic, hereditary pseudoxanthoma elasticum. Itis difficult to explain the periumbilicallocalizationof pseudoxanthoma elasticum anditslimitation to obese, multiparous, black women. Two theories havebeenproposed. Hicks et al.3 theorized that patients were genetically predisposed to pseudoxanthoma elasticum but that heavily pigmented skinmay be more resistant than light skin to the pathologic event in pseudoxanthoma elasticum. Onlyafter the trauma of multiple pregnancies, for example, is a threshold reachedat which damageto periumbilical elasticfibers may occur. However, periumbilical pseudoxanthoma elasticum may simply be an acquired event. Again,mul-
Journal of the American Academy of Dermatology
tiple traumatic events to abdominal skin would be the initiating factor. Such stresses as multiparity, obesity, massive ascites, and surgery havebeen implicated.i" Our patient was a multiparous woman with a history of several surgicalprocedures in the periumbilical region. REFERENCES 1. Lund HZ, Gilbert CF. Perforatingpseudoxanthoma elasticum. Arch PatholI976;100:544-6. 2. Schwartz RA, Richfield DF. Pseudoxanthoma elasticum with transpidermalelimination. Arch Dermatol 1978;114: 279-80. 3. HicksJ, Carpenter CL, Reed RJ. Periumbilical perforating pseudoxanthoma elasticum. ArchDermatol1979;115:300-3. 4. Kuzakis AM, Parish WR. Periumbilical perforating pseudoxanthoma elasticum. J AM ACAD DERMATOL 1988;19: 384-8. 5. Premalatha S, Yesudian P, Thamblah AS. Periumbilical pseudoxanthoma elasticum withtransepidermal elimination. Int J DermatolI982;10:604-5. 6. Neldner KH, Martinez-Hernandez A. Localized acquired cutaneous PXE. J AM ACAD DERMATOL 1979;1:423-30. 7. LeverWF, Schaumberg-Lever, Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott, 1989.
Brief communications
primary manufacturer of DINP), there are no severe hazardous side effects from DINP unless it is burned. In this case, it releases toxic fumes. The greatest hazards from contact are mild eye and skin irritation. The Federal Department of Transportation does not consider DINP hazardous (Aristech Chemical Co., personalcommunication, June 1991). The Consumer Protection Agency had no reports of problems relating to DINP or Sqwish Balls. The other possible irritant to which the patient was exposed is the cleanser. The manufacturer of Soft Scrub informed us that the primary ingredient is calcium carbonate and that there have been few reports of dermatitis from its use (personal communication, August 1991). Because of the detergent
Journal of the American Academy of Dermatology
content of Soft Scrub and its pH of 10.5, irritation is possible. In this case, the clinical appearance of the eruption was that of an irritant dermatitis rather than an allergic contact dermatitis. This may have been caused by either the material within the "Sqwish Ball" (perhaps DINP) or from efforts used to remove this sticky material, or both. It should be emphasized that the adverse reaction occurred only after misuse of the toy. c.A.P. Toys, Inc. recommends the use of strong cleansers advertising degreasing action to remove this material from walls and other objects. A similar approach will remove the material from hands, although irritation is then likely to occur, especially in sensitive persons.
Periumbilical perforating pseudoxanthoma elasticum Debra Pruzan, MD, Phoebe E. Rabbin, MD, and Edward R. Heilman, MD
Brooklyn, New York Periumbilical perforating pseudoxanthoma elasticum represents a distinct clinicopathologic entity that occurs in obese, middle-aged, multiparous, black women. Patients typically present with a slowly enlarging, hyperpigmented, periumbilical plaque. The lesion is well demarcated with serpiginous borders. It is often flat centrally with discrete and coalescent keratotic papules scattered at the periphery to give a verrucous appearance. 1-6 We report the case of a patient with periumbilical perforating pseudoxanthoma elasticum and review previously reported cases and theories of etiology. CASE REPORT
A 61-year-old, multiparous, gravida 6, black woman had a 3-month history of a gradually enlarging, hyper-
From the Department of Dermatology, State University of New York-Health Science Center at Brooklyn. Reprint requests:Phoebe E. Rabbin, MD, Dept. of Dermatology, State Universityof New York-Health Science Center at Brooklyn,450 Clarkson Ave., Box 46, Brooklyn, NY 11203. 16/54/33900
pigmented plaque on her abdomen (Fig. 1). The lesion was asymptomatic. She had hypertension and congestive heart disease of severalyearsduration. She alsoreported a long history of diet-controlled diabetes mellitus. Previous surgery included an appendectomy and umbilical hernia repair at age 32,and a hysterectomy 10 yearslater. Physicalexaminationrevealed an obesewoman with a well-demarcated, hyperpigmented, 3 em plaque surrounding a small umbilicus. The right side of the lesion was flat and slightly atrophic, whilethe left portion was covered with discrete and confluent keratotic papules. Extending vertically downward from the plaque was a well-healed 7 em scar from her previous surgeries. Ophthalmologic examination showed no evidence of angioid streaks. The biopsyspecimen revealed pseudoepitheliomatous epidermalhyperplasia and increased bundlesofabnormal elastic fibers throughout the dermis (Fig. 2). There was transepidermal elimination of altered elastic fibers through channelsformed by hyperplastic epidermis that communicated with the surface. Within these channels there was basophilic degeneration and cornified debris (Fig. 2). The abnormal elasticfibers were arrayed in irregular bundleswith basophilic stipplingtypicalof calcification of the fiber (Fig. 3).
Volume 26 Number 4 April 1992
Briefcommunications
643
Fig. 1. Hyperpigmented periumbilical plaque.
Fig. 2. Photomicrograph of biopsy specimen of periumbilical plaque. Fig. 3. Calcification of elastic fibers in mid reticular dermis.
DISCUSSION
Eleven cases of perforating pseudoxantboma elasticum limited to the periumbilical area have been reported since 1979.2-6 These patients lack the cutaneous lesions of classic heritable pseudoxanthoma elasticum and retinal and vascular complications are generally not seen. Two patients with periumbilical pseudoxanthoma elasticum, however, were noted to have angioid streaks. I, 2 The patients share a similar medical history.2.6 All but two had hypertension. Other cardiovascular findings included cardiomegaly, left ventricular hypertrophy, congestive heart failure, and coronary artery disease. Diabetes mellitus was present in 27%. Although vascular abnormalities may occur in systemic pseudoxanthoma elasticum,? in perforating pseudoxanthoma elasticum these findings are more
likely attributable to the patient's atherosclerotic disease. A biopsy specimen of perforating pseudoxanthoma e1asticum stained with hematoxylin and eosin reveals short, gnarled, basophilic elastic fibers beneath an acanthotic epidermis. The fibers stain positively for calcium. Transepidermal elimination of these altered fibers is seen." In the past the perforation was attributed to coexistent elastosis perforans serpiginosa and pseudoxanthoma elasticum, but in 1976 Lund and Gilberti proved this was not true. After evaluation of nine cases they determined there was no histologic evidence of elastosis perforans serpiginosa. A definitive term to describe this entity has not been universally accepted. Lever and SchaumburgLever? use the term perforating calcific elastosis;
644
Brief communications
Neldnerand Martinez-Hernandez'' preferlocalized acquired cutaneous pseudoxanthoma elasticum. Lund coined the term perforatingpseudoxanthoma elasticum. Perforation is not unique to patients with periumbilical pseudoxanthoma elasticum, it has also been noted in lesions of classic, hereditary pseudoxanthoma elasticum. Itis difficult to explain the periumbilicallocalizationof pseudoxanthoma elasticum anditslimitation to obese, multiparous, black women. Two theories havebeenproposed. Hicks et al.3 theorized that patients were genetically predisposed to pseudoxanthoma elasticum but that heavily pigmented skinmay be more resistant than light skin to the pathologic event in pseudoxanthoma elasticum. Onlyafter the trauma of multiple pregnancies, for example, is a threshold reachedat which damageto periumbilical elasticfibers may occur. However, periumbilical pseudoxanthoma elasticum may simply be an acquired event. Again,mul-
Journal of the American Academy of Dermatology
tiple traumatic events to abdominal skin would be the initiating factor. Such stresses as multiparity, obesity, massive ascites, and surgery havebeen implicated.i" Our patient was a multiparous woman with a history of several surgicalprocedures in the periumbilical region. REFERENCES 1. Lund HZ, Gilbert CF. Perforatingpseudoxanthoma elasticum. Arch PatholI976;100:544-6. 2. Schwartz RA, Richfield DF. Pseudoxanthoma elasticum with transpidermalelimination. Arch Dermatol 1978;114: 279-80. 3. HicksJ, Carpenter CL, Reed RJ. Periumbilical perforating pseudoxanthoma elasticum. ArchDermatol1979;115:300-3. 4. Kuzakis AM, Parish WR. Periumbilical perforating pseudoxanthoma elasticum. J AM ACAD DERMATOL 1988;19: 384-8. 5. Premalatha S, Yesudian P, Thamblah AS. Periumbilical pseudoxanthoma elasticum withtransepidermal elimination. Int J DermatolI982;10:604-5. 6. Neldner KH, Martinez-Hernandez A. Localized acquired cutaneous PXE. J AM ACAD DERMATOL 1979;1:423-30. 7. LeverWF, Schaumberg-Lever, Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott, 1989.