Coexistence of Hidradenitis Suppurativa and Steatocystoma Multiplex: Is It a New Variant of Hidradenitis Suppurativa?

Hidradenitis suppurativa and steatocystoma multiplex may coexist in the same patient. The overlap of these 2 conditions could be suggestive of an unrecognized defect in follicular proliferation mutual in the pathogenesis of both conditions. Here we present 5 patients with both hidradenitis suppurativa and steatocystoma multiplex. Recognizing the overlap between these 2 conditions is important for accurate diagnosis, management, and identification of potential surgical candidates, as well as future basic science research.

Steatocystoma multiplex suppurativa associated with hidradenitis suppurativa successfully treated with adalimumab.

Steatocystoma multiplex (SM) is a rare autosomal dominant disease characterized by multiple asymptomatic intradermal true sebaceous cysts of variable size. These lesions may occasionally rupture, become inflamed, painful and heal with scarring [SM suppurativa (SMS)], thus sharing overlapping features with hidradenitis suppurativa (HS). The question of whether SMS simply mimics HS or if the two diseases may be associated remains open. We report three patients (2 M/1 F; age: 47, 27 and 40 years) affected by SMS showing inflammatory lesions on the areas typically affected by HS. Repeated skin biopsy from each patient showed true sebaceous cysts in some specimens and findings compatible with HS, without sebaceous glands evidence in others, supporting coexistence of the two diseases. Adalimumab at the initial dose of 160 mg (day 1), followed by 80 mg (day 15), and maintained at the dose of 40 mg every week from day 29 on, improved both HS and SMS lesions, including also non-inflammatory cystic lesions.

Coexistence of steatocystoma multiplex and hidradenitis suppurativa: Assessment of this unique association by means of ultrasonography and Color Doppler.

BACKGROUND: Steatocystoma multiplex (SM) is an uncommon skin disease manifesting as multiple sebum-containing cysts arising in pilosebaceous unit-rich body areas. Hidradenitis suppurativa (HS) is a debilitating chronic inflammatory disease affecting the apocrine gland-bearing skin and presenting with both pseudocystic and inflammatory nodules, abscesses and fistulas. Considering that genetics has been reported to play a role in both entities, the albeit rare association between them suggests a shared genetic background. Although histology remains the gold standard for the diagnosis of SM, ultrasonography can be an useful diagnostic tool. This method is largely used in combination with Color Doppler for assessing disease severity in HS. MATERIALS AND METHODS: We report three cases of coexisting SM and HS and describe the ultrasonography and Color Doppler features of the two entities. RESULTS: SM lesions appeared on ultrasonography as hypoechoic nodules with well-defined hyperechoic borders and posterior acoustic enhancement, in the absence of Color Doppler signal. HS lesions had the ultrasonographic features of the fistulas, abscesses and pseudocystic nodules, some of which including hair fragments, with an intense Color Doppler signal within or around inflamed lesions. CONCLUSION: The combination of ultrasonography and Color Doppler proved to be a reliable instrument for differentiating between SM and HS lesions, particularly distinguishing HS pseudocystic nodules from true cysts of SM.

Co-occurrence of steatocystoma multiplex, eruptive vellus hair cysts, and trichofolliculomas.

An association between steatocystoma multiplex (SCM) and eruptive vellus hair cysts (EVHCs) has been recognized. Steatocystoma multiplex and EVHC have similar clinical features but distinctive histologic features. Rare cases of co-occurrence of these conditions have been known to occur on the trunk and the forehead. We report a rare case of the simultaneous occurrence of SCM, EVHC, and trichofolliculomas localized to the forehead.

Steatocystoma multiplex with hair shaft abnormalities.

Steatocystoma multiplex (SM) is an unusual benign disorder of the pilosebaceous duct characterized by multiple cysts with little or no nail and hair involvement. We report a 30-year-old woman with multiple cystic nodules located on the neck, axillae and forearms as well as patchy scalp alopecia. Histopathological examination of the lesions was diagnostic of SM. Trichoscopy revealed pili torti and pili canaliculi. This patient represents an unusual clinical presentation of SM because of the presence of hair abnormalities.

Steatocystoma multiplex associated with bilateral preauricular sinuses.

Steatocystoma multiplex is characterized by the development of numerous steatocystomas. The condition has occasionally been related to congenital bilateral preauricular sinuses. Herein, we present the third case of such an association, a 34-year-old male who was born with bilateral preauricular sinuses that were surgically repaired. When he was 14 years old, he presented with multiple steatocystomas on his forehead and temples, one of which was biopsied. There was no family history of the condition.

Acral subcutaneous steatocystoma multiplex: a distinct subtype of the disease?

Steatocystoma multiplex (SM) is a hamartomatous malformation of the pilosebaceous duct consisting of dermal cysts filled with a sebum-like material. SM lesions are typically located in areas with sebaceous follicles, although atypical presentations involving sites lacking sebaceous follicles have exceptionally been described. We reviewed retrospectively a series of 32 histologically diagnosed SM observed in our department in the period 2006-2010, evaluating the kinds of lesions and their locations, and family history of SM and associated disorders, to focus on the clinical features of the acral subcutaneous variety of SM and to estimate its prevalence. We found five patients (four women and one man) with asymptomatic deep, skin-colored nodules on the flexor surfaces of distal upper extremities with a mean age at diagnosis and at disease onset of 32.5 and 26 years, respectively. The prevalence was 15%. All five cases were sporadic. The male patient had eruptive syringomas as an associated condition, together with a family history of this tumour. Acral subcutaneous SM may represent a distinct disease variety by virtue of its distinctive clinical features. Dermatologists should be aware of this form, which has to be included in the wide panel of diseases involving subcutaneous tissue.