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Dermal spherulosis (myospherulosis) after topical treatment for psoriasis
Journal of the American Academy of Dermatology Volume 30, Number 2, Part 1
of the skin is usually evident by the fourth decade and is most prominent on the forehead, cheeks, axillae, and genitalia. Whereas cutaneous involvement is asymptomatic, skeletal involvement is a major cause of morbidity. Large joints like the knee, shoulder, hip, and spine are the most severely affected. In this patient cutaneous ochronosis was most marked on the palms. To my knowledge this feature has not been previously reported, In the darkskinned races a faint pigmentation may not be clinically evident, and areas of lighter skin such as the palms may be the first to show a generalized pigment disorder. The close association of the pigment deposition with sunlight-induced dermal degeneration was also remarkable. Solar elastotic material contains a large amount of acid mucopolysaccharides. I Gaines/ stated that ochronotic pigment has a particular af-
Briefcommunications 265 finity for collagen fibers that are surrounded. by abundant mucopolysaccharide ground substance. Electron microscopic studies have documented deposits of pigment surrounding collagen fibers in areas of solar elastosis.' Jordaan and Mulligan4 described a patient with exogenous ochronosis in whom superimposed actinic granuloma-like lesions developed.
REFERENCES
I. Lever W, Schaumburg-Lever G. Degenerative diseases. In: Histopathology of the skin. 6th ed. Philadelphia: JB Lippincott, 1983:271-2. 2. Gaines JJ. The pathology of alkaptonuric ochronosis. Hum Pathol 1989;20:40-6. 3. Attword HD, Clifton S, Mitchell RE. A histological histochemical and ultrastructural study of dermal ochronosis. Pathology 1971;3:1IS-9. 4. Jardaan HF, Mulligan RP. Actinic granuloma like change in exogenous ochronosis. J Cutan Pathol 1990;17:236-40.
Dermal spherulosis (myospherulosis) after topical treatment for psoriasis Aneta Lazarov, MD,a Ilana Avinoach, MD,b Hani Giryes, MD,a and Sima Halevy, MDa Beer-Sheva, Israel Myospherulosis, first described in 1969 by MeClatchic et aI., 1,2 refers to the histopathologic changes of "sac-like structures with endobodies" in soft tumors of the subcutaneous tissue and muscles. The previously assumed primary infectious cause has been disprovede' because there is evidence that the "spherules" are derived from erythrocytes altered by foreign lipids and human fat. 5. 8 Although there are many reported cases of myospherulosis in the otorhinolaryngologic and dental Iiterature.v 7, 9-12 the condition has not been described frequently in the dermatologic literature. Published case reports have focused on changes in the subcutaneous adipose tissue 13, 14 and on some
From the Department of Dermatology' and Institute of PathologY,b Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev. Reprint requests: Aneta Lazarov, MD, Department of Dermatology, P.O. Box lSI, Beer-Shcva 841OI,Israei.
J
AM
ACAD DERMATOL 1994;30:265-7.
Copyright@ 1994 by the American Academy of Dermatology, Inc. 0190.9622/94 $1.00
+ .10
16/54/49370
occasions the condition has been related to intramuscular injection.f 13 We report an unusual case of spherulocytic changes confined only to the dennis that developed after prolonged topical treatment of psoriasis. CASE REPORT
A 63-year-old man had a long history of psoriasis with frequent exacerbations of psoriatic erythroderma and pustular psoriasis. He had been treated with etrctinate, PUV A, UVB rays, Goeckerman regimen, anthralin ointment, and topical steroidal ointments (both with and without occlusion) for many years. The patient was admitted to the hospital for treatment of psoriatic erythroderma. Examination revealed generalized erythema, edema, and scaling. After the erythroderma subsided,infiltration and thickening of the skin in the sacroiliacarea were noted.A biopsy specimenshowed a thickenedhorny layerwithareas of parakeratosis,a hyperplasticepidermis with features of psoriasiform spongiotic dermatitis. A pronounced perivascular mononuclear inflammatory infiltrate composed predominantly of lymphocytes, some histiocytes, plasma cells, and eosinophils was present in the papillarydermis. In the same area, predominantlyin
266
Brief communications
Journal of the American Academy of Dermatology February 1994
.It Fig. 2. Higher magnification of Fig. 1 shows clumped erythrocytes in the lumina of some fat globules. (Hematoxylin-eosin stain; X400.)
the dermal papillae, diffuse and prominent extravasation of red blood cells was seen. Close to the rete ridges in the papillary dermis, fatty globules surrounded by a prominent chronic inflammatory infiltrate and fibrosis were observed (Fig. I). Clumped red blood cells were found within the lumina of the globules. Fusion of some of the fatty globules resulted in formation of crenulated microcysts (Fig. 2). The subcutaneous fat was uninvolved. A biopsy specimen from the same area 1 year later revealed similar findings confined primarily to the reticular dermis. The diffuse extravasation of red blood cells was more pronounced between collagen bundles, as well as in intraluminal spaces and adjacent to the walls of the cystic spaces.
DISCUSSION
Since the original description of myospherulosis by McClatchie et al., 1,2 there has been a considerable reevaluation of the pathogenesis and terminology of this condition. Hutt et al. 15 suggested the term subcu.taneous spheru/ocystic disease instead of myospherulosis because in their patient the nodules did not affect the muscular tissue. In fact, the term
myospherulosis is currently being replaced by subcutaneous spherulocytosis because the latter describes the changes more accurately. Kyriakos' suggested that myospherulosis might be produced by petrolatum-based antibiotic ointment. Wheeler and Mcfiavran'' suggested that fat necrosis from injections, trauma, or infection might produce the lipids responsible for the development of myospherulosis. They demonstrated that myospherules could be produced in vitro by lanolin, petrolatum, or by emulsified human fat. Carlson and Jackson 11 proposed that myospherulosis can be a complication of wound healing that results from interaction between lipids and extravasated erythrocytes. Rosaf first observed that "rnyospherules" resulted from clumping of erythrocytes that was produced in vitro with tetracycline ointment. Rosai 5 and Travis et a1. 16 confirmed the presence of hemoglobin on the surface of both the sac and the spherules, thereby providing additional evidence to support a red blood cell origin for the myospherules. Nodules, which are the typical lesions described in spherulocytic disease (myospherulosis), were not observed in our patient. There was no evidence of infection, trauma, wound healing, or injections in the sacral area of our patient, in which the histologic spherulocytic changes were seen. However, there was a history of long-term application of different topical corticosteroids containing lanolin and petrolatum, as well as repeated episodes of inflammation. The spherulocytic changes were observed only in the papillary dermis in the first biopsy specimen and deeper in the dermis in the second biopsy specimen, taken from the same area I year later. This suggests that the spherules were produced by the interaction
Journal of the American Academy of Dermatology Volume 30, Number 2, Part 1
of the exogenous fat from the topical ointments and the extravasated erythrocytes. We believe that this is the first reported case of spherulocystic changes confined only to the dermis provoked by topically applied fat-containing ointments. REFERENCES
I. McClatchie S, Bremner AD. Unusual subcutaneousswellings in African patients. East Afr Med .I 1969;46:625-33. 2. McClatchie S, Warambo MW, Bremner AD. Myospherulosis: A previously unreported disease? Am J Clin Pathol 1969;51 :699-704. 3. Kyriakos M. Myospherulosisof the paranasal sinuses, nose and middle ear. Am J Clin Patbol 1977;76: I J 8-30. 4. De Schryver-Keckemeti K, Kyriakos M. The induction of human myospherulosis in experimental animals. Am J Pathol 1977;87:33-46. 5. Rosai 1. The nature of myospherulosis of the upper respiratory tract. Am 1 Clin Pathol 1978;69:475-81. 6. Wheeler TM, McGavran MH. Myospherulosis-further observations. Am J Clin Pat hoi 1980;73:685-6. 7. Gillespie CA, Clark WB, Finkelstein E, et al. Myospherulosis of the mastoid antrum. Auris Nasus Larynx 1990; 16:199-207.
Briefcommunications 267 8. Shimada K, Kobayashi S, Yamadori I, et al. Myospherulosis in lapan: a report of two cases and an immunohistochemical investigation. Am .I Surg Pathol 1988;12:42732. 9. Lynch DP, Newland JR, McClendon JL. Myospherulosis of the oral hard and soft tissues. J Oral Maxillofac Surg 1984;42:349-55. 10. Belfiglio El, Wunderlich ST, Fox. LJ. Myospherulosis of the alveolus secondary to the use of Terra-Cortril and Gelfoam: report of a case. Oral Surg Oral Med Oral Pathol 1986;61:12-4. 11. Carlson ER, Jackson B. Myospherulosis: complicating wound healing. J Am Dent Assoc 1991;122:65-6. 12. Paugh DR, Sullivan M.I. Myospherulosis of the paranasal sinuses. Otolaryngol Head Neck Surg 1990;103:117-9. 13. Waldman lS, Barr R.I, Espinoza FP, ct al. Subcutaneous myospherulosis. J AM ACAD DERMATOL 1989;21:400-3. 14. Ono T, Kageshita T, Hirai S, et al. Coexistence of spherulocyticdisease (myospherulosis) and membranocystic degeneration. Arch Dermatol 1991;127:88-90. 15. Hutt MSR, Fernandes Bl.1, Templeton AC. Myospherulosis (subcutaneous spherulocytic disease). Trans R Soc Trop Med Hyg 1971;65:182-8. 16. Travis WD, Li CY, Weiland LH. Immunostaining for hemoglobin in two cases of myospherulosis. Arch Pathol Lab Med 1986;110:763-5.
Purpuric "gloves-and-socks" syndrome: Histologic, immunofluorescence, and polymerase chain reaction study Akiva Trattner, MD, and Michael David, MD Petah Tiqva, Israel In 1990 Harms et al. I reported a new syndrome consisting of pruritic edema and erythema of the hands and feet in a gloves-and-socks distribution, with rapid development of petechial purpura. Only two additional patients have been reported.s 3 We describe a patient with the same clinical findings and report the immunofluorescence and polymerase chain reaction findings, which were not performed in previous studies. CASE REPORT A 34-year-old woman had a 5-day history of progressive pruritic reddening and swelling of the hands and feet. Her temperature was 38.3 0 C. She was delivered of a From the Department of Dermatology. Beilinson Medical Center. Pctah Tiqva, Sackler Faculty of Medicine, Tel Aviv University. Reprint requests: A. Trauner, MD, Department of Dermatology, BeiLinson Medical Center, Petah Tiqva 49100, Israel.
J
AM ACAD DERMATOl.
1994;30:267-8.
Copyright ® 1994 by the American Academy of Dermatology, Inc. 0190-9622/94 $1.00
+ .10 16/54/49372
healthy child 2\12 months earlier after a normal pregnancy. No drug administration preceded the onset of the cutaneous eruption. The patient's hands and feet showed a sharply marginated, symmetric edema and erythema. On the seventh day many petechiae appeared within the erythematous skin on the hands (Fig. 1) and feet. At the same time, a petechial eruption developed in the inguinal areas and on the buttocks. Petechiae on the hard palate and pharyngeal hyperemia were also noted. Laboratory studies revealed leukopenia with a normal differential count (leukocyte count, 208 X l09/L); hemoglobin level, hematocrit, and the platelet count were normal. Results of blood urea nitrogen, serum creatinine, total serum protein, total bilirubin, alkaline phosphatase, SOOT, and glucose were normal. Results ofVDRL and Treponema pallidum hemagglutination assay, serology for Epstein-Barr virus, cytomegalovirus, herpes simplex virus, anti-hepatitis C virus, hepatitis B virus surface antigen, anti-hepatitis B virus surface antigen, and antihepatitis A virus were normal or negative. Anti-streptolysin titer was 200 IU/ ml and 2 weeks later was 400 IV/ ml. Bacteriologic throat cultures were negative. A skin
of the skin is usually evident by the fourth decade and is most prominent on the forehead, cheeks, axillae, and genitalia. Whereas cutaneous involvement is asymptomatic, skeletal involvement is a major cause of morbidity. Large joints like the knee, shoulder, hip, and spine are the most severely affected. In this patient cutaneous ochronosis was most marked on the palms. To my knowledge this feature has not been previously reported, In the darkskinned races a faint pigmentation may not be clinically evident, and areas of lighter skin such as the palms may be the first to show a generalized pigment disorder. The close association of the pigment deposition with sunlight-induced dermal degeneration was also remarkable. Solar elastotic material contains a large amount of acid mucopolysaccharides. I Gaines/ stated that ochronotic pigment has a particular af-
Briefcommunications 265 finity for collagen fibers that are surrounded. by abundant mucopolysaccharide ground substance. Electron microscopic studies have documented deposits of pigment surrounding collagen fibers in areas of solar elastosis.' Jordaan and Mulligan4 described a patient with exogenous ochronosis in whom superimposed actinic granuloma-like lesions developed.
REFERENCES
I. Lever W, Schaumburg-Lever G. Degenerative diseases. In: Histopathology of the skin. 6th ed. Philadelphia: JB Lippincott, 1983:271-2. 2. Gaines JJ. The pathology of alkaptonuric ochronosis. Hum Pathol 1989;20:40-6. 3. Attword HD, Clifton S, Mitchell RE. A histological histochemical and ultrastructural study of dermal ochronosis. Pathology 1971;3:1IS-9. 4. Jardaan HF, Mulligan RP. Actinic granuloma like change in exogenous ochronosis. J Cutan Pathol 1990;17:236-40.
Dermal spherulosis (myospherulosis) after topical treatment for psoriasis Aneta Lazarov, MD,a Ilana Avinoach, MD,b Hani Giryes, MD,a and Sima Halevy, MDa Beer-Sheva, Israel Myospherulosis, first described in 1969 by MeClatchic et aI., 1,2 refers to the histopathologic changes of "sac-like structures with endobodies" in soft tumors of the subcutaneous tissue and muscles. The previously assumed primary infectious cause has been disprovede' because there is evidence that the "spherules" are derived from erythrocytes altered by foreign lipids and human fat. 5. 8 Although there are many reported cases of myospherulosis in the otorhinolaryngologic and dental Iiterature.v 7, 9-12 the condition has not been described frequently in the dermatologic literature. Published case reports have focused on changes in the subcutaneous adipose tissue 13, 14 and on some
From the Department of Dermatology' and Institute of PathologY,b Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev. Reprint requests: Aneta Lazarov, MD, Department of Dermatology, P.O. Box lSI, Beer-Shcva 841OI,Israei.
J
AM
ACAD DERMATOL 1994;30:265-7.
Copyright@ 1994 by the American Academy of Dermatology, Inc. 0190.9622/94 $1.00
+ .10
16/54/49370
occasions the condition has been related to intramuscular injection.f 13 We report an unusual case of spherulocytic changes confined only to the dennis that developed after prolonged topical treatment of psoriasis. CASE REPORT
A 63-year-old man had a long history of psoriasis with frequent exacerbations of psoriatic erythroderma and pustular psoriasis. He had been treated with etrctinate, PUV A, UVB rays, Goeckerman regimen, anthralin ointment, and topical steroidal ointments (both with and without occlusion) for many years. The patient was admitted to the hospital for treatment of psoriatic erythroderma. Examination revealed generalized erythema, edema, and scaling. After the erythroderma subsided,infiltration and thickening of the skin in the sacroiliacarea were noted.A biopsy specimenshowed a thickenedhorny layerwithareas of parakeratosis,a hyperplasticepidermis with features of psoriasiform spongiotic dermatitis. A pronounced perivascular mononuclear inflammatory infiltrate composed predominantly of lymphocytes, some histiocytes, plasma cells, and eosinophils was present in the papillarydermis. In the same area, predominantlyin
266
Brief communications
Journal of the American Academy of Dermatology February 1994
.It Fig. 2. Higher magnification of Fig. 1 shows clumped erythrocytes in the lumina of some fat globules. (Hematoxylin-eosin stain; X400.)
the dermal papillae, diffuse and prominent extravasation of red blood cells was seen. Close to the rete ridges in the papillary dermis, fatty globules surrounded by a prominent chronic inflammatory infiltrate and fibrosis were observed (Fig. I). Clumped red blood cells were found within the lumina of the globules. Fusion of some of the fatty globules resulted in formation of crenulated microcysts (Fig. 2). The subcutaneous fat was uninvolved. A biopsy specimen from the same area 1 year later revealed similar findings confined primarily to the reticular dermis. The diffuse extravasation of red blood cells was more pronounced between collagen bundles, as well as in intraluminal spaces and adjacent to the walls of the cystic spaces.
DISCUSSION
Since the original description of myospherulosis by McClatchie et al., 1,2 there has been a considerable reevaluation of the pathogenesis and terminology of this condition. Hutt et al. 15 suggested the term subcu.taneous spheru/ocystic disease instead of myospherulosis because in their patient the nodules did not affect the muscular tissue. In fact, the term
myospherulosis is currently being replaced by subcutaneous spherulocytosis because the latter describes the changes more accurately. Kyriakos' suggested that myospherulosis might be produced by petrolatum-based antibiotic ointment. Wheeler and Mcfiavran'' suggested that fat necrosis from injections, trauma, or infection might produce the lipids responsible for the development of myospherulosis. They demonstrated that myospherules could be produced in vitro by lanolin, petrolatum, or by emulsified human fat. Carlson and Jackson 11 proposed that myospherulosis can be a complication of wound healing that results from interaction between lipids and extravasated erythrocytes. Rosaf first observed that "rnyospherules" resulted from clumping of erythrocytes that was produced in vitro with tetracycline ointment. Rosai 5 and Travis et a1. 16 confirmed the presence of hemoglobin on the surface of both the sac and the spherules, thereby providing additional evidence to support a red blood cell origin for the myospherules. Nodules, which are the typical lesions described in spherulocytic disease (myospherulosis), were not observed in our patient. There was no evidence of infection, trauma, wound healing, or injections in the sacral area of our patient, in which the histologic spherulocytic changes were seen. However, there was a history of long-term application of different topical corticosteroids containing lanolin and petrolatum, as well as repeated episodes of inflammation. The spherulocytic changes were observed only in the papillary dermis in the first biopsy specimen and deeper in the dermis in the second biopsy specimen, taken from the same area I year later. This suggests that the spherules were produced by the interaction
Journal of the American Academy of Dermatology Volume 30, Number 2, Part 1
of the exogenous fat from the topical ointments and the extravasated erythrocytes. We believe that this is the first reported case of spherulocystic changes confined only to the dermis provoked by topically applied fat-containing ointments. REFERENCES
I. McClatchie S, Bremner AD. Unusual subcutaneousswellings in African patients. East Afr Med .I 1969;46:625-33. 2. McClatchie S, Warambo MW, Bremner AD. Myospherulosis: A previously unreported disease? Am J Clin Pathol 1969;51 :699-704. 3. Kyriakos M. Myospherulosisof the paranasal sinuses, nose and middle ear. Am J Clin Patbol 1977;76: I J 8-30. 4. De Schryver-Keckemeti K, Kyriakos M. The induction of human myospherulosis in experimental animals. Am J Pathol 1977;87:33-46. 5. Rosai 1. The nature of myospherulosis of the upper respiratory tract. Am 1 Clin Pathol 1978;69:475-81. 6. Wheeler TM, McGavran MH. Myospherulosis-further observations. Am J Clin Pat hoi 1980;73:685-6. 7. Gillespie CA, Clark WB, Finkelstein E, et al. Myospherulosis of the mastoid antrum. Auris Nasus Larynx 1990; 16:199-207.
Briefcommunications 267 8. Shimada K, Kobayashi S, Yamadori I, et al. Myospherulosis in lapan: a report of two cases and an immunohistochemical investigation. Am .I Surg Pathol 1988;12:42732. 9. Lynch DP, Newland JR, McClendon JL. Myospherulosis of the oral hard and soft tissues. J Oral Maxillofac Surg 1984;42:349-55. 10. Belfiglio El, Wunderlich ST, Fox. LJ. Myospherulosis of the alveolus secondary to the use of Terra-Cortril and Gelfoam: report of a case. Oral Surg Oral Med Oral Pathol 1986;61:12-4. 11. Carlson ER, Jackson B. Myospherulosis: complicating wound healing. J Am Dent Assoc 1991;122:65-6. 12. Paugh DR, Sullivan M.I. Myospherulosis of the paranasal sinuses. Otolaryngol Head Neck Surg 1990;103:117-9. 13. Waldman lS, Barr R.I, Espinoza FP, ct al. Subcutaneous myospherulosis. J AM ACAD DERMATOL 1989;21:400-3. 14. Ono T, Kageshita T, Hirai S, et al. Coexistence of spherulocyticdisease (myospherulosis) and membranocystic degeneration. Arch Dermatol 1991;127:88-90. 15. Hutt MSR, Fernandes Bl.1, Templeton AC. Myospherulosis (subcutaneous spherulocytic disease). Trans R Soc Trop Med Hyg 1971;65:182-8. 16. Travis WD, Li CY, Weiland LH. Immunostaining for hemoglobin in two cases of myospherulosis. Arch Pathol Lab Med 1986;110:763-5.
Purpuric "gloves-and-socks" syndrome: Histologic, immunofluorescence, and polymerase chain reaction study Akiva Trattner, MD, and Michael David, MD Petah Tiqva, Israel In 1990 Harms et al. I reported a new syndrome consisting of pruritic edema and erythema of the hands and feet in a gloves-and-socks distribution, with rapid development of petechial purpura. Only two additional patients have been reported.s 3 We describe a patient with the same clinical findings and report the immunofluorescence and polymerase chain reaction findings, which were not performed in previous studies. CASE REPORT A 34-year-old woman had a 5-day history of progressive pruritic reddening and swelling of the hands and feet. Her temperature was 38.3 0 C. She was delivered of a From the Department of Dermatology. Beilinson Medical Center. Pctah Tiqva, Sackler Faculty of Medicine, Tel Aviv University. Reprint requests: A. Trauner, MD, Department of Dermatology, BeiLinson Medical Center, Petah Tiqva 49100, Israel.
J
AM ACAD DERMATOl.
1994;30:267-8.
Copyright ® 1994 by the American Academy of Dermatology, Inc. 0190-9622/94 $1.00
+ .10 16/54/49372
healthy child 2\12 months earlier after a normal pregnancy. No drug administration preceded the onset of the cutaneous eruption. The patient's hands and feet showed a sharply marginated, symmetric edema and erythema. On the seventh day many petechiae appeared within the erythematous skin on the hands (Fig. 1) and feet. At the same time, a petechial eruption developed in the inguinal areas and on the buttocks. Petechiae on the hard palate and pharyngeal hyperemia were also noted. Laboratory studies revealed leukopenia with a normal differential count (leukocyte count, 208 X l09/L); hemoglobin level, hematocrit, and the platelet count were normal. Results of blood urea nitrogen, serum creatinine, total serum protein, total bilirubin, alkaline phosphatase, SOOT, and glucose were normal. Results ofVDRL and Treponema pallidum hemagglutination assay, serology for Epstein-Barr virus, cytomegalovirus, herpes simplex virus, anti-hepatitis C virus, hepatitis B virus surface antigen, anti-hepatitis B virus surface antigen, and antihepatitis A virus were normal or negative. Anti-streptolysin titer was 200 IU/ ml and 2 weeks later was 400 IV/ ml. Bacteriologic throat cultures were negative. A skin