May 2018 | Case of the month

History

A 23 year old man was referred with asymptomatic skin changes on the left abdomen. These had evolved gradually dating back to the patient's teenage years. There had been no initial inflammatory episode. The patient was otherwise well.


Examination

Examination revealed multiple depressed areas of skin, that were slightly hyperpigmented, the skin was smooth and normal to touch. There were no other skin changes. 


Investigations

What is the diagnosis?


Images

Diagnosis

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The diagnosis is linear atrophoderma of Moulin (LAM).

LAM is a rare linear dermatosis. The disease usually presents in childhood or early adolescence as asymptomatic hyperpigmented atrophic linear bands along Blaschko's lines without prior inflammation or sclerotic appearance. LAM usually progresses as a linear atrophic lesion in the first few months; then the lesion ceases to progress and persists. There is no known effective treatment.

The differential diagnoses include atrophoderma of Pasini and Pierini and linear scleroderma, with the latter there is normally a history of preceding inflammation. 

Blaschko's lines (BL) represent a classic pattern of cutaneous mosaicism (two or more genetically different populations of cells existing side by side), occuring in a wide variety of congenital and acquired skin conditions. BL differ from dermatomes, and appear as single or multiple lines, whorls (swirls) and wave-like shapes in the skin. Numerous skin conditions can arise in BL.

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