Gary R. Kantor

Generalized pruritus and systemic disease

A retrospective study of 44 cases of generalized pruritus (GP) was undertaken to determine the incidence of systemic disease in this population. This data was compared to that of a group of 44 age- and sex-matched psoriatic controls. We found 13 (30%) of the GP cases to have a systemic disease that in the literature is believed to be associated with GP, versus 10 (23%) in the psoriasis group. Excluding hypothyroidism and diabetes mellitus, two disorders with questionable relationship to GP, the number of pruritus patients with an associated systemic disease was reduced to 8 (18%) compared to only 1 (2%) psoriatic patient, a difference which is statistically significant (p less than 0.02). In 6 (14%) of the GP patients a temporal relationship of systemic disease and GP was suggested. Recommendations for outpatient workup of GP are given. Patient follow-up of 1 to 6 1/2 years revealed that 29 (66%) of the patients had persistent itching. The mean duration of itching to the time of the present follow-up was 57.7 months.

Scleromyxedema associated with scleroderma renal disease and acute psychosis

Scleromyxedema is a rare cutaneous disease with occasional systemic involvement and characteristic poor response to treatment. A case of scleromyxedema associated with renal scleroderma and acute psychosis is presented, along with a review of treatment and associated internal disorders.

Dilated pore nevus ; A histologic variant of nevus comedonicus

Histology of a nevus comedonicus showed aggregates of dilated follicular cysts reminiscent of dilated pore of Winer. This finding has not been previously reported. We propose the term dilated pore nevus for this lesion, which we conceive to be a histologic variant of nevus comedonicus, similar to porokeratotic eccrine ostial and dermal duct nevus.

Dermatophyte-related granular parakeratosis.

A patient presented with a two-month history of an annular eruption on her back. Biopsies of this process revealed dermatophytosis and concurrent granular parakeratosis. This patients eruption resolved completely with antifungal therapy suggesting that the fungal organisms caused the granular parakeratosis.

Common and uncommon dermatologic diseases related to sports activities.

The athlete is exposed to a whole spectrum of skin diseases that may or may not be unique to the particular sport or activity. Although the athlete is usually a healthy young adult and sports-related skin diseases are often minor, it is important for these disorders to be recognized by physicians, nurses, coaches, and trainers. Prevention, early diagnosis, and treatment will enable the athlete to resume participation as early as medically possible.

Epidermotropic metastatic prostate carcinoma presenting as an umbilical nodule-Sister Mary Joseph nodule.

Carcinoma of the prostate accounts for fewer than 1% of all skin metastases.1 Cutaneous metastases from prostate carcinoma most often involve the penis,2-7 the anterior aspect of the thighs, the suprapubic area,8 and the perineum,8,9 but they also have been reported in the scalp, the chest, the back, and even the face.10 We report an unusual case of metastatic prostate adenocarcinoma that presented as an umbilical nodule (Sister Mary Joseph nodule) and demonstrated significant epidermotropism histologically. A review of the literature has found only one documented case of prostatic carcinoma metastasizing to the umbilicus,11 and one other documented case of epidermotropic metastatic prostate carcinoma.12

Tertiary syphilis of the face

We report a case of noduloulcerative tertiary syphilis that had gone misdiagnosed as discoid lupus erythematosus for many years. This case is noteworthy for (1) a Jarisch-Herxheimer-like reaction, (2) a dramatic response to penicillin therapy despite the presence of long-standing disease, and (3) posttreatment granulomas of possible hypersensitivity origin.

Eosinophils are not a clue to the pathogenesis of granuloma annulare.

Eosinophils are variably present in biopsy specimens of granuloma annulare (GA). A retrospective study of 77 biopsy specimens from 73 patients with GA was performed to quantitate eosinophils and correlate the findings with both histologic and clinical features. Specimens were divided into absent, low (10 > x > or = 1 eosinophils per 3 sections), and high (x > or = 10 eosinophils per 3 sections) eosinophil groups and evaluated for eight histologic and 11 clinical features. Eosinophils were identified in 66% (51/77) of biopsy specimens, with 51.9% (40/77) and 14.3% (11/77) in the low and high eosinophil groups, respectively. Clinically, patients in the high eosinophil group were found to be significantly younger with a propensity towards nonannular, localized disease. These patients also did not have diabetes or use systemic medications. Histologically, the presence of eosinophils was strongly associated with palisaded architectural pattern and the presence of necrobiosis. Eosinophils were not present in areas of necrobiosis but were found predominantly perivascularly. A moderate to marked lymphocytic infiltrate at the periphery of the granulomas and reactive vessel changes were common in all three groups, but vasculitis was not identified. In summary, this study showed eosinophils to be commonly present in GA but was unable to correlate their presence with specific clinical or histologic features supportive of an allergic precipitant. A cell-mediated immune mechanism is likely involved in GA, with eosinophils recruited nonspecifically by mediators released during granuloma formation.

Localized cutaneous amyloidosis associated with mycosis fungoides

REFERENCES 1. yon den Driesch P. Sweets syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994;31:535-56. 2. Jorizzo JL. Blood vessel-based inflammatory disorders. In: Moschella SL, Hurley HJ, editors. Dermatology. 2nd ed. Philadelphia: WB Saunders; 1992. p. 577-93. 3. Amichai B, Lazarov A, Halevy S. Sweets syndrome. J Am Acad Dermatol 1995;33:144-5. 4. Graham DY. Campylobacterpylori and peptic ulcer disease. Gastroenterology 1989;96:615-25. 5. Kelly SM, Geraghty JM, Neale G. H. pylori, gastric carcinoma, and MALT lymphoma. Lancet 1994; 1:343-418. 6. Reinauer S, Megahed M, Goerz G, et aL Sch6nleinHenoch purpura associated with gastric Helicobacter pylori infection. J Am Acad Dermatol 1995;33:876-9.

Sézary Syndrome Coexisting with B-Cell Chronic Lymphocytic Leukemia: Case Report and Review of the Literature

Introduction: The simultaneous presentation of chronic B-cell lymphocytic leukemia (B-CLL) and cutaneous T-cell lymphoma (CTCL) is extremely rare. Case Report: We describe a patient with B-CLL and Sézary syndrome (SS), an erythrodermic and leukemic variant of CTCL. Despite treatment, the SS progressed to involve internal organs and eventual death of the patient from sepsis. This is the first reported case of SS coexisting with chronic lymphocytic leukemia in which an anti-Vβ13.6 antibody was used to serially track changes in circulating neoplastic T cells vis-à-vis neoplastic B cells and to detect neoplastic T cells in ascitic fluid near the end of the patient’s life. Discussion: We speculate that the coexistence of B-CLL and CTCL is the result of an initiating genetic or epigenetic defect at the level of the common lymphoid stem cell that predisposes both B-cell and T-cell lineages to additional oncogenic changes at a more advanced stage of differentiation.