Autosomal recessive form is often associated with severe internal complications such as genitourinary and gastrointestinal diverticula, diaphragmatic hernia, and emphysema leading to cor pulmonale and death in first few years of life. Approximately half the cases of acquired CL are associated with preceding inflammatory eruption such as urticaria, eczema, erythema multiforme, vesicular eruption, as well as reactions to penicillin, penicillamine, or other drugs. In many cases of acquired CL, involvement of face and neck occurs first and progresses in a cephalocaudal fashion. Visceral involvement is common and has occurred in all reported patients with onset of acquired disease after the age of 20 years. X-linked recessive variant of CL is rare, with skin laxity, skeletal, and genitourinary tract abnormalities.
CL affects all races.
Acquired CL may develop at any age but often begins in adulthood. Patients complain of progressive laxity and sagging of skin as they notice development of loose, wrinkled skin. There may be a history of inflammatory process involving the skin preceding development of loose folds of skin. Patients may have a family history of skin disorder. There may be a history of prior penicillin or penicillamine use.