Dermatology

Cutaneous ciliated cyst(Cutaneous Mullerian cyst)

Cutaneous ciliated cyst (Cutaneous Mullerian cyst, ICD-9 code 706.2)

Are You Confident of the Diagnosis?

Cutaneous ciliated cysts are rare, benign cysts that are most commonly found on the lower extremities of young women. Most come to the attention of the patient with growth after puberty. Some may be tender, but many are asymptomatic.

Characteristic findings on physical examination

Cutaneous cilated cysts present as a subcutaneous nodule with no epidermal change or connection to the epidermis. There are no physical exam findings to differentiate a cutaneous ciliated cyst from many other subcutaneous nodules, including other cysts and lipomas

Expected results of diagnostic studies

Histopathologic evaluation reveals a uni- or multilocular cyst in the dermis or subcutis.As the name implies, the cyst wall consists of ciliated epithelium, which may be cuboidal, columnar, or pseudostratified (Figure 1, Figure 2). Papillary projections into the cyst lumen may be encountered. Mucous-containing cells are not seen.

Figure 1.

Cutaneous ciliated cyst. Low-power photomicrograph of a multilocular cyst with papillary projections.

Figure 2.

Cutaneous ciliated cyst. High-power photomicrograph demonstrating ciliated columnar epithelium.

While not necessary for diagnosis, immunohistochemical staining with CEA is negative within the epitheliuim while staining for estrogen and progesterone receptors and EMA is positive. Stains for myoepithelial cells are typically negative.

Diagnosis confirmation

Cutaneous ciliated cysts can be differentiated from other cysts containing ciliated epithelium (i.e. bronchogenic, branchial cleft, thyroglossal duct) by history, location and, frequently, by the lack of other cyst wall components and surrounding structures found in the others. Epidermoid and trichilemmal cysts do not contain ciliated epithelium.

Who is at Risk for Developing this Disease?

Cutaneous ciliated cysts are almost always found in young postpubertal women in the second or third decade.

What is the Cause of the Disease?

Pathophysiology

While the pathogenesis of cutaneous ciliated cysts remains controversial, most authors believe that they arise from persistent and ectopic Mullerian epithelium. Mullerian heterotopia is suggested by the cyst’s morphologic and immunohistochemical similarity to fallopian tube epithelium (see above). Ciliated metaplasia of eccrine glands has also been proposed and may account for the few cases arising in unusual locations (ie, the feet) and in men, and these cases may show an immunohistochemical profile similar to eccrine glands.

Systemic Implications and Complications

Cutaneous ciliated cysts have no known systemic implications.

Treatment Options

Cutaneous ciliated cysts are benign. If a patient desires treatment, surgical excision is the only option.

Optimal Therapeutic Approach for this Disease

Complete surgical excision is the only treatment option and is necessary to make the diagnosis.

Patient Management

Patients with a history of a cutaneous ciliated cyst require no long-term management.

Unusual Clinical Scenarios to Consider in Patient Management

The differential diagnosis for an aymptomatic or tender subcutaneous nodule is broad. Surgical excision and histopathologic evaluation are required to make a specific diagnosis.

Cases of cutaneous ciliated cysts have been reported in men and in atypical locations, including the feet, inguinal area, abdominal wall, back and head. While the pathogenesis of many of these cases may differ from the more typical cysts arising on the legs of young women, there is no indication that they are anything but benign.

A few rare reports of a mixture of appocrinne and cutaneous cilitated cysts have been reported.

What is the Evidence?

Al-Nafussi, AI, Carder, P. "Cutaneous ciliated cyst: a case report and immunohistochemical comparison with fallopian tube". Histopathology. vol. 16. 1990. pp. 595-8.

(These authors describe a cutaneous ciliated cyst with a histochemical comparison to fallopian tube epithelium. They suggest a Mullerian derivation for cutaneous ciliated cysts.)

Bivin, WW, Heath, JE, Drachenberg, CB, Strauch, ED, Papadimitriou, JC. "Cutaneous ciliated cyst: a case report with focus on Mullerian heterotopia and comparison with eccrine sweat glands". Am J Dermatopathol. vol. 32. 2010. pp. 731-4.

(The authors report a cutaneous ciliated cyst occurring on the leg of a teenage girl and perform a microscopic, immunohistochemical and ultrastructural comparison of the cyst with nearby eccrine glands. They conclude that cutaneous ciliated cysts do not arise from eccrine glands and suggest a Mullerian origin.)

Chong, S-J, Kim, S-Y, Kim, H-S, Kim, GM, Kim, SY, Jung, J-H. "Cutaneous ciliated cyst in a 16-year-old girl". J Am Acad Dermatol. vol. 56. 2007. pp. 159-60.

(This is a report of a typical cutaneous ciliated cyst arising on the thigh of a young female. The authors present an immunohistochemical staining pattern suggestive of Mullerian derivation.)

Farmer, ER, Helwig, EB. "Cutaneous Ciliated Cysts". Arch Dermatol. vol. 49. 1978. pp. 70-3.

(This is the original report of 11 cases of cutaneous ciliated cysts evaluated at the Armed Forces Institute of Pathology. The authors note the resemblance of the cyst epithelium to that of fallopian tube and also propose the name “cutaneous ciliated cyst.”)

Fontaine, DG, Lau, H, Murray, SK, Fraser, RB, Wright, JR. "Cutaneous ciliated cyst of the abdominal wall -- a case report with a review of the literature and discussion of pathogenesis". Am J Dermatopathol. vol. 24. 2002. pp. 63-6.

(These authors describe a cutaneous ciliated cyst, with an immunohistochemical staining profile suggestive of Mullerian derivation but arising in an unusual location.)

Lee, JS, Kim, YC, Lee, ES. "Cutaneous ciliated cyst of the inguinal area in a man". J Dermatol. vol. 2. 2006. pp. 146-9.

(These authors report a cutaneous ciliated cyst arising in a man. They describe an immunohistochemical staining pattern that differs from that of the more common cysts arising on the legs of young women and suggest an eccrine gland origin for their case.)

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