Clinical features associated with transformation of cerebriform T-cell lymphoma to a large cell process.

Some patients with cerebriform T-cell lymphoma (CTCL) undergo morphologic transformation to a large cell lymphoma. From a series of 113 patients with CTCL, 22 patients were identified with transformed CTCL. Stages of involvement at diagnosis were: I (seven), II (four), III (four), IV (seven). Nine patients had transformation at the initial diagnosis while the median time from diagnosis to transformation in the other 13 patients was 16 months (range: 3 months-6 years). Thirteen had transformation extracutaneously: lymph nodes (eight), central nervous system (two), and other extranodal sites (three). T cell markers were identified in all cases; of 15 cases with complete phenotypes, there were eight T-helper, three T-suppressor, and four aberrant T phenotypes. Serology for human T-leukemia virus-I (HTLV-I) was negative in eight patients tested. Median survival from diagnosis was 27 months compared to 53 months in 53 patients without transformation (p = 0.003). Despite combination chemotherapy in 12 patients following transformation, median survival after transformation was 12 months and only 7 months with extracutaneous disease. The likelihood of transformation could not be predicted by the initial histology, immunophenotype, or stage of disease.

Transformation of cutaneous T cell lymphoma to large cell lymphoma. A clinicopathologic and immunologic study.

Some patients with cutaneous T cell lymphoma (CTCL) develop a high-grade, large-cell lymphoma associated with rapid deterioration of clinical status. This change in histologic appearance and clinical behavior of CTCL is similar to transformations of other hematopoietic and lymphoid neoplasms. From a group of 92 cases of CTCL, morphologic, immunologic and clinical features were studied in 17 cases of transformed CTCL. Transformation was noted, at presentation or subsequently, in either cutaneous or extracutaneous sites; remarkably, transformation was found at initial diagnosis of CTCL in 7 of 17 patients. T cell characteristics were maintained in all 17 cases of transformed CTCL; in 11 cases with complete phenotypes, there were 6 T-helper, 3 T-suppressor, and 2 aberrant T subtypes. The pre- and posttransformation phenotypes were similar in 3 of 7 cases tested over time (all T-helper); retention of T-suppressor phenotype was suggested in another case. T cell features were maintained in the other 3 cases, but the T subtypes were altered in 2 of these cases. Absent or diminished pan-T antigens (CD 5, CD 3, or UCHL1) were found in 9 of 17 cases. Leu-M1, Ki-1, or LN 2 antigens were expressed by transformed cells in 10 of 17 cases, often in patterns identical to Reed-Sternberg cells. Survival in patients with transformed CTCL was significantly shorter (median, 29 months) than in 44 CTCL patients without transformation (58 months, P = 0.015); survival after diagnosis of transformation was short (12 months). Patients with extracutaneous transformation had a shorter median survival after transformation (8 months) than those with transformation limited to skin (19 months). It is concluded that CTCL can transform morphologically to a large cell variant associated with aggressive behavior and shortened survival. Extracutaneous transformation apparently indicates a poorer prognosis than cutaneous transformation. Although transformed CTCL usually retains a T cell phenotype, some antigens are lost while other new antigens may be expressed. Recognition of transformed CTCL is facilitated by identification of the dysplastic cerebriform cell component, but often requires correlation of immunologic and clinical features.

Linear granuloma annulare of the finger.

We document an unusual presentation of localized granuloma annulare: a flesh-colored, linear, painful lesion on the lateral aspect of the left index finger. The characteristic histopathologic findings of focal necrobiosis and palisading histiocytes were present.

Subcutaneous Whipple's disease.

Cutaneous findings in Whipple's disease are rare and often exhibit nonspecific lesions such as hyperpigmentation, subcutaneous nodules, and erythema nodosum. A 37-year-old black man with previously documented Whipple's disease developed soft, subcutaneous nodules on the chin, neck and extremities. Biopsy of these nodules showed a nonspecific panniculitis and intracellular inclusions stained by periodic acid-Schiff reagent. The inclusions were of the type thought characteristic of Whipple's disease. These findings indicate that patients with Whipple's disease can have characteristic skin findings that can be documented by skin biopsy.

Do brown recluse spider bites induce pyoderma gangrenosum?

Brown recluse spider bites are usually self-limited skin lesions that infrequently progress to bullae, ulceration, and scarring. We treated a patient with a documented brown recluse bite who had recurring lesions resembling pyoderma gangrenosum (PG) that persisted for months. Three other patients referred to Vanderbilt University because of probable brown recluse bite also had pyoderma for the first time after a suspected arthropod bite. The persistent and recurrent pyodermas in these four patients indicate that (1) brown recluse spider bites may not be self-limited but induce PG or PG-like lesions; (2) arthropod bites in general may induce PG in susceptible people; and (3) treatment of the PG-like lesions in these patients may be difficult.

Granuloma annulare in herpes zoster scars.

We have described a patient in whom GA developed at the site of remotely healed herpes zoster scars. This case represents another example of GA occurring in traumatized or injured skin and provides further evidence for the existence of the isomorphic response in the pathogenesis of this common disorder.

Cutaneous mastocytosis without clinically obvious skin lesions.

A patient with cutaneous mastocytosis had intractable pruritus but no visible skin lesions. Skin biopsies and urinary histamine and prostaglandin D2 metabolite assays confirmed the diagnosis. Adding therapy with psoralens and ultraviolet A to the antihistamine regimen markedly decreased the patient's pruritus. In patients with pruritus, flushing, syncope, or other symptoms associated with mastocytosis, this diagnosis should be considered even in the absence of specific skin lesions.

Treatment of prurigo nodularis: use of cryosurgery and intralesional steroids plus lidocaine.

Two patients with prurigo nodularis resistant to most modalities of therapy were treated successfully with the combined use of cryosurgery and intralesional steroids plus lidocaine. This appears to be a successful treatment regimen for a dermatosis that is often difficult to control.

Leiomyosarcoma of the skin and subcutaneous tissue.

In a study of 65 primary cutaneous leiomyosarcomas and 15 primary superficial subcutaneous leiomyosarcomas, tumors occurred at any age but were more common in middle age and most common on the extremities. They developed as solitary painful or tender intracutaneous or subcutaneous nodules. Microscopically, the cutaneous leiomyosarcomas consist of a poorly delineated proliferation of spindle-shaped atypical myomatous cells arranged in interlacing fascicles which merge into collagenous stroma. Subcutaneous leiomyosarcomas are more sharply circumscribed and typically include a vascular pattern. About 40% of the cutaneous leiomyosarcomas recurred, but none metastasized despite a high mitotic frequency and marked cytologic atypia. Among the 12 patients with subcutaneous leiomyosarcomas, one-half of the tumors recurred and one-third eventuated in metastasis or tumor-related death. Cutaneous leiomyosarcomas have a relatively benign biologic course and may be excised conservatively, but are less likely to recur if the local excision is wide enough to require a skin graft for closure of the surgical defect. For primary subcutaneous leiomyosarcoma, early wide local excision with adequate clear histologic borders constitutes rational treatment.

Lepromin-induced suppressor cells in patients with leprosy.

The possibility of an active mechanism of immunologic suppression in leprosy was explored by assessing the in vitro lymphocyte responses of 61 leprosy patients and 30 normal individuals to the mitogen Con A in the presence or absence of Dharmendra lepromin. Lepromin-induced suppression of Con A stimulation was found in 32 of 35 lepromatous patients and 15 of 15 borderline patients, but only 2 of 15 tuberculoid patients and 2 of 30 normal controls. Cell fractionation studies indicated at least two cell populations involved in the in vitro lepromin-induced suppressor activity, adherent cells and T gamma-cells.

Hereditary late-onset lymphedema.

Two different types of hereditary late-onset lymphedema are presented. In one family the father and one son had recurrent streptococcal lymphangitis beginning in childhood. In the son there was lymphatic hypoplasia in both legs with the infection having only occurred in one. Prophylaxis with penicillin prevented the recurrent lymphangitis. Because of 30 years of untreated lymphangitis, the father has chronic severe lymphedema. The second type, lymphedema associated with extra eyelashes (distichiasis) and a wide spinal canal, occurred in a woman whose lymphedema began at age 12 but in whom the hereditary nature of the disorder was not recognized until she was 29. Both of these types of late-onset lymphedema, lymphedema with lymphangitis and lymphedema with distichiasis, are due to autosomal dominant genes. Both families would have benefited from early diagnosis of the cause of the lymphedema.