Congenital cutaneous mastocytosis
ICD-10 Q82.2 is a billable code used to indicate a diagnosis of congenital cutaneous mastocytosis.
Congenital cutaneous mastocytosis is a rare skin disorder characterized by an abnormal accumulation of mast cells in the skin. This condition typically presents at birth or in early infancy and manifests as brownish-yellow macules, papules, or plaques that can vary in size and distribution. The lesions are often asymptomatic but may become itchy or urticarial upon physical stimulation. The condition is generally benign and may resolve spontaneously over time, although some cases can persist into adulthood. Diagnosis is primarily clinical, supported by histological examination showing an increased number of mast cells in the dermis. It is important to differentiate congenital cutaneous mastocytosis from other skin lesions and conditions, such as urticaria pigmentosa and other mast cell disorders, to ensure appropriate management and follow-up.
Pediatric documentation should include detailed descriptions of skin lesions, onset, and any associated symptoms. Family history of mast cell disorders should also be documented.
Common scenarios include newborns presenting with skin lesions, parents concerned about the appearance of the skin, and cases requiring referral to dermatology for further evaluation.
Special considerations include monitoring for potential systemic involvement and educating families about the benign nature of the condition.
Genetic documentation should include family history, any known genetic syndromes, and results from genetic testing if performed.
Genetic counseling may be needed for families with a history of mast cell disorders or when there is uncertainty about the diagnosis.
Considerations include understanding the genetic basis of mast cell disorders and the implications for family members.
Used when a biopsy is performed to confirm the diagnosis of congenital cutaneous mastocytosis.
Documentation must include the reason for the biopsy and the findings.
Dermatology may require specific details about the lesion's characteristics.
The prognosis for congenital cutaneous mastocytosis is generally good, as many cases resolve spontaneously during childhood. However, some patients may experience persistent lesions or develop systemic symptoms, necessitating ongoing monitoring and management.
