April 2018 | Case of the Month

History

A 16 year old boy presented with redness and rough skin over his cheeks and eyebrows, along with some thinning of the eyebrows. This had come on gradually. No other family members were affected and the patient was otherwise well.


Examination

On examination there was fixed erythema of the cheeks and eyebrows, along with follicular plugging. The skin felt rough. There was also some hair loss of the lateral eyebrows. There was no hair loss from the scalp.


Investigations

What is the diagnosis?


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Diagnosis

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The diagnosis is keratosis rubra pilaris faciei atrophicans (syn. ulerythema ophryogenes), a variant of keratosis pilaris. 

Ulerythema ophryogenes is a relatively infrequent disorder that mainly affects children and young adults. It is usually sporadic, although cases inherited in an autosomal dominant pattern also are described. It is a more inflammatory form of keratosis pilaris, which has the following clinical findings:

  • Involvement of the face, in particular of the lateral eyebrows and cheeks
  • Prominent erythema
  • Follicular plugging
  • Progression to an atrophic stage with loss of hairs in the involved areas

There is little in the way of effective treatment.

For more information please refer to the chapter on keratosis pilaris.

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