Steatocystoma Multiplex: a Review ⇑ Manahel Mahmood Alsabbagh

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Journal of Dermatology & Dermatologic Surgery xxx (2016) xxx–xxx

Steatocystoma multiplex: A review ⇑ Manahel Mahmood AlSabbagh

Received 16 January 2016; accepted 14 February 2016

Abstract

Steatocystoma multiplex is an uncommon benign disorder typically manifests as multiple asymptomatic intradermal cysts. It can be sporadic, familial or a part of a syndrome. Rare variants can be easily misdiagnosed. Histologically, it appears as an epithelium-lined cystic wall associated with sebaceous lobules. Treatment is needed if symptomatic or for cosmetic issues with the best results reported following radiofrequency, laser therapy and minimally invasive procedures. Ó 2016 The Author. Production and hosting by Elsevier B.V. on behalf of King Saud University. This is an open access article under the CC BY- NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Keywords: Steatocystoma multiplex; Cyst; Laser; Isotretinoin

Contents

1. Introduction ...... 00 2. Methodology...... 00 3. Clinical presentation ...... 00 4. Concurrences/associations ...... 00 5. Genetics ...... 00 6. Differential diagnosis ...... 00 7. Histopathology and cytology ...... 00 8. Management ...... 00 9. Conclusion ...... 00 Conflict of interest...... 00 References ...... 00

1. Introduction

⇑ Tel.: +973 39729242. Steatocystoma multiplex is an uncommon benign cuta- E-mail address: [email protected] Peer review under responsibility of King Saud University. neous disorder. It was ﬁrst described by Jamieson in 1873 and the name was given by Pringle in 1899 (Davey, 2014). (Steato) and (Cyst) are Greek roots which mean fatty bag. (Multi) and (Plex) are Latin preﬁx and root Production and hosting by Elsevier which mean many networks (Borro, 1960). http://dx.doi.org/10.1016/j.jdds.2016.02.001 2352-2410/Ó 2016 The Author. Production and hosting by Elsevier B.V. on behalf of King Saud University. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Total of 272 publicaons

EBSCOhost automacally excluded 33 exact duplicaons.

Author reviewed all 239 tles, abstracts and full arcles.

Author excluded 130 publicaons for the following reasons:

- Duplicates (were not excluded by EBSCOhost); - Titles or abstracts were uninformave (in absence of full text arcles); - Publicaons provide basic informaon already provided by other publicaons.

Author analysed each of the remaining 109 tles, abstracts and arcles and developed the theme of the current arcle:

- Clinical presentaon; - Associaons/concurrences; - Relevant potenal mutaons; - Diﬀerenal diagnosis and reported confusions; - Findings on histopathological and cytological examinaons; and - Reported treatment methodologies.

Figure 1. Methodology of the literature review.

The aim of this paper is to review publications relevant 3. Clinical presentation to this disease; highlighting its presentation, potential associations, documented underlying mutations, important dif- Steatocystoma multiplex manifests as numerous, recur- ferentials, histological findings and treatment trials. rent (Barone et al., 1988), commonly asymptomatic but might be pruritic (Senel, 2010) or painful if infected 2. Methodology (Marley et al., 1981), skin-coloured or yellowish- and rarely dark blue (Beyer and Vossmann, 1996) or calcified The author used EBSCOhost (includes the following (Hasibur Rahman et al., 2011), elastic or firm, cystic or databases: Academic Search Complete, CINAHL Plus dome-shaped (Setoyama et al., 1997), papules or nodules with Full Text, MEDLINE with Full Text and PsycINFO) (Duffy et al., 2011; Kaur and Kanwar, 2003). Yet, it can to search for ‘‘steatocystoma AND multiplex” on January present with unusual variants: suppurative (Gordon 9th, 2016. Fig. 1. Spratt et al., 2013; Adams and Shawayder, 2008), papular (Hansen et al., 1995), as steatocystoma multiplex conglobata (Gollhausen et al., 1988) or as a foreign body granuloma (Sina and Lutz-Nagey, 1984), and rarely, it may have a linear distribution (Almeida and Basso, 2009; Park et al., 2000)(Fig. 2). If pricked, yellowish fluid can be expressed (Kaur and Kanwar, 2003). The incidence of steatocystoma multiplex is equal across males and females. Though lesions start to develop in ado- lescence or early adulthood at an average age of 26 years (Cho et al., 2002) suggesting a possible hormonal trigger (Kromann et al., 2015); few reports documented unusual Figure 2. Linear unilateral steatocystoma multiplex on the ventral surface of the left underarm. onset in neonatal period (Lanćucki and Samochocki,

groin (Fig. 3), the scalp and the proximal extremities (Chu and Ronald, 2003). Yet, it may also affect the face, buttocks (Gordon Spratt et al., 2013; Kaur and Kanwar, 2003), breasts (Pollack and Kuerer, 1991; Aboody et al., 1992), unexpectedly the forearms (Pietrzak et al., 2015) (Fig. 4), and even more surprisingly, it can follow an acral distribution, suggesting a possible defected keratin 17 gene along with ectopic sebaceous glands (Marzano et al., 2012; Jain et al., 2013; Yanagi and Matsumura, 2006). Steatocystoma multiplex can be sporadic; however, familial presentations are well-documented in literature: over one generation (Harper and Davis, 1971), two generations (Lanćucki and Samochocki, 1989; Kromann et al., 2015; Kamra et al., 2013; Choudhary et al., 2010; Ichikawa et al., 2006), three generations (Noojin and Reynolds, 1948; Antal et al., 2012; Marzano et al., 2009), four generations (Kaur and Kanwar, 2003; Feinstein et al., 1983; Hollmig and Menter, 2010), and five generations (Pamoukian and Westreich, 1997); suggesting an autosomal dominant pattern of inheritance.

4. Concurrences/associations

Figure 3. Steatocystoma multiplex with testicular distribution. Steatocystoma multiplex can present, concurrently, with other diseases. (Table 1). Eruptive vellus hair cysts are seen commonly with steatocystoma multiplex. Given the name multiple pilosebaceous cysts (Yoshida et al., 2010; Ohtake et al., 1992; Cho et al., 2002), some researchers suggest that both diseases, in addition to persistent infantile milia (Patrizi et al., 1998) and epidermoid cysts (Ahn et al., 1996), are two extremes of one spectrum. They propose that the level of the sebaceous duct where the cyst originates determines the presentation (Patrizi et al., 1998). On the other hand, literature documented that epidermoid cysts express keratin 10; eruptive vellus hair cysts stains positive for keratin 17 and negative for keratin 10; whereas steatocystoma multiplex expresses both, keratin 10 and 17. Accordingly, other researchers suggest that steatocystoma multiplex and erup- Figure 4. Asymptomatic subcutaneous steatocystoma multiplex on the tive vellus hair cysts are two diﬀerent entities (Tomkova right forearm. et al., 1997).

5. Genetics 1989), middle-age (Kaur and Kanwar, 2003; Ferrandiz and Peyri, 1984; Uc¸mak et al., 2013; Hasibur Rahman et al., R94C mutation, a G ? A mutation, was detected in the 2011; Yanagi and Matsumura, 2006; Romanelli et al., base 428, 94(th) codon in keratin 17 gene of patients with 2002; Belincho´n et al., 1995; Ahn et al., 1997), and old- steatocystoma multiplex. Further analysis showed that age (Kumakiri and Yajima, 1991; Park et al., 2014; the mutated allele lacks enzyme cutting locus; thus, causing Riedel et al., 2008). Other suggested causes are trauma, an uncut band with 200 base pairs, while the specimens of infection and immunological reaction (Jain et al., 2013). normal controls showed only these two bands of 108 base Lesions can almost involve anywhere (Marley et al., pairs and 92 base pairs (Wang et al., 2001). 1981; Antal et al., 2012; Fekete and Fekete, 2010; Another report documented that a heterogeneous muta- Apaydin et al., 2000), commonly where pilosebaceous units tion, c.280C/T (R94C), located in exon 1 of the keratin 17 are well-developed: the trunk, the neck, the axillae, the gene was detected in 10 members with steatocystoma

Please cite this article in press as: AlSabbagh, M.M., Steatocystoma multiplex: A review, Journal of Dermatology & Dermatologic Surgery (2016), http:// dx.doi.org/10.1016/j.jdds.2016.02.001 4 laect hsatcei rs s labg,MM,Setcsoamlilx eiw ora fDraooy&Draooi ugr (2016), Surgery Dermatologic & Dermatology of Journal review, A multiplex: Steatocystoma M.M., AlSabbagh, as: dx.doi.org/10.1016/j.jdds.2016.02.001 press in article this cite Please Table 1 Reported cases of steatocystoma multiplex in concurrence with other presentations. Publication Characteristics Concurrence/association Ofaiche et al. (2014) 12-year-old, female, facial microcysts and axillary sebaceous cysts Pachyonychia congenita Patel et al. (2013) 12-year-old, female, facial papules Mumcuog˘lu et al. (2010) 16-year-old, female, cysts on the anterior chest, forehead, axillae, and around the knees Kanda et al. (2009) a. 36-year-old, female, axillary cysts b. 3-year-old, female (daughter of A), pilosebaceous cysts on the face c. 62-year-old, male (father of A), steatocystomas on trunk Lim et al. (1999) 30-year-old, male, nodules on back and neck Kim et al. (1998) 33-year-old, male, nodules over entire skin xxx–xxx (2016) xxx Surgery Dermatologic & Dermatology of Journal / AlSabbagh M.M. Hohl (1997) A family Smith et al. (1997) Two families Moon et al. (1994) Young adult, male Aoyagi and Ohnishi (1976) A family Vineyard and Scott (1961) – Yoneda et al. (2014) 48-year-old, female, yellowish papules over her entire body surface Polycystic kidney disease Nestor Cabrera et al. (2013) 29-year-old, female, neck cystic lesions Multiple eccrine and apocrine hidrocystomas, eruptive syringomas, and pilonidal cyst Angus et al. (2012) 32-year-old, male, scrotal sebaceous cysts Fournier’s gangrene Marzano et al. (2012) 19-year-old, male, steatocystoma on the presternal area Lichen planus Marzano et al. (2012) 26-year-old, female, abdominal steatocystoma multiplex Nodulocystic acne Harms, 1983 – Lee et al. (2011) 50-year-old, male, cystic nodules and alopecic patches on scalp Alopecic patch (trichotillomania) Hollmig and Menter (2010) 32-year-old, female, cystic, nodules on forehead, neck, chest, back, arms, labia majora and Hidradenitis suppurativa intergluteal region Gass et al. (2009) 17-year-old, male, papules of axillae, groins, neck and upper chest Partial non eruption of secondary teeth, thickening of palmar skin on pressure points, syndactyly of hands and feet Marzano et al. (2009) 17-year-old, male, nodules on the flexor surface of forearms Familial syringomas Sardana et al. (2002) 30-year-old, male, facial nodules Facial steatocystomas and occipital pilar cyst, and bilateral preauricular sinus Cambiaghi et al. (1998) 6-year-old, male, scattered cysts over body, mainly localised on trunk and proximal limbs Alagille syndrome and leukonychia Gianotti et al. (1997) 55-year-old, male, facial and trunk papules and nodules Trichoblastomas and trichoepithelioma Mochizuki-Osawa et al. – LEOPARD syndrome and hyperelastic skin (1995) Nagayama et al. (1992) 46-year-old, female, steatocystoma multiplex Intracranial dermoid Araki et al. (1991) 55-year-old, man, steatocystoma multiplex Familial hypobetalipoproteinaemia and cerebellar ataxia Lanćucki and Samochocki 21-year-old, sebocystomatosis involving trunk, upper extremities, neck and clavicular areas Spinal abnormalities and Schmorl’s nodules (1989) King and Lee (1987) Family over five generations Natal or defective teeth, or both McDonald and Reed (1976) Family over generations Sohn et al. (1980) Middle aged, male Multiple keratoacanthoma and rheumatoid arthritis Verbov (1972) 50-year-old, female, pre sternal steatocystoma multiplex Lichen planus and acrokeratosis verruciformis of Hopf Contreras and Costello (1957) – Embryonal hair formation http:// M.M. AlSabbagh / Journal of Dermatology & Dermatologic Surgery xxx (2016) xxx–xxx 5

Figure 5. (A) Low and (B) high power histological ﬁgures of steatocystoma multiplex showing sebocytes on the cyst wall.

multiplex from a single family, if compared with other cord, the remnant of the follicular infundibulum. The members without the disease (Liu et al., 2015). infundibulum contains sebocytes. Few areas of the cord A third report stated that two missense mutations, contain a lumen filled with cellular debris of keratinocytes, R94H and N92S, were detected in two Chinese families corneocytes, sebocytes, or trapped hairs (Plewig et al., with steatocystoma multiplex and were absent in the 1982). Classically, the epidermis appears normal with a controls. These two mutations are thought to disturb the mid-dermal, and very rarely a subcutaneous (Yanagi and formation and assembly of keratin intermediate filaments Matsumura, 2006) cyst. The cystic wall is lined by stratified (Wang et al., 2009). squamous epithelium, with sebaceous lobules found in or adjacent to the cystic wall. Cystic lumen is lined by a thick 6. Differential diagnosis homogeneous eosinophilic cuticle and the granular layer is absent (Marzano et al., 2012). Histopathological examina- Steatocystoma multiplex conglobata can be confused tion might also reveal multinucleated giant cells and lym- with hidradenitis suppurativa, acne conglobata phocytes in the cyst wall (Dong et al., 2014), and large (Gollhausen et al., 1988), and retinoid resistant acne vul- lining cells with granular cytoplasm adjacent to typical garis (Cunliffe, 2002). Metastasized pulmonary adenocarci- steatocystoma epithelial lining cells (Setoyama et al., noma to the skin might be misdiagnosed as steatocystoma 1997). (Fig. 5). multiplex (Tsai et al., 2014). And more importantly, steato- Some cysts lack the typical histopathological features of cystoma multiplex involving the breasts can be mistaken steatocystoma multiplex; hence, multiple biopsies should for other mammary lesions. Mammary steatocystoma mul- be obtained (Ferrandiz and Peyri, 1984). tiplex appears on mammogram as well-circumscribed Aspiration of lesions reveals yellowish-dirty fluid that, round radiolucent nodules with thin radio-dense rims; under the microscope, has crystalline structures, amor- and on ultrasound as intradermal hypoechoic nodule, isoe- phous debris and anucleate squames resembling cells choic nodule or lesions with mixed echoes and debris-fluid (Elhence et al., 2012). levels (Wan et al., 2012; Park et al., 2003). The combination of these imaging findings with a family history of steatocys- 8. Management toma multiplex and an extensive bilateral spatial distribution of nodules, confirm the diagnosis of steatocystoma There is no standard treatment for steatocystoma multi- multiplex (Park et al., 2003). Eruptive vellus hair, multiple plex. Management is usually guided by personal experience epidermoid cysts, lipomas, xanthomatosis, neurofibro- and literature. Different methods of treatment were matosis and syringomas are other possible differentials reported with the best outcomes documented with mini- (Naveen et al., 2012; Marzano et al., 2012; Khatu et al., mally invasive surgical procedures including radiofre- 2013; Perdondo et al., 1995; Elhence et al., 2012). quency; vein hook and mosquito forceps; and blades and artery forceps. Where these procedures used, follow up 7. Histopathology and cytology showed excellent cosmetic results in terms of no scarring and no recurrence. Where cryotherapy is used, documenta- Steatocystoma multiplex is a tumour of the nevoid duct tion of scarring, dyspigmentation and blistering were the and the sebaceous gland. It originates from sebaceous fol- main limitations. Laser therapy shows satisfactory out- licles. It is connected with the epidermis by an epithelial comes: minimal scarring and low recurrence rates. Table 2.

Table 2 Different Methods of Treatment, their Advantages and Disadvantages. Publication Method Advantage Disadvantage a. Medical management Apaydin et al. (2000) Isotretinoin Prevents new lesion formation Recurrence Moritz and Silverman (1988) Lesions may shrink after Good results limited to inflamed Schwartz and Goldsmith the discontinuation of isotretinoin lesions (1984) Flaring after initial improvement Statham and Cunliffe (1984) Rosen and Brodkin (1986) Friedman (1987) Senel (2010) Antibiotics Tetracycline and its derivatives are of benefit for suppurative variant of steatocystoma multiplex b. Surgical management Kanekura et al. (1995) Trepan with artery forceps Adams et al. (1999) Using number 11 blade to incise lesions and Does neither result in scarring nor artery forceps to remove the cystic wall recurrence Lee et al. (2007) Vein hooks with mosquito forceps Cost-effective Transient post-inflammatory No recurrence hyperpigmentation Feinstein et al. (1983) Excision with skin grafting Ichikawa et al. (2006) Tumour resection from inside with the frontal Holmes and Black (1980) hairline approach Du¨zova and Sentu¨rk (2004) Using a needle to puncture the cyst from one Excellent outcomes side followed by expression of its contents No recurrence Kamra et al. (2013) Radiofrequency used to make mini incisions Bloodless field Choudhary et al. (2010) to the cyst to express its contents along with its No scaring, post inflammatory wall hyperpigmentation or recurrence Fekete and Fekete (2010) Cryotherapy Scarring Senel (2010) Blistering Choudhary et al. (2010) Dyspigmentation Madan and August (2009) Poor cosmetic outcomes Kaya et al. (2001) Cautery used to puncture the cyst with Well tolerated Hypopigmentation contents being squeezed and wall cyst No recurrence Occasional depressions gasped and extracted with forceps Senel (2010) Thin needle aspiration Inexpensive Recurrence Oertel and Scott (1998) Well-tolerated No morbidity Egbert et al. (1979) Incision, drainage and electrocautery Beneficial for extensive and infected lesions c. Laser Bakkour and Madan (2014) Carbon dioxide laser alone or combined Combination: Madan and August (2009) with a surgical technique Significant clinical improvement Riedel et al. (2008) Minimal scarring Rossi et al. (2003) Low recurrence rate Kra¨henbu¨hl et al. (1991) Patients’ satisfaction Improved quality of life Moody et al. (2012) Two complementary lasers: a 1450-nm targeting the abnormal sebaceous glands and a 1550-nm targeting the dermal cysts Mumcuog˘lu et al. (2010) Er:Yag laser combined with subsequent Good toleration topical tetracycline ointment No scarring

9. Conclusion Acknowledgements

Steatocystoma multiplex is a benign disease. Three I acknowledge Dr Hiram Almeida Jr., the Associate mutations, c.280C/T (R94C), R94H and N92S, were Professor of Dermatology at Federal University of Pelotas detected. It is being treated successfully with laser, radiofre- and Catholic University of Pelotas, Brazil for sharing quency, and minimally invasive procedures. Figs. 2 and 5; Dr Swapna Khatu, the Associate Professor at Department of Dermatology, Smt. Kashibai Navale Conﬂict of interest Medical College and General Hospital in Pune for sharing Fig. 3; and Dr Teruki Yanagi from Department of Derma- Author declares no funding resources or conﬂict of tology at Hokkaido University Graduate School of Medi- interest cine, Japan for sharing Fig. 4.

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Please cite this article in press as: AlSabbagh, M.M., Steatocystoma multiplex: A review, Journal of Dermatology & Dermatologic Surgery (2016), http:// dx.doi.org/10.1016/j.jdds.2016.02.001