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Background Hydroxyurea is an antitumor agent used to treat chronic myeloproliferative disorders. Leg ulcerations have been reported in patients undergoing long-term hydroxyurea therapy for myeloproliferative diseases. To better define this dermatological adverse effect of hydroxyurea therapy and to try to understand the pathophysiological process of this disease, we collected medical information for such patients in a multicenter retrospective study. Observations Forty-one patients (mean age, 67 years) developed leg ulcerations while undergoing hydroxyurea therapy (mean therapy duration, 5 years). The sex ratio was 1, and there was no underlying vascular disease. Hematologic abnormalities were identified. Complete recovery from the ulcerations occurred quickly after withdrawal of treatment in 33 (80%) of the cases. Conclusions This longest-reported series of patients confirms the role of hydroxyurea therapy in the onset of leg ulcerations. Healing or improvement requires cessation of treatment. Cutaneous atrophy and impaired wound healing may explain the relationship between hydroxyurea and leg ulcers. In addition, the megaloblastic erythrocytes resulting from the presence of hydroxyurea may circulate poorly through the capillary network. A prospective study in hematologic centers would be valuable.

Background: Joint and nail involvement in psoriasis is relatively common. In contrast, bony involvement of the terminal phalanx under a psoriatic nail is rare. We report on this psoriatic onycho-pachydermo-periostitis and suggest pathophysiologic mechanisms. Observations: Two new cases are reported and the data are compared with the data from eight similar cases reported in the literature. All subjects presented with similar changes— onychopathy, soft-tissue thickening, and radiologic features consisting of bone erosions and a periosteal reaction of the terminal phalanx. Of the 10 patients, two had no history of psoriasis before a diagnosis of psoriatic onycho-pachydermo-periostitis was made. The inflammation is likely transmitted from the psoriatic nail to the adjacent underlying bone by the same mechanism as in enthesopathies. Conclusion: Psoriatic onycho-pachydermo-periostitis should be recognized as a specific entity in the spectrum of psoriatic disease. (Arch Dermatol. 1996;132:176-180)

Background and Design: p53 overexpression has been reported in systemic lymphoproliferations, but our study is the first large series, to our knowledge, dealing with p53 expression in primary cutaneous lymphomas. p53 immunohistochemical detection using DO7 monoclonal antibody was performed in 54 cutaneous lymphoproliferative disorders, including 37 primary cutaneous T- or B-cell lymphomas. Results: No expression of p53 was found in 14 mycosis fungoides and seven pleomorphic T-cell lymphomas of either low or high grade of malignancy. Furthermore, no p53-positive cells were shown in low-grade B-cell lymphomas and in benign chronic dermatoses. p53 overexpression was seen over lymphomatous cells in 10 cutaneous lymphomas of high grade of Tor B-cell origin with two immunoblastic, three centroblastic, and five anaplastic lymphomas. Five of them occurred in human immunodeficiency virus—infected individuals, suggesting a possible interaction between p53 and viral proteins. p53 immunoreactivity was found over Reed-Sternberg—like cells in two cases of lymphomatoid papulosis. A labeling of keratinocytes located mainly in basal cell layers of the epidermis was observed in only five cases regardless of the lymphoma histologic subtype. Conclusions: p53 overexpression was not found in the most frequent primary cutaneous lymphomas, epidermotropic T-cell lymphomas. Alternatively, p53 immunoreactivity was observed in most primary cutaneous lymphomas of high grade of malignancy occurring in individuals either infected or not infected by human immunodeficiency virus. Molecular studies will determine if p53 overexpression is associated with p53 gene alteration and help to understand the role of p53 in primary cutaneous lymphoma genesis. (Arch Dermatol. 1995;131:1019-1024)

Background: In the course of mycosis fungoides, pilofollicular manifestations without mucinosis (papules, keratoses, comedones, or epidermal cysts) are rare (15 cases reported). Therefore, histological and clinical diagnoses may be difficult. The clinical course and histopathological and immunohistochemical findings in 9 patients are described. Observations: Pilofollicular lesions were present at the onset (n=3), before (n=3), or during a relapse of mycosis fungoides (n=3). Comedones and epidermal cysts were most frequent (n=5). They disappeared with lymphoma therapy (n=4), therapy with isotretinoin (n=3), or spontaneously (n=1), or they persisted (n= 1). Clues to the histopathological diagnosis consisted of pilotropism of the infiltrate with minor alteration of the hair follicle walls. The infiltrate was monomorphous and composed of sezariform CD4 + lymphocytes. Pilotropic or peripilofollicular infiltrates, or the absence of infiltrate, were detected in consecutive biopsy specimens obtained from the same patient. The keratinocyte expression of intercellular adhesion molecule type 1 was observed in the hair follicle bulb in front of the pilotropic infiltrate but not in the epidermis. No staining was observed in biopsy specimens of 6 of 7 patients with follicular mucinosis, of folliculitis lesions, or of normal hair follicles. Conclusions: Our findings indicate the role of adhesion molecules in pilotropism leading to mechanical obstruction of the follicle by tumoral cells followed by hyperkeratosis and cyst formation. It remains to be determined if the expression of intercellular adhesion molecule type 1 is the cause or the consequence of pilotropism. By becoming more aware of it, this variant of mycosis fungoides is probably not so rare. (Arch Dermatol. 1996;132:683-687)

To the Editor.— Alagille's syndrome, due to interlobular biliary duct deficiency, associates various cardiovascular, vertebral, ocular anomalies and especially, a typical facies, making the diagnosis possible at first glance, according to Alagille and coworkers. 1 There are also cutaneous signs, however, that are rarely described in the literature. Therefore, it seemed worthwhile to report a case herein. Report of a Case.— When we saw this 22-year-old woman for the first time, we immediately noticed the facial aspect as described by Alagille and coworkers 1 : a rounded forehead, eyes deep in the sockets with subdued hypertelorism, a saddle nose, and a small chin (Fig 1). The patient complained of an unbearable pruritus with insomnia. Indeed, on the back side of her legs and ankles, there are discretely keratotic, multiple papules in streaks (Fig 2). Microscopic examination of the skin biopsy specimen disclosed deposits of an amorphous substance under a hyperkeratotic epidermis

• We retrospectively analyzed 63 observations collected in nine French departments of dermatology of an acute pustular dermatosis, recently named in the French literatureacute generalized exanthematous pustulosis(AGEP). Even though 11 of these cases occurred in patients with a history of psoriasis, AGEP appeared distinct from pustular psoriasis based on several slight pathologic differences, drug induction in most cases, and a more acute course of fever and pustulosis, with rapid spontaneous healing. We, therefore, suggest that AGEP is a reaction pattern, perhaps favored by a "psoriatic background." The most frequent causes of AGEP seem to be drug reactions, acute infections with enteroviruses, and hypersensitivity to mercury. With 55 (87%) of 63 cases attributed to drugs in this series, AGEP should be added to the list of cutaneous adverse drug reactions. Among druginduced skin eruptions, AGEP is remarkable by its short time to onset after the administration of the suspected drug ( (Arch Dermatol.1991;127:1333-1338)