Search

The clinical, light microscopic, and fine structural features of an unusual case of ichthyosiform dermatosis are presented. Clinically, this 44 year old Caucasian male had been affected by stable ichthyosis hystrix-like lesions on his extremities since infancy. In middle age, clinically different, but histologically similar lesions developed in the form of scaly plaques all over his trunk as a second phase. Microscopically, the epidermis consisted of abnormally large prickle cells, which faded without formation of a distinct granular layer into a thick and mainly orthokeratotic stratum corneum. The cytoplasm of the prickle cells showed division into inner and outer zones separated by a peculiar shell. While the exoplasmatic zone had fairly normal structures including well developed tonofilament-desmosome complexes, the inner zone was devoid of tonofilaments, but rich in mitochondria, endoplasmic reticulum, and other organelles. The shell appeared to consist of disorganized tonofilaments associated in higher layers with small keratohyalin granules. While no structured membranes divided the zones, there seemed to be a phase boundary which prevented keratohyalin granules and tonofilaments from entering the endoplasm. The term biphasic ichthyosiform dermatosis thus seems applicable on the clinical as well as on the fine structural level.

• Acquired ichthyosis was recognized in four patients, in whom the associated malignant disorders were carcinoma of the lung, breast, and cervix. This skin lesion may be a valuable clinical sign in recognizing the existence of a malignant condition. Although the emergence of an ichthyosiform dermatosis may have serious prognostic implications, it may also be useful in monitoring the course of the malignant disorder and the patient's response to therapy. The relationship to nonlymphoproliferative malignant neoplasms may be more common than has been previously recognized. ( Arch Dermatol 111:1446-1447, 1975)

Two sisters and two unrelated patients with an ichthyosiform dermatosis resembling nonbullous ichthyosiform erythroderma were found to have lipid accumulations in the granulocytes of the peripheral blood, in the granulocyte precursors in the bone marrow, and in the liver. In each case, the blood anomaly was readily detected on examining a peripheral smear. One patient showed many of the features seen in Refsum syndrome. This association of findings in four patients may constitute another neurocutaneous lipidosis.

Historical Considerations Porokeratosis is a disease characterized by sharply circumscribed, kerato-atrophic areas in the midst of unchanged skin occurring in sites of predilection. The lesions are usually multiple—rarely single; familial incidence is frequent, with males being affected twice as often as females. Although Majocchi1was the first to recognize porokeratosis as an entity (1883), naming it "linear and hystrix ichthyosiform dermatosis," his successor Mibelli described the condition in such detail (1893) that it bears his designation (porokeratosis). Independently of Mibelli, Respighi described it and named it "hyperkeratose figuree centrifuge atrophicante." Gilchrist, in the United States, emphasized the hereditary aspects of the dermatosis.1 Porokeratosis is a rare disease, yet Bopp2encountered 16 cases in one year. Although there are numerous reports of individual cases in the literature, detailed and exhaustive studies of the disease are nevertheless lacking. In their report published in 1943, Bloom and Abramowitz3prophesied