Cutaneous cysts with nail dystrophy in a young female: A classical association |
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Cutaneous cysts with nail dystrophy in a young female: A classical association

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A 28-year-old female, who was married, presented with a history of multiple asymptomatic yellowish to skin colored cutaneous cysts over the face, neck, and sternal region for the past 7–8 years. 

The cysts started to appear over the anterior aspect of the neck which gradually spread over the other areas. Painful fissuring with crusting of plantar skin, particularly over pressure areas was also found.

Her 1.5-year-old male child also had a similar presentation over the plantar skin, with no skin-colored cutaneous cyst. They both had hypertrophic, thickened, brittle nails with typical blackish-brown discoloration and excess keratin deposition over the nail bed. 

She rendered history of having brittle nails since childhood affecting only toenails while her son had both finger and toenails affected since birth. She also rendered history of consanguineous marriage.

Multiple, smooth, well-defined, round to oval cutaneous cysts without a punctum, of diameter 2-5 mm, were seen on examination. The incision of the cyst revealed the presence of oily material inside.

No oral mucosal lesions were detected in both mother and child. 

No significant findings were rendered after systemic examination and family history details. KOH microscopy and culture of nail clippings were performed to rule out onychomycosis. Fine-needle aspiration cytology from the cutaneous cyst showed the presence of some squamous cells in an oily background. 

Steatocystoma and multiple epidermoid cysts were considered differential diagnoses and subjected to biopsy. Histopathological examination revealed keratinized, stratified, squamous epithelium lining. The dermis showed cyst lined by flattened, stratified, squamous epithelium, absence of granular layer, presence of a cellular eosinophilic cuticle over its surface and the sebaceous gland lobules approximating the cyst wall. 

She and her son were rendered the diagnosis of steatocystoma multiplex in PC-II (Jackson-Lawler type). Oral Isotretinoin 20 mg/day was prescribed along with and radiofrequency ablation of a few lesions of steatocystoma multiplex over cosmetically sensitive sites. She refused further surgical intervention. White soft paraffin cream was given for plantar keratoderma.

Source- Ghosh R, Chatterjee K, Barua JK, Roy A. Cutaneous Cysts with Nail Dystrophy in a Young Female: A Classical Association. Indian J Dermatol. 2017;62(6):661-664. doi:10.4103/ijd.IJD_473_16

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