Soft pink nodules on the scalp.

A 56-year-old female presented with multiple asymptomatic nodules over her scalp of 10 years duration. Her family history was not contributory. On examination, the patient had multiple discrete to confluent, soft, pink-colored nodules, some of them pedunculated, over the occipital region of the scalp [Figure 1]. The nodules were non-tender and did not bleed to touch. The patient did not have skin lesions elsewhere, and her systemic examination was unremarkable. Her routine investigations were within normal limits. Skin biopsy from one of the nodules revealed multiple islands of tumor cells in the dermis of various shapes arranged in the form of a “jigsaw puzzle.” Higher magnification showed the islands of tumor cells to be composed of two types of cells and surrounded by a hyaline sheath. The outer layer had small cells with basophilic staining nuclei and peripheral palisading, while the inner cells were large with an ample cytoplasm and pale staining nuclei, and hyaline droplets were seen interspersed between the cells [Figure 2]. A final diagnosis of multiple cylindromas was made. The lesions were surgically excised.

Figure 1

Soft pink nodules on the occipital region of the scalp

Figure 2

Tumor island showing outer small cells with basophilic nuclei and palisading (lower arrow), inner large cells with pale nuclei (vertical arrow) and hyaline droplets (upper arrow), H and E, ×400

Cylindromas are benign skin appendageal tumors of unknown origin, though electron microscopy and immunohistochemistry have linked them to the intradermal coiled duct of the eccrine glands.[12] They usually present in the middle or old age with a female preponderance. They present with pink to red soft nodules on the scalp, while rarely the face, neck, and trunk may also be affected. They are usually solitary, but sometimes multiple tumors are seen, especially when they are familial. The tumors can sometimes be extensive, coalescing together to cover the entire scalp forming the so-called “turban tumor.” A combination of multiple cylindromas, trichoepitheliomas, and eccrine spiradenomas occurring on the scalp, with autosomal dominant inheritance, is denoted the “Brooke–Spiegler” syndrome.[3] Cylindromas may clinically mimic granuloma pyogenicum or angiomas due to their pinkish red color. However, cylindromas have a characteristic histologpathology, where the tumor islands are arranged closely and fitting together like a jigsaw puzzle. The tumor islands contain two types of cells and hyaline droplets, as seen in our case [Figure 2], surrounded by a hyaline sheath. In addition, tubular lumina lined by ductal cells may be seen. Malignant transformation is very rare in cylindromas.

The ideal treatment is surgical excision with grafting in cases of extensive tumors. CO2 laser, Erbium-YAG laser, cryotherapy, radiotherapy, and electrodessication are other modalities. Topical aspirin is known to prevent recurrences, due to its ability to down-regulate nuclear factor-kappa B expression.[4]