Biomedical subjects

J S Fortson

Publications and source records attributed to J S Fortson.

3 recordsLinked to original sources

PubMed1990

Cutaneous T-cell lymphoma (parapsoriasis en plaque). An association with pityriasis lichenoides et varioliformis acuta in young children.

Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) are related benign disorders without recognized association with cutaneous T-cell lymphoma (CTCL). We report the cases of two children with documented PLEVA evolving into CTCL over several years. One child had the clinical lesions of PLC but the dermatopathologic findings of PLEVA at age 2 years. At age 12 years, he had skin changes of poikiloderma atrophicans vasculare and dermatopathologic findings consistent with parapsoriasis en plaque. The second child presented at age 7 years with scaling dermatitis and dermatopathologic findings of PLEVA. At age 12 years, the histologic diagnosis was parapsoriasis. Monoclonal antibody studies performed on biopsy specimens from both patients revealed 70% to 100% cells staining with CD5, 80% to 90% staining with CD4, 30% to 50% staining with CD8, and an increase in CD1-staining cells in the papillary dermis, indicating a predominantly helper T-cell infiltrate. We believe that PLC and PLEVA may be part of the spectrum of CTCL. Furthermore, CTCL may be more common in young children than once thought.

Child↗

PubMed1990

Necrobiotic xanthogranuloma with IgA paraproteinemia and extracutaneous involvement.

Numerous nodular xanthomatous lesions arose, gradually increasing in number on the back, chest, lips, tongue, and exposed "V" area of the chest on a 78-year-old man. Painful oral nodules and hoarseness developed subsequently. Histologic examination of the skin revealed a suppurative and granulomatous inflammation with an accumulation of acute polymorphonuclear neutrophils, nuclear dust, and xanthomatous histiocytes. Giant cells were rare and cholesterol clefts were absent. Biopsy of the oral mucosa and the larynx showed a similar process. Direct immunofluorescence was positive for vascular immune reactants at the basement membrane and fibrin in between collagen bundles. The patient had a serum IgA level of 432 mg/dl (normal level, up to 380), with normal IgG and IgM serum concentrations. A Raji cell assay was 240 micrograms/AHGEQ/ml (normal less than 5). CH-50 was decreased and C2, C4, and C3 were within normal limits. The dermatopathology was initially thought to be consistent with erythema elevatum diutinum, and the patient was treated with dapsone and prednisone, with a marginal response. There were no dermatopathologic findings of vasculitis. On review, the patient's dermatopathology was more consistent with necrobiotic xanthogranuloma (NXG). To our knowledge, this is the first report of an association of NXG with an IgA gammopathy and the second reported case of extracutaneous involvement with NXG.

Aged↗

PubMed1986

Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone.

A 45-year-old woman with cutaneous urticaria-like lesions subsequently developed polyarthritis, glomerulonephritis, and chronic obstructive pulmonary disease. Biopsy of skin showed leukocytoclastic vasculitis of superficial capillaries. Biopsy of the kidney revealed mesangioproliferative glomerulonephritis, and lung biopsy revealed severe emphysema and thick-walled blood vessels with immunoglobulin deposition. Therapy with dapsone produced dramatic improvement of the patient's cutaneous vasculitis and arthritis. This case uniquely demonstrates the efficacy of dapsone for both the urticarial vasculitic and the arthritic components of this syndrome and reemphasizes the potentially severe pulmonary and renal complications.

Arthritis↗