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Steatocystoma Multiplex EJA cop somm_Stesura D’Alessandro 09/07/12 17:06 Pagina 1 Volume 3 - Number 1/2012 EJA cop somm_Stesura D’Alessandro 09/07/12 17:06 Pagina 3 EuVropoelaun mJouern a3l ,o fN Acunem anbd eRrel a1te/d2 D0is1ea2ses Volume 3, n. 1, 2012 The 6th edition of Acne Day will take place Therefore it has to be considered in the beautiful setting of Naples, a complex medical, aesthetical, at Palazzo Salerno Army Officers Club, Giuseppe Monfrecola psychological and social problem. on 14-15 September 2012. The Acne Day 2012 represents The meeting, sponsored by a good opportunity to discuss about the Italian Acne Board, has been organized the many faces of acne by Giuseppe Monfrecola, Dept. of Dermatology and in particular University of Naples Federico II. the newest knowledges about Acne, one of the most frequent skin disorders, its pathogenetic mechanisms strongly influences the quality of life and therapeutic approaches useful of adolescents and young adults. for active phases, maintenance and scars. 3 EJA cop somm_Stesura D’Alessandro 09/07/12 17:06 Pagina 5 Volume 3, Number 1/2012 Editorial Board Content Steatocystoma multiplex: a case report pag 5 Editor Elena Guanziroli, Franco Greppi, Mauro Barbareschi Stefano Veraldi Milano Refractory SAPHO syndrome in association Co-Editor with resistant E. coli cutaneous infection pag 9 Mauro Barbareschi Milano Caterina Fabroni, Carla Cardinali, Antonia Gimma, Giovanni Lo Scocco Scientific Board Vincenzo Bettoli Ferrara Treatment of comedonal-papular, mild acne with Stefano Calvieri Roma a fixed combination of hydroxypinacolone retinoate, Gabriella Fabbrocini Napoli retinol glycospheres and papain glycospheres. Giuseppe Micali Catania Preliminary results of a sponsor-free, pilot, open, Giuseppe Monfrecola Napoli multicentre study pag 13 Nevena Skroza Roma Stefano Veraldi, Rossana Schianchi Annarosa Virgili Ferrara Managing Editor Antonio Di Maio Milano Italian Acne Club Mario Bellosta (Pavia), Enzo Berardesca (Roma), Carlo Bertana (Roma), Alessandro Borghi (Ferrara), Francesco Bruno (Palermo), Maria Pia De Padova (Bologna), Paolo Fabbri (Firenze), Carlo Pelfini (Pavia), Mauro Picardo (Roma), Maria Concetta Potenza (Roma), Marco Romanelli (Pisa), Alfredo Rossi (Roma), Patrizio Sedona (Venezia), Riccarda Serri (Milano), Aurora Tedeschi (Catania), Antonella Tosti (Bologna/Miami), Matteo Tretti Clementoni (Milano) International Editorial Board Zrinka Bukvic Mokos (Zagreb, Croatia), Tam El Ouazzani (Casablanca, Morocco), May El Samahy (Cairo, Egypt), Uwe Gieler (Giessen, Germany), Marius-Anton Ionescu (Paris, France), Monika Kapinska Mrowiecka (Cracow, Poland), Nayera Moftah (Cairo, Egypt), Nopadon Noppakun (Bangkok, Thailand), Gerd Plewig (Munich, Germany), Robert Allen Schwartz (Newark, Usa), Jacek Szepietowski (Breslau, Poland), Shyam Verma (Ladodra, India). Editorial Staff Direttore Responsabile: Pietro Cazzola Consulenza grafica: Piero Merlini Direttore Generale: Armando Mazzù Impaginazione: Stefania Cacciaglia Registr. Tribunale di Milano n. 296 del 01/06/2011. È vietata la riproduzione totale o parziale, con qualsiasi mezzo, Scripta Manent s.n.c. Via Bassini, 41 - 20133 Milano di articoli, illustrazioni e fotografie senza l’autorizzazione scritta dell’Editore. Tel. 0270608091/0270608060 - Fax 0270606917 L’Editore non risponde dell’opinione espressa dagli Autori degli articoli. E-mail: [email protected] Ai sensi della legge 675/96 è possibile in qualsiasi momento Abbonamento annuale (3 numeri) Euro 50,00 opporsi all’invio della rivista comunicando per iscritto Pagamento: conto corrente postale n. 20350682 la propria decisione a: intestato a: Edizioni Scripta Manent s.n.c., via Bassini 41- 20133 Milano Edizioni Scripta Manent s.n.c. Stampa: Arti Grafiche Bazzi, Milano Via Bassini, 41 - 20133 Milano 01 Guanziroli art_Stesura D’Alessandro 09/07/12 17:09 Pagina 5 European Journal of Acne and Related Diseases Volume 3, n. 1, 2012 Elena Guanziroli, Franco Greppi, Mauro Barbareschi Department of Anaesthesiology, Intensive Care and Dermatological Sciences, Università degli Studi di Milano, Fondazione IRCCS, Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Steatocystoma multiplex: a case report SUMMARY i l o r i z n Steatocystoma multiplex (SM) is a squamous epithelium lines the intradermal cysts. a u G rare inherited or sporadic disorder Sebaceous gland or atrophic sebaceous elements are a n e l characterized by multiple, round, constituently present adjacent to or within the cyst wall. E firm, yellow to skin-colored, mobile Various treatment options include surgical excision, CO2 cystic papules and nodules of variable size. laser therapy, cryotherapy, oral antibiotics, and oral They are usually distributed on chest, but can also isotretinoin. involve the entire trunk, face, scalp, arms, axillae, thighs Here, we present a patient with asymptomatic, skin-col- and, more rarely, the genitalia. Incision of the cysts ored nodules on the chest. They were histopathologically releases an oily, milky or yellowish, odourless fluid, or diagnosed as SM. cheesy, solid material. Because the patient had no cosmetic or functional com- On histological examination, a thin wall of stratified plaint, no treatment was considered. Key words: Steatocystoma multiplex, Eruptive vellus hair cyst. Introduction Steatocystoma multiplex (SM) is an face of the epithelial cyst wall 5. uncommon cutaneous disorder that is often inher- Non-inflamed lesions are asymptomatic and except ited as an autosomal dominant trait, resulting from for cosmetic problems, there is no need for treat- a mutation of keratin 17 on chromosome 17 1. ment. Onset is usually around adolescence or early adult life, when the activity of pilosebaceous glands increases 2. Case report It is characterized by multiple cutaneous cystic lesions 1-30 mm in size, which are most common- A 43 year-old man presented with an 28 ly found on the trunk, neck, axillae, and inguinal year history of multiple, asymptomatic nodules on region 3. SM should be differentiated from other the chest. The lesions had first appeared when he cystic lesions such as eruptive vellus hair cysts, was an adolescent and had gradually increased in epidermoid cysts, hidrocystomas, and persistent size and number. milia, but these conditions are often clinically very There was no family history of note. similar, and histological examination is needed to Skin examination showed multiple, smooth, skin- make a correct diagnosis 4. The distinctive features colored, freely movable nodules of soft consisten- of SM are the intramural sebaceous glands, the cy on the sternal region, which varied in size absence of a granular layer, and the crenulated sur- between 8 mm and 1.5 cm (Figure 1). 5 01 Guanziroli art_Stesura D’Alessandro 09/07/12 17:09 Pagina 6 European Journal of Acne and Related Diseases Volume 3, n. 1, 2012 Figure 1 Figure 1 Clinical aspect of steatocystoma multiplex. Figure 2 Figure 3 Figure 2 Figure 3 An enfolded cyst wall consisting of two or three layers The crenulated surface of the epithelial cyst wall. of squamous epithelium. 6 01 Guanziroli art_Stesura D’Alessandro 09/07/12 17:09 Pagina 7 European Journal of Acne and Related Diseases Volume 3, n. 1, 2012 A single nodule of the upper chest was excised. Steatocystoma also occurs as a solitary, noninher- Biopsy specimens revealed a well-encapsulated ited tumor referred to as steatocystoma simplex 13. cyst with an enfolded wall consisting of a thin Histopathologically, steatocystomas are well- layer of stratifying epithelium characterized by a encapsulated dermal cysts with enfolded walls crenulated surface, without a granular layer and lined by stratified squamous epithelium without a without hairs in the lumen (Figure 2-3). granular layer. These histological findings were consistent with a Sebaceous glands, either associated with the cyst diagnosis of SM. or within its wall, are routinely detected. We informed the patient on the benign nature of Occasionally, vellus hairs fragments and amor- the lesions and we illustrated to him the treatment phous keratin material may be found in the cyst possibilities that could be applied in case the cysts space which usually appears empty 3, 5. would become an aesthetic or functional problem It has been reported in association with pachyony- for him. chia congenita, acrokeratosis verruciformis, hyper- trophic lichen planus, hypohydrosis, hypothy- roidism, hidradenitis suppurativa, ichthyosis, hypo- Discussion trichosis, and multiple keratoacanthomas 14. Differential diagnosis include eruptive vellus hair SM is a nevoid tumor of the sebaceous cysts, epidermoid cysts, persistent milia, acne con- gland duct and acini 6. globata, hidradenitis suppurativa and beard pseu- It is thought to be inherited in an autosomal domi- dofolliculitis 9. nant fashion, but many sporadic cases have also Occasionally, multiple adnexal tumors, especially been reported 2, 7. if cystic, such as hidrocystomas, may be identical. Mutation of keratin 17, a protein found in several Its relation with eruptive vellus hair cysts has been epithelial structures such as ungual lay, hair folli- a matter of debate. These two diseases have some cles and sebaceous glands has been associated with overlapping features such as clinical appearance familiar SM, as well as with paronychia congenita and mode of inheritance, and moreover, there are type 2 1. reports of hybrid forms showing histological fea- Onset occurs during adolescence or early adult life tures of both SM and eruptive vellus hair cysts, but it has also been described at birth and in
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