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2025 ICD-10-CM code D47.01

Cutaneous mastocytosis is a skin condition characterized by the accumulation of excessive mast cells, which are white blood cells that produce histamine.

Use additional codes to identify any associated functional activity.Do not use this code for congenital cutaneous mastocytosis (Q82.2) or extracutaneous mastocytoma (D47.09).

Medical necessity for diagnostic testing and treatment is established by the clinical presentation and confirmation of cutaneous mastocytosis. The specific treatment approach should be justified based on the severity of symptoms and the potential for systemic involvement.

Diagnosis involves patient history, physical examination, and laboratory tests like CBC, liver function tests, and genetic testing for KIT mutations. Skin biopsy is essential for confirmation. Treatment may include antihistamines, topical steroids, and epinephrine for anaphylaxis risk.

In simple words: Cutaneous mastocytosis involves too many mast cells in the skin, causing itchy patches, blisters, and sometimes more serious problems. The most common type affects infants and children.

Cutaneous mastocytosis encompasses various forms, including urticaria pigmentosa (maculopapular cutaneous mastocytosis), diffuse cutaneous mastocytosis, solitary mastocytoma, and telangiectasia macularis eruptiva perstans. Each form presents with distinct skin manifestations, ranging from itchy brown patches to diffuse skin thickening.Symptoms may also include blistering, flushing, and systemic reactions like low blood pressure and anaphylaxis in severe cases.

Example 1: A 6-month-old infant presents with multiple small, reddish-brown spots on the skin that become raised and itchy when rubbed. The diagnosis of urticaria pigmentosa (maculopapular cutaneous mastocytosis) is made based on clinical presentation and biopsy findings., A 2-year-old child exhibits diffuse thickening of the skin, giving it an "orange peel" appearance. Biopsy confirms the diagnosis of diffuse cutaneous mastocytosis., An adult develops persistent brown skin patches and telangiectasias (spider veins). The diagnosis of telangiectasia macularis eruptiva perstans is confirmed, and systemic involvement is ruled out.

Documentation should include detailed clinical findings, biopsy results, and any systemic symptoms. Genetic testing results, if performed, should also be documented. Specific details regarding the type and extent of skin involvement are crucial.

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