Ann Dermatol.  2011 Oct;23(Suppl 2):S185-S187. 10.5021/ad.2011.23.S2.S185.

Folliculosebaceous Cystic Hamartoma in a Patient with Neurofibromatosis Type I

• Noh S
• Kwon JE
• Lee KG
• Roh MR

• KMID: 2156785
• DOI: http://doi.org/10.5021/ad.2011.23.S2.S185

Abstract

Folliculosebaceous cystic hamartoma (FSCH) is a rare cutaneous hamartoma composed of dilated folliculosebaceous units and mesenchymal elements. It presents as a papule or nodule usually on the face and scalp, rarely on the genital or trunk area. Histologically, FSCH shares several similar features to sebaceous trichofolliculoma. We report one case of FSCH misdiagnosed as a neurofibroma. He was a 38-year-old man with a neurofibromatosis type I and a nodule on his left earlobe was excised under the impression of neurofibroma. Pathological examination revealed FSCH. Although FSCH is clinically not distinctive, awareness of the lesion is important to differentiate papulonodular or cyst-like cutaneous lesions.

Keyword

Folliculosebaceous cystic hamartoma; Neurofibroma; Sebaceous trichofolliculoma

MeSH Terms

Adult
Follicular Cyst
Hamartoma
Humans
Neoplasms, Basal Cell
Neurofibroma
Neurofibromatoses
Neurofibromatosis 1
Scalp
Skin Neoplasms
Follicular Cyst
Neoplasms, Basal Cell
Skin Neoplasms

Figure

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  Fig. 1 (A) A patient with neurofibromatosis type I. (B) A 0.8×1.2 cm sized skin colored nodule on his Lt. earlobe.

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  Fig. 2 (A, B) This hematoxylin-eosin-stained section shows dilated follicular structures, adjacent multiple sebaceous lobules and surrounding fibrovascular stroma (A: ×5, B: ×200).

Reference

1. Kimura T, Miyazawa H, Aoyagi T, Ackerman AB. Folliculosebaceous cystic hamartoma. A distinctive malformation of the skin. Am J Dermatopathol. 1991. 13:213–220.

2. Templeton SF. Folliculosebaceous cystic hamartoma: a clinical pathologic study. J Am Acad Dermatol. 1996. 34:77–81.

3. Lee SY, Lee DR, You CE, Park MY, Son SJ. Folliculosebaceous cystic hamartoma on the nipple. Am J Dermatopathol. 2006. 28:205–207.

4. Bolognia JL, Longley BJ. Genital variant of folliculosebaceous cystic hamartoma. Dermatology. 1998. 197:258–260.

5. Hamada M, Kiryu H, Satoh E, Moroi Y, Urabe K, Furue M. A case of genital folliculosebaceous cystic hamartoma with an unique aggregated manifestation. J Dermatol. 2006. 33:191–195.

6. Brücher JJ, Franke I, Ulrich J, Gollnick H, Leverkus M. Giant genital variant of folliculosebaceous cystic hamartoma: successfulmanagement by CO2 laser and acitretin therapy. Br J Dermatol. 2007. 157:833–835.

7. Schulz T, Hartschuh W. Folliculo-sebaceous cystic hamartoma is a trichofolliculoma at its very late stage. J Cutan Pathol. 1998. 25:354–364.

8. Ramdial PK, Chrystal V, Madaree A. Folliculosebaceous cystic hamartoma. Pathology. 1998. 30:212–214.

9. Sturtz DE, Smith DJ, Calderon MS, Fullen DR. Giant folliculosebaceous cystic hamartoma of the upper extremity. J Cutan Pathol. 2004. 31:287–290.

10. Tanimura S, Arita K, Iwao F, Kasai M, Fujita Y, Kawasaki H, et al. Two cases of folliculosebaceous cystic hamartoma. Clin Exp Dermatol. 2006. 31:68–70.

11. El-Darouty MA, Marzouk SA, Abdel-Halim MR, El-Komy MH, Mashaly HM. Folliculo-sebaceous cystic hamartoma. Int J Dermatol. 2001. 40:454–457.