(Acantholysis bullosa; congenital tranumatic pemphigus)
Definition. A rare, inflammatory, bullous disease following a slight traumatism.
Symptoms. Most cases show a distinct history of heredity, the tendency being so pronounced that it has manifested itself in four or five generations. Some cases, however, do not show such a tendency but all usually develop in infancy or early childhood and persist indefinitely. Slight traumas such as rubbing, pressure or irritation may institute the eruption. Naturally, because of their location, the hands, feet, elbows, knees and anterior surfaces of the legs are often attacked. The eruption consists of small or large bullae, some few being partially hemorrhagic, appearing on parts of the surface subject to irritation, pressure or knocks. The mouth may be involved. Naturally the susceptibility is greater in some subjects than in others. Pigmentation and slight scarring from superficial ulceration may result, but many of the blebs disappear without a trace. If the finger ends are affected, there is often some atrophy and nail loss. There may even be alopecia. As a rule, the general health is not involved.
Etiology and Pathology. The true nature of the disease is not known. It has been asserted that it is not a distinct disease but a cutaneous conditions in which the individual possesses a congenital irritability of the vascular supply, causing response to every irritation. Degenerative changes in the rete have been noted also the absence of elastic tissue.
Prognosis and Treatment. When it is possible, the various irritating causal factors should be avoided but there has been no specific treatment used which has given satisfactory results. The X-ray, high-frequency currents and general galvanization have been urged. A case of my own failed to respond to long continued internal medication.