Localized forms of steatocystoma multiplex: Case report and review of the literatureDepartment of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. email@example.com
Hossein Mortazavi1, Arash Taheri, Parisa Mansoori, and Zahra Asadi Kani
Dermatology Online Journal 11 (1): 22
A 70-year-old man presents with multiple asymptomatic, yellow to skin-colored firm papules scattered on the scalp; the lesions had been present for about 30 years. Histological findings are consistent with the diagnosis of steatocystoma. We review the English-language literature of the published cases of localized forms of steatocystoma multiplex.
Steatocystoma multiplex is characterized by multiple, asymptomatic, slow-growing, firm, yellow to skin-colored, cystic lesions 2-3 cm in diameter, located principally on the arms, chest, axillae, and neck . In severe cases, the lesions may be generalized . Usually the overlying epidermis is normal with no central punctum present. The contents may be either a clear oily liquid or a white or yellow creamy or cheesy material [3, 4, 5]. The onset of steatocystoma multiplex tends to occur during adolescence or early adult life but has been described at birth and in persons who presented in their sixth decade [5, 6, 7]. It has an autosomal dominant mode of transmission in some cases .
|Multiple papules on the scalp.|
A 70-year-old man presents with multiple asymptomatic, round-to-oval, well-defined, smooth-surfaced, yellow to skin-colored, 5-11-mm diameter firm papules scattered on the scalp; the lesions have been present for about 30 years (Fig. 1). There are no similar lesions on the other parts of the body. The smaller papules are skin-colored and, when punctured, discharge a clear or milky, oily liquid (Fig. 2). The larger lesions are yellow and, when punctured, discharge a yellow, creamy-to-cheesy material (Fig. 3). On examination, he also has Hamilton type VIII androgenetic alopecia. There is no history of erythema, tenderness, or infection of the lesions. There is no family history of similar lesions.
|Figure 2||Figure 3|
|A clear oily discharge after puncture of a skin-colored cyst (Fig. 2).|
|A yellow creamy discharge after puncture of a yellow cyst (Fig. 3).|
|Histological examination of a yellow papule showing sebaceous gland lobules within the cyst wall.|
Biopsy specimens shows well-encapsulated dermal cysts with infolded walls lined by stratified squamous epithelium without a granular layer. Sebaceous gland lobules are found within the cyst wall. The smaller, skin-colored lesions have atrophic walls with from two to five layers of flat epithelial cells and empty lumina. The larger, yellow lesions have from three to five layers of cuboidal epithelial cells with a thin layer of crenulated, eosinophilic material on the luminal surface and a little eosinophilic horny material in some parts of the lumen (Fig. 4). No connection between the cyst walls and overlying epidermis is found in the serial sections.
In contrast with the typical steatocystoma multiplex patients, lesions are limited to the scalp in our case. There have been only a few cases with such a limited distribution [4 6, 9, 10] Table 1 presents a review of the English-language literature of the published cases of localized forms of steatocystoma multiplex. In the reported cases of localized steatocystoma multiplex, the lesions are confined to the head and neck or genitalia. The terms facial papular variant of steatocystoma multiplex and sebocystomatosis have been used to describe some of these localized forms as distinctive variants of the disease [3, 11, 12]. However, involvement of these areas is not infrequent in typical cases of steatocystoma multiplex . There are reports of the cases with steatocystoma multiplex involving predominantly the face or head and neck with scattered lesions on the trunk . The pathological and clinical features of the localized forms are not different from typical cases. Therefore, we believe that the localized forms of steatocystoma multiplex are not distinctive variants of the disease. Steatocystoma multiplex should be considered as a spectrum with different variations in anatomical distribution.
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