eISSN: 2299-0046
ISSN: 1642-395X
Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii
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vol. 37
Letter to the Editor

A case of late onset steatocystoma multiplex

Meng Jiang
Mengli Zhang
Heng Gu
Xu Chen

Adv Dermatol Allergol 2020, XXXVII (1): 117-118
Online publish date: 2018/10/15
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A 74-year-old woman presented with multiple nodules on her scalp which were asymptomatic and discovered incidentally 10 years previously. The patient did not receive any treatment since there had been no subjective symptoms. Over time, however, the lesions increased gradually both in size and number. The patient was systemically healthy and there was no history of hypertension, diabetes, hyperlipidemia or similar lesions in her family. Dermatological examination showed that multiple white and yellow cystic nodules were discretely distributed over her scalp. These nodules were dome-shaped, smooth-surfaced, well defined and varied in size from 5 to 10 mm in diameter. The lesions were slightly hard and confined to the scalp, with no inflammation around the nodules. No other lesions could be seen around the body and no abnormality could be found in her nail, teeth or hair (Figure 1). Histopathological examination from one of the nodules demonstrated multiple epithelial cysts in the dermis. The folded cystic wall was lined with stratified squamous epithelium without a granular layer. Sebaceous glands were present in the cyst wall (Figure 2). Based on the clinical and histopathological features, a diagnosis of steatocystoma multiplex was made. No treatment was performed because of the benign process of this disease and asymptomatic character of the lesions. She was followed up for 1 year and showed no improvement in the involved lesions.
Steatocystoma multiplex is an autosomal dominant genodermatosis characterized by the presence of multiple sebum-containing dermal cysts. It is regarded as a nevoid or hamartomatous malformation of the pilosebaceous duct junction [1, 2]. Steatocystoma multiplex tends to occur in adolescents or early adulthood with no predilection for sex. Clinically, steatocystoma multiplex presents as multiple, smooth, dome-shaped, skin-colored or yellowish cystic nodules of various sizes. The nodules are firm and asymptomatic. The prominent histopathological features of steatocystoma multiplex are dermal cysts composed of thin walls of stratified squamous epithelium without a granular layer. The inner surface of the cyst wall shows a wavy appearance lined with homogenized eosinophilic cuticles and sebaceous glands are usually present in the cyst wall [2]. It is reported that early-onset steatocystoma multiplex is mostly caused by a mutation in KRT17 [3].
Steatocystoma multiplex most often occurs on the chest, neck, axillae and...

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