|Year : 2015 | Volume
| Issue : 1 | Page : 42-44
Localized form of steatocystoma multiplex-partially suppurativa mimicking scrofuloderma
Neha Kawatra Madan, Karsing Patiri, Shailaja Shukla
Department of Pathology, Lady Hardinge Medical College, New Delhi, India
|Date of Web Publication||16-Jan-2015|
Neha Kawatra Madan
Department of Pathology, Lady Hardinge Medical College, New Delhi - 110 002
Source of Support: None, Conflict of Interest: None
Steatocystoma multiplex is a rare disorder of the pilosebaceous unit, which presents as multiple sebum containing subcutaneous cysts. Usually, the involvement is generalized;however, localized forms of the disease have been described. In exceptional cases, acute episodes occur associated with fever and suppuration when the term "steatocystoma multiplex suppurativum" is used. In some cases, the inflamed cysts rupture forming sinus tracts, which may undergo scarring. This clinical presentation with discharging sinuses may be easily mistaken for tuberculosis, especially in endemic countries. We herein report a patient with localized form of steatocystoma multiplex suppurativum where initially a diagnosis of scrofuloderma had been considered. Cytological and histopathological examination allowed a correct diagnosis to be established.
Keywords: Scrofuloderma, steatocystoma, suppuration
|How to cite this article:|
Madan NK, Patiri K, Shukla S. Localized form of steatocystoma multiplex-partially suppurativa mimicking scrofuloderma. J Sci Soc 2015;42:42-4
| Introduction|| |
Steatocystoma multiplex also known as "epidermal polycystic disease" and "sebocystomatosis" is an uncommon, benign disorder of the pilosebaceous unit. Patients with autosomal dominant inheritance pattern and sporadic cases have been described. It usually starts in adolescence or early adult life when the activity of the pilosebaceous unit increases. Characteristic clinical feature is the presence of multiple sebum containing dermal cysts. Common sites of involvement, include face, trunk, abdomen, and extremities.  Localized forms of disease have been described with the lesions being confined to scalp or genitalia.  "Steatocystoma multiplex suppurativum" is one of the rare variants wherein the cysts become inflamed and rupture forming sinus tracts, which may undergo scarring. Secondary bacterial infection can lead to malodorous discharge. 
The suppurative variant may be confused with severe nodulocystic acne, infected fibroadenoma and pyodermas including tubercular abscess/scrofuloderma.
| Case report|| |
A 40-year-old female patient presented with gradually enlarging bilateral axillary swellings of 10 years duration. There was a history of low grade fever associated with pain and discharge from the left axillary swelling for the past 2 months. On examination, 3-4 firm, mobile and nontender subcutaneous nodules ranging in size from 0.5 cm × 0.5 cm to 1 cm × 1 cm were palpable in the right axilla. Overlying skin was normal. A draining sinus was visible in the left axilla with scarred margins [Figure 1]a. Underlying the sinus were, 2-3 well-defined, firm and slightly tender nodules measuring 1 cm × 1 cm each. General physical examination was unremarkable. A possibility of tubercular lymphadenitis with scrofuloderma was considered and fine-needle aspiration cytology was performed.
Fine-needle aspiration cytology from right axilla yielded thick whitish cheesy material whereas yellow colored creamy material was obtained from the left side. Smears from right axillary swelling showed only acellular debris [Figure 1]b, whereas those from the left side showed acute and chronic inflammatory cells along with necrotic debris. Ziehl-Neelsen's stain for acid fast bacilli and periodic acid Schiff stain for fungus were negative. Excision biopsy was performed.
Hematoxylin and Eosin stained sections from the right axillary tissue showed multiple variable sized cysts lined by two to three layers of cuboidal to flattened cells. Few showed vacuolization appearing like sebaceous gland cells (sebocytes) [Figure 2]a. Some had crenulated hyaline inner lining. Furthermore, many of the cysts showed intraluminal acellular amorphous eosinophilic material with numerous cholesterol clefts [Figure 2]b. Biopsy sections from the left axilla showed similar cysts, with dense mixed inflammatory infiltrate comprising predominantly of lymphocytes, in close proximity to and destroying few of these cysts [Figure 2]c. Histopathological examination was consistent with a diagnosis of "steatocystoma multiplex suppurativum." As suppuration was evident in only few areas, a final diagnosis of "steatocystoma multiplex partially suppurativum" was rendered. Following excision, a course of broad spectrum oral antibiotics was administered to the patient and patient improved.
| Discussion|| |
Steatocystoma multiplex affects both sexes equally and without any racial predilection. The usual age of onset is adolescence or early adult life when the activity of the pilosebaceous unit increases due to hormonal influences, but it can present at any age with cases being reported at birth as well as in elderly.  Lesions present as multiple dermal cysts varying in size from 3 mm to 3 cm which are usually nontender and asymptomatic. During disease progression, acute episodes can occur associated with pain, fever and increase in size of the cysts associated with suppuration.
Rupture of cysts, discharge and secondary bacterial infection can occur, leading to the clinical presentation of steatocystoma multiplex suppurativum. Only a few cases of this variant have been reported in the past. Fekete and Fekete described a similar case of steatocystoma multiplex suppurativum in a 27-year-old male with multiple suppurative lesions in the axilla and buttocks, whereas Adams and Shwayder reported it in the lower extremity of a 21-year-old African American male. , In the autosomal dominant form of the disease, mutations are seen in keratin 17 (K17) gene, which are identical to those seen in pachyonychia congenital type 2 that are characterized by hypertrophic nail dystrophy.  The index patient as well as cases described by Fekete and Fekete and Adams and Shwayder did not show any nail changes. Sporadic forms of Steatocystoma multiplex have not been shown to be associated with K17 mutations.
On aspiration of these cysts clear oily liquid, yellow creamy or cheesy material is usually obtained, which on cytological examination shows smears with acellular material, sometimes with cholesterol crystals.  Histological examination shows well encapsulated cysts with crenulated hyaline inner lining. Furthermore, sebaceous glands may be seen close to the cyst wall. Clinical differential diagnoses are lipomatosis, fat necrosis, galactocele, and epidermal cyst. 
Steatocystoma multiplex suppurativum lesions usually demonstrate chronic inflammation, but sometimes may also show granulomatous inflammation with necrosis.  Suppurative variant may be confused with severe nodulocystic acne, infected fibroadenoma and pyodermas, including tubercular abscess. In the absence of a correct pathological diagnosis, these patients are often likely to receive inappropriate anti tuberculous medications for extended duration in tuberculosis (TB) endemic countries.
Treatment includes antibiotics and aspiration, which is diagnostic as well as therapeutic. Surgical removal was the most common method of treatment in the past but its role is limited in widespread lesions. Cryotherapy and CO 2 laser excision have been described as efficacious for localized variants. Combined treatment with antibiotics and isotretinoin has also been reported with good results. 
| Conclusion|| |
Steatocystoma multiplex is an uncommon disorder with varied clinical presentation. The suppurative variants of the condition can occur at any time during the course of the disease. Physicians need to be aware of this clinical entity as it can be easily mistaken for TB in endemic countries as occurred in the index patient. The presence of long standing history of asymptomatic nodular lesions should prompt toward consideration of this diagnosis. Cytological/histopathological examination is the cornerstone to establishing a correct diagnosis and appropriate management.
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[Figure 1], [Figure 2]