Multisystem disorder of Punjabi children exhibiting spontaneous dermal and submucosal granulation tissue formation: LOGIC syndrome.

ABSTRACT

We describe a multisystem disease that affects children of Muslim families originating in the Punjab region of Pakistan and India. An altered cry due to vocal cord thickening, skin ulceration, nail abnormalities, and conjunctival scarring appear in the first few months of life. Progression and spread of the disease in these sites may be accompanied by involvement of other epithelial surfaces. The teeth may exhibit defective enamel formation. Histology reveals the formation of simple granulation tissue arising in the dermis and submucosa which become massively thickened and ulcerated. There is good evidence for an autosomal recessive gene defect, but the actual mechanism of the disease is not known. Medical and surgical therapy have been ineffective in altering the course of this devastating and usually fatal condition. We suggest the term LOGIC (laryngeal and ocular granulation tissue in children from the Indian subcontinent) for this newly established disease.