Steatocystoma multiplex: A case report of a rare entity

Steatocystoma multiplex is an uncommon benign skin disease, which typically manifests as numerous intradermal cysts that can be scattered anywhere on the body. Although usually asymptomatic, it can be significantly disfiguring. One type of steatocystoma multiplex is known to be associated with the autosomal dominant inheritance of a mutation in the gene coding for keratin 17 (KRT17). In such cases, it is often concurrent with other developmental abnormalities of the ectoderm-derived tissues, such as the nails, hair, and teeth. To the best of our knowledge, few cases have been reported of steatocystoma multiplex of the oral and maxillofacial region. This report describes a case of steatocystoma multiplex of both sides of the neck and multiple dental anomalies, with a focus on its clinical, radiological, and histopathological characteristics, as well as the possibility that the patient exhibited the familial type of this condition.

Esteatocistoma múltiple: reporte de caso / Steatocystoma multiplex: a case report

El esteatocistoma múltiple corresponde a una entidad cuya lesión elemental es el quiste cutáneo, lesión de carácter benigno y de muy baja frecuencia, en la mayoría de los casos su presentación es esporádico, originado en la unidad pilosebácea. Su principal diagnóstico diferencial corresponde al quiste velloso eruptivo. El diagnóstico se obtiene a través de la conjunción entre la clínica con la histopatología. Actualmente se dispone de varias alternativas para su tratamiento. En la literatura está descrita su asociación con el Síndrome de Gardner, el cual tiene un carácter autosómico dominante. Es posible encontrar: poliposis, osteomas, hipertrofía congénita del epitelio pigmentario de la retina y quistes cutáneo. Se presenta a continuación un caso de un paciente de sexo masculino de 17 años con un esteatocistoma múltiple.

Steatocystoma multiplex is a disorder of very low frequency whose principal characteristic is benign cutaneous cysts originating in the pilosebaceous unit. Most cases present sporadically. The differential diagnosis is principally eruptive villous hair cyst. Diagnosis is reached by considering both the clinical picture and the histology. There are several treatment alternatives. Its association with Gardner´s syndrome, an autosomal dominant trait, is described in the literature. It may be accompanied by polyposis, osteomas, congenital hypertrophy of the retinal pigment epithelium and cutaneous cysts. We present the case of a 17 year old male patient with steatocystoma multiplex.

Comparison of carbon dioxide laser versus high-frequency electroacupuncture in the treatment of steatocystoma multiplex: a self-controlled clinical study / 中华皮肤科杂志

Objective To compare the clinical efficacy and safety of carbon dioxide (CO2) versus high-frequency electroacupuncture based on the assistance of drilling and blunt dissection in the treatment of steatocystoma multiplex.Methods A randomized,self-controlled study was performed in 15 patients.The median line of the body served as the dividing line,and the lesions on the two sides of the median line of the body were randomly divided into 2 groups to be treated with CO2 laser or high-frequency electroacupuncture,which left micropathways into the cyst cavity.After the above treatment,contents in the cysts were squeezed out or drawn out with a mosquito clamp,and the cysts were bluntly dissected.The decrustation time,erythema subsiding time,adverse reactions and recurrence were compared among lesions at different sites or with different sizes.Results For the lesions at the same sites or with similar sizes after treatment,the decrustation time and erythema subsiding time were both significantly shorter in the CO2 laser group than in the high-frequency electroacupuncture group (both P ＜ 0.05).There were significant differences in the decrustation time and erythema subsiding time among lesions at different sites or with different sizes (all P ＜ 0.05).Mild pain occurred in the 2 groups,and no hematoma or secondary infection occurred.There were significant differences in the incidence of scar formation or pigmentation between the 2 groups (both P ＜ 0.05).During the follow-up of 6-18 months,relapse occurred in 1 patient in each group.Conclusion Compared with the high-frequency electroacupuncture,CO2 laser assisted by drilling and blunt dissection shows better efficacy in the treatment of steatocystoma multiplex,and it can remove lesions at various sites at one time.

Steatocystoma multiplex suppurativa: case report of a rare condition

Abstract Steatocystoma multiplex is a rare genetic disorder characterized by the presence of hamartomatous malformations at the junction of the pilosebaceous duct. It consists of encapsulated cystic lesions in the dermis, with adjacent sebaceous gland. When associated with inflammation, resembling hidradenitis, it is called steatocystoma multiplex suppurativa, a condition rarely reported. This is the first case of steatocystoma multiplex suppurativa reported in the Brazilian literature. Female patient, 23 years old, with papular and nodular cystic lesions that started in the armpits and groin, later spreading to the trunk, lower limbs, anticubital fossa, face and scalp. The presence of papular-nodular lesions associated with disseminated hidradenitis-like lesions in flexural areas and the histopathological diagnosis of steatocystoma defined the diagnosis of steatocystoma multiplex suppurativa.

Steatocystoma Multiplex Limited to the Vulva: Report of a Very Rare Case Successfully Treated by a Simple Surgical Method.

Aim: Steatocystoma multiplex (SM) is a rare malformation characterized by multiple cutaneous cystic lesions containing oily substance. SM has autosomal dominant mode of inheritance, though there are a significant number of sporadic cases reported as well. Characteristically, the lesions manifest around the time of puberty and are found on the sternal areas, face, trunk and scrotum. SM limited to the vulva is a very rare condition as there are only a five cases in the literature. Case: A 32-year-old woman presented with a 1 year-history of nodules, gradually increasing in size and number, on the labia majora. Similar lesions were not present on other parts of her body and family members. On physical examination, yellowish papules and nodules ranging in size from 2-15mm without punctum were present on the labia majora. Biopsy performed at the time revealed histological changes with steatocystoma multiplex. After a mini incision, evacuation of the cyst followed by the removal of the cyst wall was performed without complication. The lesions healed rapidly without scarring. The patient was followed by yearly controls. Recurrence was not observed until the fourth year of surgery. Smaller lesions were observed on previously untreated areas of vulva which were removed again successfully. Discussion: SM treatment is difficult. In addition to medical treatments including antibiotic and anti-inflammatory drugs and isotretinoin, there are different surgical treatments including total excision and grafting, cryotherapy and CO2 laser with limited success. Conclusion: This is a very rare case of SM limited to the vulva, simply and successfully treated by a simple surgical method, emphasizing the importance of cyst wall removal to achieve long time remission.

Pachyonychia Congenita Type II: A Case Report

Pachyonychia congenita type II is an autosomal dominant inherited rare genodermatosis characterized by dystrophic wedge shaped thickened nails with subungual hyperkeratosis, symmetric palmoplantar keratoderma, steatocystoma multiplex. Here we report a 23-year-old male with characteristic features of dystrophic nails, palmoplantar keratoderma, steatocystoma multiplex, follicular hyperkeratotic papules and history of natal teeth at birth.

A Case of Steatocystoma Multiplex Developed on the Labium Major / 대한피부과학회지

No abstract available.

A Case of Steatocystoma Multiplex Treated with Combination of Needle Aspiration and Trichloracetic Acid / 대한피부과학회지

Steatocystoma multiplex is a clinical condition characterized by numerous dermal cysts that have their origin in the pilosebaceous duct, which appear and grow at puberty. The cystic nodules range from 3 mm to 3 cm in size, and they are soft and freely movable. Although there are numerous reports of different treatment methods, the long-term management of steatocystoma multiplex remains to be difficult. We, herein, report on a case of 23-year-old man with steatocystoma multiplex on the buttock. He was successfully treated with dot peeling of 100% trichloracetic acid after aspiration of the oily contents without any complications, such as atrophy, scarring and skin necrosis. We report the combination therapy of needle aspiration and trichloroacetic acid peeling as new therapeutic modality for multiple steatocystoma multiplex.

Steatocystoma Multiplex Confined to the Scalp with Concurrent Alopecia

Steatocystoma multiplex (SM) is an uncommon disorder of the pilosebaceous unit characterized by the development of numerous sebum-containing dermal cysts which rarely involves the scalp. Here, we report a case of a 50-year-old man with multiple cystic nodules and alopecic patches on his scalp. On histopathological examination, the folded cyst was found to be lined by stratified squamous epithelium, while flattened sebaceous gland cells were identified in the cystic wall. Pigment casts were present in the hair papillae and perifollicular regions, suggesting trichotillomania as a possible cause of the observed alopecia. This case appears to represent an unusual clinical manifestation of SM.

Steatocystoma Multiplex Treated with a Suction-assisted Cartilage Shaver / 대한피부과학회지

Steatocystoma multiplex is a rare autosomal-dominant disorder characterized by asymptomatic multiple, skin-colored to yellowish nodules on the trunk, proximal extremities, and axillae. Although surgical excision is the most effective method, treatment is difficult when lesions occur in multiple numbers. Therefore, various treatments such as CO2 laser therapy, needle aspiration, and oral isotretinoin have been attempted, but results are variable. A 37-year-old man presented with multiple skin-colored subcutaneous nodules on the abdomen and both axillae. A histopathological examination was consistent with steatocystoma multiplex. The lesions were removed by dermal shaving with a suction-assisted cartilage shaver. It was an effective therapeutic method for multiple lesions with no significant adverse events. Herein, we report a case of steatocystoma multiplex treated with a suction-assisted cartilage shaver.

Successful Treatment of Steatocystoma Multiplex with OK-432 Sclerotherapy / 대한피부과학회지

Steatocystoma multiplex is a hamartomatous malformation of the pilosebaceous junction and this manifests as multiple, asymptomatic, yellowish or skin-colored cystic papules and nodules located most commonly on the upper anterior portion of the trunk, upper arms and axillae. Various treatment methods such as radical excision, simple surgery, CO2 laser, cryotherapy, needle aspiration and oral retinoids have been reported, but there is currently no standard treatment. OK-432 is a biological preparation consisting of lyophilized powder containing Streptococcus pyogenes Su strain cells (group A, type3) that were treated with benzylpenicillin potassium. This has been used for sclerotherapy in patients with lymphangioma. We herein report on a case of 36-year-old female with steatocystoma multiplex on the trunk and axillary area. She was successfully treated with intralesional infusion of OK-432 after aspiration of the oily contents without recurrence or any complications such as scarring, atrophy and skin necrosis. We suggest that this sclerotherapy is a cosmetically acceptable treatment option for the multiple lesions of steatocystoma multiplex.

A Case of Steatocystoma Multiplex Limited to the Scalp

We report here on a 63-year-old woman who had several small, yellowish papules on the scalp for the previous 2 years. There was no family history of similar lesions. Yellowish, creamy material was expressed from a papule during punch biopsy. Histologic examination from the lesion revealed the typical features of steatocystoma multiplex. We report here on this rare variant of steatocystoma multiplex that was limited to the scalp.

Treatment of Steatocystoma Multiplex on Whole Body: A Case Report

PURPOSE: Steatocystoma multiplex is a rare benign disease that occurred multiply on whole body surface. Many physicians have tried managing steatocystoma in variable methods. However it is hard to define the optimal way to cure steatocystoma. We performed both aspiration and excisional method to study the usefulness of both methods. METHODS: A 28-year-old woman has asymptomatic multiple subcutaneous nodules on whole body. Most lesions were aspirated with 26-guage needled 3cc syringe but large and purulent three nodules were excised. RESULTS: We diagnosed the lesion histologically as steatocystoma multiplex. Aspirated wound healed without scar, excised wound remained scar but esthetically acceptable. Axillary lesion contained so clustered type cysts that was difficult to aspirate whole cyst. Thus additional excisional method was needed. CONCLUSION: There are many practical methods to cure steatocystoma. However, there is no appropriate method to cure it. Therefore we should select different therapeutic method according to anatomical location and cyst size. Especially at subcutaneous fat-rich lesion like axilla and abdomen, it is better to excise the clustered cyst than to aspirate.

A Case of Steatocystoma Multiplex on the Forehead / 대한피부과학회지

Steatocystoma multiplex is a hamartomatous malformation of the pilosebaceous junction. It is thought to be hereditary or non-hereditary and rarely occurs on the face. We report a case of steatocystoma multiplex located only on the forehead of a 98-year-old woman. Histologic examination revealed a folded cyst wall consisting of several epithelial layers and flattened sebaceous gland lobules close to the cyst wall, which are relatively typical findings of steatocystoma multiplex.

A Case of Steatocystoma Multiplex: Successful Treatment with Mini-incisions

No abstract available.

Steatocystoma Multiplex in a Family

Steatocystoma multiplex is a rare, inherited disorder that is characterized by multiple, asymptomatic, variably sized dermal cysts. The condition is transmitted in an autosomal dominant fashion; although sporadic cases have been documented. Keratin 17 has been proposed to be an important factor in inherited steatocystoma. In this study, a 29-year old man has a 4-year history of asymptomatic, movable, skin-colored nodules on his face, neck, scalp, anterior chest and back. His father and elder-brother have similar lesions. Histologically, the cysts show a thin stratified squamous epithelium with sebaceous glands arising from its wall and an absence of the granular cell layer. Generally, there are two treatments-medical treatment and surgical treatment. In case of non- inflamed lesions, surgical excision or drainage is regarded as the best treatment. We tried excisional biopsy and until now there has been no recurrence in the operation area over the past 12 months following the operation.

A Case of Steatocystoma Multiplex on the Face / 대한피부과학회지

Steatocystoma multiplex is a hamartomatous condition of the pilosebaceous junction. It is thought to be hereditary or non-hereditary and occurs on the face rarely. We report a case of steatocystoma multiplex located only on the face in an 82-year-old woman. Histologic findings showed relatively typical findings of steatocystoma multiplex such as a folded cyst consisting of several layers of epithelial cells, palisading basal layer, absence of granular layer and outer hyalin-like cuticle.

A Case of Steatocystoma Multiplex on the Face in a Family / 대한피부과학회지

We describe a family with a two-generation history of steatocystoma multiplex with an unusual clinical manifestation. A 7-year-old boy had multiple asymptomatic, small, yellowish cysts on the face since birth. His father and 4-year-old brother also presented several similar persistent cysts localized to the face. This family had no other abnormality on physical examination. The cysts discharged oily yellow material when incised. Biopsy of several lesions disclosed that the cysts had typical features of steatocystoma multiplex histologically.

A Case of Eruptive Vellus Hair Cyst Combined With Steatocystoma Multiplex / 대한피부과학회지

Eruptive vellus hair cyst and steatocystoma multiplex are two clinically similar conditions. Most cases can be differentiated by histological examination. However, in some patients overlapping histological features have been described. We present herein a 23-year-old man with combined eruptive vellus hair cyst and steatocystoma multiplex. Histopathologically, the cyst shows feature of steatocystoma multiplex and eruptive vellus hair cyst simultaneously. This case suggests that eruptive vellus hair cyst and steatocystoma multiplex are within the same spectrum of the disease process.

A Case of Steatocystoma simplex / 대한피부과학회지

Steatocystoma simplex or solitary steatocystoma, first reported by Brownstein in 1982, is exceedingly rare. It is characterized by solitary, noninherited tumor in adults and seems to be a counterpart of steatocystoma multiplex with an identical histologic pattern. We describe a case of steatocystoma simplex in a 52-year-old woman who had a bluish papule on the nose. Histopathological findings showed a thin-walled cyst lined by stratified squamous epithelium with hyaline cuticle and sebaceous glands in or near the wall. We think that this is the first report of steatocystoma simplex in Korean literature.