William A. Caro

Photochemotherapy in cutaneous T cell lymphoma and parapsoriasis en plaques: Long-term follow-up in forty-three patients

Forty-three patients with clinical plaque- and tumor-stage mycosis fungoides, the erythrodermic/Sézary syndrome variant of mycosis fungoides, and parapsoriasis en plaques were treated with oral psoralens and ultraviolet A (PUVA). Pretreatment skin biopsies, evaluated by light microscopy, revealed seventeen diagnostic, seventeen suggestive, and nine nonspecific specimens. Clinical and histologic parameters were followed for an average of 38.4 months (range, 4-67 months). Twenty-five patients had complete clearing, and fourteen did not respond. Most patients in the complete-response group had either plaque lesions of mycosis fungoides or parapsoriasis en plaques prior to PUVA. Most patients in the no-response group had either tumor lesions or the erythrodermic/Sézary mycosis fungoides at the start of PUVA. In the no-response group the treatment modalities used prior to PUVA were twice the number used in the complete-response group. Patients in the complete-response group had clearing of their lesions after an average PUVA dose of 117 joules/cm2. Relapse occurred in seventeen patients after an average remission time of 6.3 months and responded to additional PUVA. Patients whose skin remained clear after the first course of PUVA continued to have clear skin for up to 58 months, with an average complete remission of 29.5 months by the end of the study period. Histologic evaluation before PUVA and at clearing revealed a definite trend toward a normal microscopic picture, but at least a mild inflammatory infiltrate usually persisted. At the end of the study period, the lesions of ten patients had entirely cleared for an average of 44 months, the lesions of five had cleared during a second course of PUVA, five had stable limited-plaque disease while receiving maintenance PUVA, eleven were undergoing electron beam radiation therapy or chemotherapy for progressive disease, ten had died, and two patients were lost to follow-up. Therefore, in the early stage of mycosis fungoides, PUVA may induce significant disease-free intervals. Prior treatment with a variety of modalities, the patients age, and/or the duration of disease may affect response to PUVA.

Skin cancer in the PUVA-48 cooperative study

A 4-year follow-up study of 631 patients with psoriasis given 8-methoxypsoralen and UVA in the PUVA-48 cooperative study revealed only ten patients with skin cancer and eight patients with actinic keratosis. The skin cancer group averaged 14 years older than the group without lesions. The cumulative treatment ranged from 255 to 1,737 joules/cm2 at the time of development of skin cancer. The ratio of basal cell carcinoma to squamous cell carcinoma and the anatomic location of lesions generally follow the pattern of spontaneously developing lesions. Ionizing radiation was a significant risk factor, but previous arsenic exposure or skin cancer was not. Comparison with previous studies and lack of control population may indicate less risk of skin cancer following PUVA than previously suggested.

Malignant lymphoma presenting with cutaneous granulomas

A 49-year-old man with fever, malaise, weight loss, and pneumonia developed cutaneous nodules and neurologic symptoms. Skin biopsy studies revealed granulomatous inflammation consistent with a sarcoid reaction, and mild granulomatous changes were noted on biopsy specimens of liver and bone marrow. A lymph node biopsy was unremarkable. Neurologic deterioration prompted an extensive workup that revealed an intracranial mass. A brain biopsy study revealed malignant lymphoma, large cell type. Autopsy study confirmed the diagnosis and showed no evidence of granulomatous infiltrates. The cutaneous granulomas represent a nonspecific immune response possibly related to the underlying lymphoma. The relationship between sarcoidosis and sarcoid reactions and lymphoma is discussed.

Neonatal Acute Hemorrhagic Edema of Childhood: Case Report and Review of the English‐Language Literature

Acute hemorrhagic edema of childhood is an unusual form of leukocytoclastic vasculitis previously reported in children age 4 months to 3 years. The etiology remains unknown, however, many authors describe the process as an immune‐mediated vasculitis in response to a variety of antigenic stimuli. We report a case of congenital acute hemorrhagic edema of childhood associated with maternal gastroenteritis six weeks before delivery.

Parapsoriasis and mycosis fungoides: The Northwestern University experience, 1970 to 1985

One hundred sixty skin biopsy specimens from 89 patients with the clinical diagnosis of large plaque parapsoriasis and 240 specimens from 106 patients with mycosis fungoides were reviewed. Through the use of chart reviews and a retrospective questionnaire, various factors (sex, age, history of eczema/atopy, occupation) were examined in these two patient groups. Mycosis fungoides developed in 30% of the patients in the parapsoriasis group. Nineteen percent of patients in the mycosis fungoides group had worked in industry. Once the clinical diagnosis of mycosis fungoides was considered, an average of four biopsy specimens were needed to establish the diagnosis. The average interval from the initial visit to the diagnosis of mycosis fungoides from examination of biopsy specimens was 22 months. These findings support further the view that large plaque parapsoriasis represents an important precursor of mycosis fungoides. A designation of premycosis fungoides would emphasize this relation more than the term parapsoriasis.

Report of an infant with GM1 gangliosidosis type I and extensive and unusual mongolian spots.

An infant with GM1 gangliosidosis was found to have an eruption at birth consisting of extensive and unusual slate blue macules resembling mongolian spots. All areas of skin were involved except face, scalp, palms, and soles. A biopsy of a macule obtained at 5 months of age demonstrated melanocytic cells in the dermis consistent with mongolian spot but also a perivascular histiocytic infiltrate. At 8 months of age, absence of β‐galactosidase activity was documented in both peripheral leukocytes and skin fibroblasts confirming the diagnosis of GM1 gangliosidosis. The dermal histiocytic cells noted on skin biopsy were interpreted as a manifestation of this storage disease. The coexistence of the hyperpigmented lesions and the heritable enzyme defect was believed to be coincidental.

Lichenoid changes in mycosis fungoides

BACKGROUND The lichenoid tissue reaction (LTR) is present in a variety of dermatoses. Numerous histologic variants of mycosis fungoides (MF) have also been described. OBJECTIVE Our purpose was to analyze a subset of patients with mycosis fungoides (MF) who had a prominent LTR at the time of presentation. METHODS Clinical and pathologic features were evaluated in 12 patients with MF and an LTR. RESULTS The primary complaint was intense pruritus (in 10 of 12 patients). An accelerated course was noted in one half of the patients including four with Sézary syndrome. Four patients died: two with Sézary syndrome and two with extensive patch/plaque MF. Lymphocyte atypia, prominent basal cell layer epidermotropism, and the presence of plasma cells and eosinophils may help to distinguish lichenoid MF from lichen planus. CONCLUSION A subset of patients with MF present with lichenoid changes. The pathologic features may have a striking resemblance to lichen planus. Our findings suggest that lichenoid changes in MF tend to be associated with intense pruritus and may connote a poor prognosis.

Histologic, immunofluorescent, and antinuclear antibody findings in PUVA-treated patients

Twenty-two patients with psoriasis were treated with PUVA therapy for over two years. Skin biopsies from sun-exposed skin were evaluated before, during, and after PUVA therapy. No statistically significant epidermal changes were found when looking for hyperkeratosis, atrophy, melanin, or melanocyte numbers. No dyskeratosis was found. Dermal changes, including alterations in elastic tissue and collagen, were not statistically significant. Amyloid deposits were absent. The amount of mucin deposition was found to correlate with the length of PUVA therapy. In four patients, the mucin disappeared after withdrawal of PUVA, suggesting that this change is reversible.

Cutaneous squamous cell carcinoma and hypercalcemia

An elderly man was admitted to the hospital with a large squamous cell carcinoma involving the right side of the chest wall. The patient was severely confused, and evaluation revealed pronounced hypercalcemia. With total surgical removal of the tumor, the serum calcium level fell to normal and his confusion cleared. Although hypercalcemia has been reported in association with a number of malignant tumors, including metastatic squamous cell carcinoma, this finding has been encountered only rarely with squamous cell carcinoma localized to the skin. The secretion of transforming growth factor or of another humoral substance has been postulated as the possible cause of hypercalcemia in this patient.