Numerous Flesh-Colored Nodules on the Trunk

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Numerous Flesh-Colored Nodules on the Trunk
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Matthew McEwen, MD
Zachary Bryan, MD
Anand Rajpara, MD

The Diagnosis: Steatocystoma Multiplex 

The punch biopsy of an abdominal lesion demonstrated a folded cyst wall with a wavy eosinophilic cuticle (Figure), characteristics consistent with steatocystoma multiplex (SM).  

Punch biopsy demonstrated a folded cyst wall with a wavy eosinophilic cuticle characteristic of steatocystoma (H&E, original magnification ×200).

Also known as eruptive steatocystoma, SM consists of numerous flesh-colored, dome-shaped papules and nodules that most commonly arise during adolescence, with a median age of onset of 26 years.1 These hamartomatous nevoid malformations arise in areas with well-developed pilosebaceous units, such as the upper extremities, neck, axillae, and trunk.1,2 They occur less commonly on the scalp, face, and acral surfaces.2-5 The lesions range in size from 2 to 30 mm6 and usually are asymptomatic.1 Occasionally, steatocystomas become tender or can rupture.7 

Steatocystoma multiplex may arise sporadically or may be inherited in an autosomal-dominant fashion. Mutations in exon 1 of the keratin 17 gene, KRT17, have been identified in autosomal-dominant SM.6,8KRT17 mutations also are responsible for pachyonychia congenita type 2, which is associated with SM.9 Some patients with pachyonychia congenita type 2 who have prominent SM and mild nail findings may be misdiagnosed as having pure SM.2 

The histopathologic features of SM were described in a study by Cho and colleagues1 of 64 patients. Steatocystomas have cyst walls that may be either intricately folded or round/oval, comprised of an average of 4.9 epithelial cell layers. In most cases, the cyst wall contains sebaceous lobules. In all cases, an acellular eosinophilic cuticle was present, and no granular layer was seen. Few vellus hairs may be observed in the cystic cavity.1 

The differential diagnosis of SM includes eruptive vellus hair cysts, lipomas, Muir-Torre syndrome, and Gardner syndrome. Some have suggested that eruptive vellus hair cysts and SM exist on a disease spectrum because of their similar clinical presentation.10 In contrast to SM, however, eruptive vellus hair cysts originate in the infundibulum of the hair shaft rather than the sebaceous duct, and more numerous vellus hair shafts are seen on histopathology.1 

Various treatment modalities have been described, including isotretinoin for inflamed lesions,11 cryotherapy for noninflamed lesions,11 aspiration of lesions smallerthan 1 cm,12 and electrocautery combined with topical retinoids.13 Laser treatment has been described, with a 1450-nm diode laser used to target the abnormal sebaceous glands and a 1550-nm fractionated erbium-doped fiber laser used to target the dermal cysts.14 Carbon dioxide lasers also may be used to open the cyst for drainage.15 Surgical excision or mini-incision also may be performed.16,17  
 
Acknowledgment
The authors thank Garth Fraga, MD (Kansas City, Kansas), for his assistance with interpretation of the dermatopathology in this case.  

References
  1. Cho S, Chang SE, Choi JH, et al. Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol. 2002;29:152-156. 
  2. Rollins T, Levin RM, Heymann WR. Acral steatocystoma multiplex.J Am Acad Dermatol. 2000;43(2, pt 2):396-399. 
  3. Setoyama M, Mizoguchi S, Usuki K, et al. Steatocystoma multiplex: a case with unusual clinical and histological manifestation. Am J Dermatopathol. 1997;19:89-92. 
  4. Cole LA. Steatocystoma multiplex. Arch Dermatol. 1976;112:1437-1439. 
  5. Marzano AV, Tavecchio S, Balice Y, et al. Acral subcutaneous steatocystoma multiplex: a distinct subtype of the disease? Australas J Dermatol. 2012;53:198-201. 
  6. Liu Q, Wu W, Lu J, et al. Steatocystoma multiplex is associated with the R94C mutation in the KRTl7 gene. Mol Med Rep. 2015;12:5072-5076. 
  7. Egbert BM, Price NM, Segal RJ. Steatocystoma multiplex. Report of a florid case and a review. Arch Dermatol. 1979;115:334-335. 
  8. Covello SP, Smith FJ, Sillevis Smitt JH, et al. Keratin 17 mutations cause either steatocystoma multiplex or pachyonychia congenita type 2. Br J Dermatol. 1998;139:475-480. 
  9. McLean WH, Rugg EL, Lunny DP, et al. Keratin 16 and keratin 17 mutations cause pachyonychia congenita. Nat Genet. 1995;9:273-278. 
  10. Ohtake N, Kubota Y, Takayama O, et al. Relationship between steatocystoma multiplex and eruptive vellus hair cysts. J Am Acad Dermatol. 1992;26(5, pt 2):876-878. 
  11. Apaydin R, Bilen N, Bayramgurler D, et al. Steatocystoma multiplex suppurativum: oral isotretinoin treatment combined with cryotherapy. Australas J Dermatol. 2000;41:98-100. 
  12. Sato K, Shibuya K, Taguchi H, et al. Aspiration therapy in steatocystoma multiplex. Arch Dermatol. 1993;129:35-37. 
  13. Papakonstantinou E, Franke I, Gollnick H. Facial steatocystoma multiplex combined with eruptive vellus hair cysts: a hybrid? J Eur Acad Dermatol Venereol. 2015;29:2051-2053. 
  14. Moody MN, Landau JM, Goldberg LH, et al. 1,450-nm diode laser in combination with the 1550-nm fractionated erbium-doped fiber laser for the treatment of steatocystoma multiplex: a case report. Dermatol Surg. 2012;38(7, pt 1):1104-1106. 
  15. Rossi R, Cappugi P, Battini M, et al. CO2 laser therapy in a case of steatocystoma multiplex with prominent nodules on the face and neck. Int J Dermatol. 2003;42:302-304. 
  16. Schmook T, Burg G, Hafner J. Surgical pearl: mini-incisions for the extraction of steatocystoma multiplex. J Am Acad Dermatol. 2001;44:1041-1042. 
  17. Adams BB, Mutasim DF, Nordlund JJ. Steatocystoma multiplex: a quick removal technique. Cutis. 1999;64:127-130.
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Author and Disclosure Information

Dr. McEwen is from the Department of Dermatology, University of Tennessee Health Science Center, Memphis. Drs. Bryan and Rajpara are from the Division of Dermatology, University of Kansas, Kansas City.

The authors report no conflict of interest.

Correspondence: Matthew McEwen, MD, Department of Dermatology, University of Tennessee Health Science Center, 930 Madison Ave, Ste 840, Memphis, TN 38163 (mmcewen2@uthsc.edu).

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Author(s)
Matthew McEwen, MD
Zachary Bryan, MD
Anand Rajpara, MD
Author(s)
Matthew McEwen, MD
Zachary Bryan, MD
Anand Rajpara, MD
Author and Disclosure Information

Dr. McEwen is from the Department of Dermatology, University of Tennessee Health Science Center, Memphis. Drs. Bryan and Rajpara are from the Division of Dermatology, University of Kansas, Kansas City.

The authors report no conflict of interest.

Correspondence: Matthew McEwen, MD, Department of Dermatology, University of Tennessee Health Science Center, 930 Madison Ave, Ste 840, Memphis, TN 38163 (mmcewen2@uthsc.edu).

Author and Disclosure Information

Dr. McEwen is from the Department of Dermatology, University of Tennessee Health Science Center, Memphis. Drs. Bryan and Rajpara are from the Division of Dermatology, University of Kansas, Kansas City.

The authors report no conflict of interest.

Correspondence: Matthew McEwen, MD, Department of Dermatology, University of Tennessee Health Science Center, 930 Madison Ave, Ste 840, Memphis, TN 38163 (mmcewen2@uthsc.edu).

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CT104006022_e.PDF
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The Diagnosis: Steatocystoma Multiplex 

The punch biopsy of an abdominal lesion demonstrated a folded cyst wall with a wavy eosinophilic cuticle (Figure), characteristics consistent with steatocystoma multiplex (SM).  

Punch biopsy demonstrated a folded cyst wall with a wavy eosinophilic cuticle characteristic of steatocystoma (H&E, original magnification ×200).

Also known as eruptive steatocystoma, SM consists of numerous flesh-colored, dome-shaped papules and nodules that most commonly arise during adolescence, with a median age of onset of 26 years.1 These hamartomatous nevoid malformations arise in areas with well-developed pilosebaceous units, such as the upper extremities, neck, axillae, and trunk.1,2 They occur less commonly on the scalp, face, and acral surfaces.2-5 The lesions range in size from 2 to 30 mm6 and usually are asymptomatic.1 Occasionally, steatocystomas become tender or can rupture.7 

Steatocystoma multiplex may arise sporadically or may be inherited in an autosomal-dominant fashion. Mutations in exon 1 of the keratin 17 gene, KRT17, have been identified in autosomal-dominant SM.6,8KRT17 mutations also are responsible for pachyonychia congenita type 2, which is associated with SM.9 Some patients with pachyonychia congenita type 2 who have prominent SM and mild nail findings may be misdiagnosed as having pure SM.2 

The histopathologic features of SM were described in a study by Cho and colleagues1 of 64 patients. Steatocystomas have cyst walls that may be either intricately folded or round/oval, comprised of an average of 4.9 epithelial cell layers. In most cases, the cyst wall contains sebaceous lobules. In all cases, an acellular eosinophilic cuticle was present, and no granular layer was seen. Few vellus hairs may be observed in the cystic cavity.1 

The differential diagnosis of SM includes eruptive vellus hair cysts, lipomas, Muir-Torre syndrome, and Gardner syndrome. Some have suggested that eruptive vellus hair cysts and SM exist on a disease spectrum because of their similar clinical presentation.10 In contrast to SM, however, eruptive vellus hair cysts originate in the infundibulum of the hair shaft rather than the sebaceous duct, and more numerous vellus hair shafts are seen on histopathology.1 

Various treatment modalities have been described, including isotretinoin for inflamed lesions,11 cryotherapy for noninflamed lesions,11 aspiration of lesions smallerthan 1 cm,12 and electrocautery combined with topical retinoids.13 Laser treatment has been described, with a 1450-nm diode laser used to target the abnormal sebaceous glands and a 1550-nm fractionated erbium-doped fiber laser used to target the dermal cysts.14 Carbon dioxide lasers also may be used to open the cyst for drainage.15 Surgical excision or mini-incision also may be performed.16,17  
 
Acknowledgment
The authors thank Garth Fraga, MD (Kansas City, Kansas), for his assistance with interpretation of the dermatopathology in this case.  

The Diagnosis: Steatocystoma Multiplex 

The punch biopsy of an abdominal lesion demonstrated a folded cyst wall with a wavy eosinophilic cuticle (Figure), characteristics consistent with steatocystoma multiplex (SM).  

Punch biopsy demonstrated a folded cyst wall with a wavy eosinophilic cuticle characteristic of steatocystoma (H&E, original magnification ×200).

Also known as eruptive steatocystoma, SM consists of numerous flesh-colored, dome-shaped papules and nodules that most commonly arise during adolescence, with a median age of onset of 26 years.1 These hamartomatous nevoid malformations arise in areas with well-developed pilosebaceous units, such as the upper extremities, neck, axillae, and trunk.1,2 They occur less commonly on the scalp, face, and acral surfaces.2-5 The lesions range in size from 2 to 30 mm6 and usually are asymptomatic.1 Occasionally, steatocystomas become tender or can rupture.7 

Steatocystoma multiplex may arise sporadically or may be inherited in an autosomal-dominant fashion. Mutations in exon 1 of the keratin 17 gene, KRT17, have been identified in autosomal-dominant SM.6,8KRT17 mutations also are responsible for pachyonychia congenita type 2, which is associated with SM.9 Some patients with pachyonychia congenita type 2 who have prominent SM and mild nail findings may be misdiagnosed as having pure SM.2 

The histopathologic features of SM were described in a study by Cho and colleagues1 of 64 patients. Steatocystomas have cyst walls that may be either intricately folded or round/oval, comprised of an average of 4.9 epithelial cell layers. In most cases, the cyst wall contains sebaceous lobules. In all cases, an acellular eosinophilic cuticle was present, and no granular layer was seen. Few vellus hairs may be observed in the cystic cavity.1 

The differential diagnosis of SM includes eruptive vellus hair cysts, lipomas, Muir-Torre syndrome, and Gardner syndrome. Some have suggested that eruptive vellus hair cysts and SM exist on a disease spectrum because of their similar clinical presentation.10 In contrast to SM, however, eruptive vellus hair cysts originate in the infundibulum of the hair shaft rather than the sebaceous duct, and more numerous vellus hair shafts are seen on histopathology.1 

Various treatment modalities have been described, including isotretinoin for inflamed lesions,11 cryotherapy for noninflamed lesions,11 aspiration of lesions smallerthan 1 cm,12 and electrocautery combined with topical retinoids.13 Laser treatment has been described, with a 1450-nm diode laser used to target the abnormal sebaceous glands and a 1550-nm fractionated erbium-doped fiber laser used to target the dermal cysts.14 Carbon dioxide lasers also may be used to open the cyst for drainage.15 Surgical excision or mini-incision also may be performed.16,17  
 
Acknowledgment
The authors thank Garth Fraga, MD (Kansas City, Kansas), for his assistance with interpretation of the dermatopathology in this case.  

References
  1. Cho S, Chang SE, Choi JH, et al. Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol. 2002;29:152-156. 
  2. Rollins T, Levin RM, Heymann WR. Acral steatocystoma multiplex.J Am Acad Dermatol. 2000;43(2, pt 2):396-399. 
  3. Setoyama M, Mizoguchi S, Usuki K, et al. Steatocystoma multiplex: a case with unusual clinical and histological manifestation. Am J Dermatopathol. 1997;19:89-92. 
  4. Cole LA. Steatocystoma multiplex. Arch Dermatol. 1976;112:1437-1439. 
  5. Marzano AV, Tavecchio S, Balice Y, et al. Acral subcutaneous steatocystoma multiplex: a distinct subtype of the disease? Australas J Dermatol. 2012;53:198-201. 
  6. Liu Q, Wu W, Lu J, et al. Steatocystoma multiplex is associated with the R94C mutation in the KRTl7 gene. Mol Med Rep. 2015;12:5072-5076. 
  7. Egbert BM, Price NM, Segal RJ. Steatocystoma multiplex. Report of a florid case and a review. Arch Dermatol. 1979;115:334-335. 
  8. Covello SP, Smith FJ, Sillevis Smitt JH, et al. Keratin 17 mutations cause either steatocystoma multiplex or pachyonychia congenita type 2. Br J Dermatol. 1998;139:475-480. 
  9. McLean WH, Rugg EL, Lunny DP, et al. Keratin 16 and keratin 17 mutations cause pachyonychia congenita. Nat Genet. 1995;9:273-278. 
  10. Ohtake N, Kubota Y, Takayama O, et al. Relationship between steatocystoma multiplex and eruptive vellus hair cysts. J Am Acad Dermatol. 1992;26(5, pt 2):876-878. 
  11. Apaydin R, Bilen N, Bayramgurler D, et al. Steatocystoma multiplex suppurativum: oral isotretinoin treatment combined with cryotherapy. Australas J Dermatol. 2000;41:98-100. 
  12. Sato K, Shibuya K, Taguchi H, et al. Aspiration therapy in steatocystoma multiplex. Arch Dermatol. 1993;129:35-37. 
  13. Papakonstantinou E, Franke I, Gollnick H. Facial steatocystoma multiplex combined with eruptive vellus hair cysts: a hybrid? J Eur Acad Dermatol Venereol. 2015;29:2051-2053. 
  14. Moody MN, Landau JM, Goldberg LH, et al. 1,450-nm diode laser in combination with the 1550-nm fractionated erbium-doped fiber laser for the treatment of steatocystoma multiplex: a case report. Dermatol Surg. 2012;38(7, pt 1):1104-1106. 
  15. Rossi R, Cappugi P, Battini M, et al. CO2 laser therapy in a case of steatocystoma multiplex with prominent nodules on the face and neck. Int J Dermatol. 2003;42:302-304. 
  16. Schmook T, Burg G, Hafner J. Surgical pearl: mini-incisions for the extraction of steatocystoma multiplex. J Am Acad Dermatol. 2001;44:1041-1042. 
  17. Adams BB, Mutasim DF, Nordlund JJ. Steatocystoma multiplex: a quick removal technique. Cutis. 1999;64:127-130.
References
  1. Cho S, Chang SE, Choi JH, et al. Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol. 2002;29:152-156. 
  2. Rollins T, Levin RM, Heymann WR. Acral steatocystoma multiplex.J Am Acad Dermatol. 2000;43(2, pt 2):396-399. 
  3. Setoyama M, Mizoguchi S, Usuki K, et al. Steatocystoma multiplex: a case with unusual clinical and histological manifestation. Am J Dermatopathol. 1997;19:89-92. 
  4. Cole LA. Steatocystoma multiplex. Arch Dermatol. 1976;112:1437-1439. 
  5. Marzano AV, Tavecchio S, Balice Y, et al. Acral subcutaneous steatocystoma multiplex: a distinct subtype of the disease? Australas J Dermatol. 2012;53:198-201. 
  6. Liu Q, Wu W, Lu J, et al. Steatocystoma multiplex is associated with the R94C mutation in the KRTl7 gene. Mol Med Rep. 2015;12:5072-5076. 
  7. Egbert BM, Price NM, Segal RJ. Steatocystoma multiplex. Report of a florid case and a review. Arch Dermatol. 1979;115:334-335. 
  8. Covello SP, Smith FJ, Sillevis Smitt JH, et al. Keratin 17 mutations cause either steatocystoma multiplex or pachyonychia congenita type 2. Br J Dermatol. 1998;139:475-480. 
  9. McLean WH, Rugg EL, Lunny DP, et al. Keratin 16 and keratin 17 mutations cause pachyonychia congenita. Nat Genet. 1995;9:273-278. 
  10. Ohtake N, Kubota Y, Takayama O, et al. Relationship between steatocystoma multiplex and eruptive vellus hair cysts. J Am Acad Dermatol. 1992;26(5, pt 2):876-878. 
  11. Apaydin R, Bilen N, Bayramgurler D, et al. Steatocystoma multiplex suppurativum: oral isotretinoin treatment combined with cryotherapy. Australas J Dermatol. 2000;41:98-100. 
  12. Sato K, Shibuya K, Taguchi H, et al. Aspiration therapy in steatocystoma multiplex. Arch Dermatol. 1993;129:35-37. 
  13. Papakonstantinou E, Franke I, Gollnick H. Facial steatocystoma multiplex combined with eruptive vellus hair cysts: a hybrid? J Eur Acad Dermatol Venereol. 2015;29:2051-2053. 
  14. Moody MN, Landau JM, Goldberg LH, et al. 1,450-nm diode laser in combination with the 1550-nm fractionated erbium-doped fiber laser for the treatment of steatocystoma multiplex: a case report. Dermatol Surg. 2012;38(7, pt 1):1104-1106. 
  15. Rossi R, Cappugi P, Battini M, et al. CO2 laser therapy in a case of steatocystoma multiplex with prominent nodules on the face and neck. Int J Dermatol. 2003;42:302-304. 
  16. Schmook T, Burg G, Hafner J. Surgical pearl: mini-incisions for the extraction of steatocystoma multiplex. J Am Acad Dermatol. 2001;44:1041-1042. 
  17. Adams BB, Mutasim DF, Nordlund JJ. Steatocystoma multiplex: a quick removal technique. Cutis. 1999;64:127-130.
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Numerous Flesh-Colored Nodules on the Trunk
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A 33-year-old woman presented with numerous firm, noncompressible, flesh-colored nodules that measured 3 to 4 mm and were distributed across the abdomen, chest, back, and neck. The lesions had been present for approximately 10 years. The patient denied any lesion-associated pain, itching, or bleeding, and there was no family history of similar lesions. A punch biopsy of a lesion on the central abdomen was obtained. 

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