Familial Disseminated Cutaneous Glomuvenous Malformation: Treatment with Polidocanol Sclerotherapy.
Glomuvenous malformations (GVMs) present as asymptomatic multiple pink-to-blue nodules or plaques. Disseminated lesions are rare, representing 10% of all the cases. Familial cases are caused by mutations in the glomulin gene. A young male presented with multiple bluish-to-dusky red-coloured nodules 10-15 in numbers over the trunk, limbs and buttocks since 12 years of age. They ranged in size from 1 to 3 cm, partially to non-compressible and tender on palpation. There was no history of any systemic complaint. His sister and mother had similar lesions but in a limited distribution. Biopsy showed multiple ectatic dilated vascular channels lined by multiple layers of glomus cells consistent with the diagnosis of GVM. The biopsy of the lesions from the mother and sister also showed similar features. Mutation analysis for glomulin gene could not be done because of the unavailability of the facility at our setting. He underwent sclerotherapy with 3% polidocanol every 2 weeks, and there was significant improvement in the lesions after six sessions of sclerotherapy. The patient is under follow-up and there is no recurrence of the lesions over treated sites after 6 months.
Department of Dermatology and STD, VM Medical College and Safdarjung Hospital, New Delhi, India.
Aditi
A
Jha
Department of Dermatology and STD, VM Medical College and Safdarjung Hospital, New Delhi, India.
Niti
N
Khunger
Department of Dermatology and STD, VM Medical College and Safdarjung Hospital, New Delhi, India.
K
K
Malarvizhi
Department of Dermatology and STD, VM Medical College and Safdarjung Hospital, New Delhi, India.
V
V
Ramesh
Department of Pathology, Indian Council of Medical Research, Safdarjung Hospital, New Delhi, India.
Avninder
A
Singh
Disseminated; familial glomuvenous malformation; polidocanol sclerotherapy