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March 3, 1982

of birth. She was left with an exfoliative erythroderma and bilateral ectropion. Early biopsies of the exfoliative erythroderma were suggestive of psoriasis. The patient maintained a persistent scaling eruption, clinically consistent with a moderately severe but typical lamellar ichthyosis. She did experience a diminution of the generalized erythroderma and ectropion. On several occasions during the past few years, possibly in association with mild URIs and fever, she experienced episodic crops of pustular lesions. On one occasion, a biopsy specimen from a pustular lesion on the leg revealed a spongiform pustule of Kogoj. The most recent flare in July 1981 appeared as a generalized pustular eruption. This was her most severe flare to date. Cutaneous findings reveal palmoplantar keratoderma with mild overlying scales, erythema, and increased skin markings. There is fine scaling and erythema ofthe face, extremities, and trunk. There is no evidence of ectropion. Pustular lesions are not present. During hospitalization for her most recent pustular flare, the patient had a negative streptococcal screen, a WBC of 22,500 with 76 segs, 11 bands, 10 lyphs, and 3 monos. Hemoglobin, platelet count, and urinalysis were normal. Repeat CBC at the time of discharge revealed a normalization of the WBC. Initial biopsies taken 1 2/68, 8/69, and 6/70 revealed mild to moderate epidermal acanthosis with mild elongation of the rete ridges and papillae. There were spotty areas of parakeratosis and absence of the granular layer. An upper dermal, spotty, mostly lymphocytic perivascular inflammatory cell infiltrate was seen. These changes were interpreted as psoriasis. Subsequent biopsies taken 3/71, 11/74, 4/76, and 2/79 revealed epidermal acanthosis, slight hypergranulosis, and an overlying compact orthokeratotic keratin layer. The upper dermis demonstrated telangiectasia, slight edema, and a mild, chronic, mostly lymphocytic perivascular inflammatory cell infiltrate. These findings were consistent with lamellar ichthyosis. A biopsy taken during the early convalescent phase of the most recent pustular flare revealed changes consistent with pustular psoriasis. She has obtained good symptomatic control of the hyperkeratotic lesions with hydrophilic petrolatum and 5% lactic acid in a water washable base. Flares of pustular psoriasis have responded favorably to topical corticosteroids.