Grover's disease: clinicopathologic review of 72 cases.

OBJECTIVE: To report the clinicopathologic findings in patients with Grover's disease. MATERIAL AND METHODS: We reviewed the medical records and biopsy specimens from 72 patients with transient acantholytic dermatosis (Grover's disease) examined at Mayo Clinic Rochester. Hematoxylin-eosin-stained biopsy specimens (from all patients) were assessed. Immunohistochemistry stains BRST-2, CAM 5.2, and CD44 were used to stain eight specimens. Direct immunofluorescence reports were reviewed. Selected specimens were stained by indirect immunofluorescence to detect major basic protein. RESULTS: Of the 72 patients, 63 (88%) were men, and the mean age was 48 years (range, 31 to 85). Lesions were distributed mainly on the trunk (in 71 patients) and proximal extremities (in 25). Heat and sweating frequently were exacerbating factors. Fifteen patients (21%) were bedbound. Concurrent nondermatologic malignant disease was present in 18 patients (25%). Two patients (3%) had acquired immunodeficiency syndrome. Follow-up in 28 patients (mean, 38 months; range, 3 months to 7 years) revealed that the disease had recurred in 13, persisted in 3, and resolved in 12. Review of the biopsy specimens showed that acantholysis was pemphigus vulgaris-like in 40 patients (56%), Darier's disease-like in 16 (22%), spongiotic in 12 (17%), pemphigus foliaceus-like in 2 (3%), and Hailey-Hailey disease-like in 2 (3%). A perivascular lymphocytic infiltrate of varied intensity in 64 specimens (89%) was associated with eosinophils in 16 (22%). In nine biopsy specimens with dermal eosinophilia stained for major basic protein, varied dermal cellular and extracellular deposition of major basic protein was present. Results of direct immunofluorescence studies, performed in 17 cases, were negative or nonspecific. CD44 stained acantholytic areas in addition to sweat glands in two of eight specimens (25%). CONCLUSION: Further studies of the pathogenesis of Grover's disease are needed. The predisposing conditions, site of involvement, and relapsing nature of this disorder may implicate acrosyringeal dysfunction as the cause.

Sebaceous epithelioma: a review of twenty-one cases.

BACKGROUND: Sebaceous epithelioma is a benign tumor that must be differentiated from basal cell carcinoma and other appendageal tumors. OBJECTIVE: Histologic and clinical findings were reviewed in 21 patients with sebaceous epitheliomas. METHODS: Twenty-one cases of sebaceous epithelioma collected during a 5-year period were reviewed. RESULTS: The tumors appeared as yellow to flesh-colored papules, most often on the face and neck. They consisted of basaloid cells with foci of sebaceous differentiation. Cystic spaces formed by holocrine degeneration or simulating sebaceous duct lining were common findings. Patients with sebaceous epitheliomas were more likely than the general population to have an internal malignancy. CONCLUSION: Patients with sebaceous epithelioma should be examined for an underlying malignancy.

Scleromyxedema.

BACKGROUND: Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and that is associated with significant morbidity and mortality. OBJECTIVE: Our purpose was to study the natural history of the disease and its response to therapy with alkylating agents. METHODS: A clinicopathologic review of 26 patients with scleromyxedema was performed, and the extracutaneous findings and response to therapy with alkylating agents were noted. RESULTS: Extracutaneous manifestations, most often gastrointestinal, were present in 20 of 26 patients. An abnormal paraprotein was found in 23 of 26 patients, most commonly IgG-lambda (18 patients). Melphalan was used as therapy for 17 patients. The disease proved fatal in 10 of the treated patients. CONCLUSION: The overall prognosis in scleromyxedema is poor. Therapy is difficult. Although alkylating agents may prove beneficial in the short term, significant toxicity of these agents is apparent with long-term use.

Aberrant proliferation of lumen-forming cells in stratified epithelium of porokeratosis skin.

The present investigation was based on the postulate, first proposed by Reed and Leone in 1970, that porokeratosis lesions represent clonal growth of epidermal cells. Comparative studies using immunocytochemical staining, epidermal cell culture, and non-equilibrium pH gradient (NEpHG) gel electrophoresis analysis of the keratins extracted were carried out on five patients with various types of porokeratosis. Positive type IV collagen staining on the stratum corneum was found in lesional skin specimens of all patients, but not in normal controls. A search for connections between type IV collagen in the basement membrane of epidermis and the skin surface disclosed infrequent intra-epidermal streaking of type IV collagen and one positively stained trans-epidermal acrosyringium. Positive intra-epidermal laminin staining in porokeratosis lesions confirmed the trans-epidermal passage of basement-membrane materials. Epidermal cells cultured from lesional skin showed low plating efficiency, and all colonies exhibited intracytoplasmic vacuole formation and excessive top cell shedding. NEpHG gel electrophoresis of keratins extracted showed that lesional profiles not only contained keratins normally present in glabrous skin, but also possibly K9 and some additional proteins. K9 had been immunolocalized to periductal cells in previous studies. Our findings, taken together, strongly suggest that porokeratosis epidermis consists of epithelial cells with lumen-forming ability.