(Atrophoderma pigmentosum; Angioma pigmentosum et atrophicum; Dermatitis Kaposi; Lentigo maligna)
Definition – A rare chronic disease, beginning in early childhood and characterized by pigmentation, telangiectases, cutaneous atrophy and malignant tumors, usually ending fatally.
Symptoms – The lesions appear principally on those parts exposed to the sunlight, namely, the face, scalp, neck, upper shoulders, forearms, back of hands and lower extremities. Starting in the first or second year of life as a diffused and mottled redness usually of the face, neck and back of hands, plus a roughness of the skin, there soon develops small lentiginous, yellowish-brown pigmentations among which small telangiectases may appear. These pigmentations may represent the primary eruption. Though the lesions are variable disappearing at one point only to appear at another, they always increase in number and, after a few years of existence, small areas of atrophy appear corresponding to previous freckles or telangiectases. These latter often appear at the margin of the atrophic areas and may form angiomatous nodules. The parchment-like skin or xeroderma is caused by the increase in size of the atrophic areas forming thing, shrunken, dry, whitish patches. After a variable period of years, warty nodules appear and these are the seat of malignant growths. They increase in size, usually ulcerate, may bleed freely and, together with metastases to the lymph glands or other internal organs, cause the patient’s death from cancerous cachexia in two years or less from the time of development of the tumors. The final tumors occur chiefly upon the face and have been variously described as sarcomata, angiomata and carcinomata. Subjective sensations are lacking although the ulcerative lesions may be painful. Nearly a hundred cases of this peculiar disease have been reported. Its duration is from five to fifteen years, although some few cases have reached middle or later life.
Etiology and Pathology – Beyond the fact of hereditary and congenital influences, the causes of this disease are obscure. The condition often begins in summer and at an age when children are first apt to have the uncovered parts of the skin exposed to the sunlight. In fact many authorities regard sun- or wind-exposures as causal factors. In a number of instances several children in the same family have been attacked. Both sexes and those of far different circumstances are liable to the disease. Pathologically there are no distinctive changes. The findings correspond to the various stages of the disease, being similar to those found in lentigo, verruca, nevus pigmentosus, atrophy and degenerative growths.
Diagnosis – At an early stage the lesions might resemble the eruptions of measles, erythema solare, lentigo, nevi or urticaria pigmentosa, but the persistent though erratic course, limited distribution, absence of subjective sensations and other equally apparent differences should exclude these affections. It is hard to conceive how a well-defined case could be mistaken for any other affection although scleroderma, lupus vulgaris and macular leprosy might atypically simulate this disease.
Fig. 188 – Xeroderma pigmentosum in the pigmentary stage, presenting freckle- like lesions on face and neck. These spots are persistent, have slowly increased in number for the past five years and present no subjective sensations.
Prognosis and Treatment – The progress of the disease may be delayed, cancerous development prevented and life prolonged if seen in its early stages. After the disease is fully developed a fatal termination may be expected. Treatment, while not satisfactory, should not be neglected and may embrace protection of the surface from the sun’s rays and other irritants, the removal of the lesions by solidified carbon dioxid, excision or cauterization, the application of boric acid lotion to the frequently coexisting conjunctivitis, or the healing of ulcers, after curetting, by the application of a mild ointment such as ammoniated mercury. The X-rays should always be employed when possible, especially in the later stage of the disease, and internal medication may be indicated at any time.