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Stay CALM and have Coffee: Café-au-lait macule (CALM)

Dr Sampurna Roy MD         


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             Cafe-au lait is French for "Coffee with milk"

Image : Freckle - Melanin is increased in the basal layer of the epidermis. Melanocytes are normal.

Image : Neurofibroma  Visit: Dermatopathology Quiz Case 183

Cafe-au-lait spots or macule (CALM) are uniform tan to dark brown macules.

These lesions vary in size from small freckle-like lesion to large patches.

Solitary CALMs are common birthmarks in normal neonates and their incidence rises in preschool-aged children.

Multiple Cafe-au-lait spots are a feature of Neurofibromatosis.

Histologically, the lesions are similar to freckles with hyperpigmentation of basal layer. Giant melanin granules can be seen in Cafe-au-lait spots in many patients with Neurofibromatosis.

Neurofibromatosis 1 occurs in 2 to 3 people per 10,000. 

Common clinical features are café-au-lait macules, neurofibromas, intertriginous freckling, Lisch nodules, and learning disabilities.

Optic and other gliomas, malignant peripheral nerve sheath tumors, and characteristic osseous lesions also can be present.

It is important to recognize whether the presence of multiple CALMs in a particular patient is normal or indicates an association with a multisystem disorder.


Further reading:

Common hyperpigmentation disorders in adults: Part I. Diagnostic approach, café au lait macules, diffuse hyperpigmentation, sun exposure, and phototoxic reactions.

The diagnostic value of café-au-lait macules.

Genetic modifiers of neurofibromatosis type 1-associated café-au-lait macule count identified using multi-platform analysis.

Neurofibromatosis 1: clinical manifestations and diagnostic criteria.

Multiple or familial café-au-lait spots is neurofibromatosis type 6: clarification of a diagnosis.


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Dr  Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)







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