Nodular lymphoid disease of the head and neck: lymphocytoma cutis, benign lymphocytic infiltrate of Jessner, and their distinction from malignant lymphoma

Skin biopsy specimens from six patients with nodular lymphoid disease of the head and neck were studied by routine histology, direct immunofluorescence microscopy, and leukocyte monoclonal antibodies to T and B cell subsets and monocytes. Initially, these lesions were clinically considered to be benign lymphocytic infiltrates of Jessner, lymphocytoma, or lymphoma. Direct immunofluorescence was negative or showed nonspecific staining in all four patients in whom it was performed. Leukocyte monoclonal antibody stains revealed two distinct patterns of lymphocytes. Lymphocytoma was represented by nodular masses of B lymphocytes with peripheral and intervening zones of T cells. The second pattern consisted of solid nodular masses of T lymphocytes occupying the dermis and subcutaneous tissue. In the specimens interpreted as benign lymphocytic infiltration, the T cells were composed equally of helper and suppressor cells.     

Double-blind crossover study comparing doxepin with diphenhydramine for the treatment of chronic urticaria

Fifty patients with chronic idiopathic urticaria were studied to compare responses to treatment with doxepin (10 mg three times a day) and with diphenhydramine (25 mg three times a day). All patients had an evaluation that failed to disclose a cause for their disease. Therapeutic response was assessed according to the suppression of symptoms and symptom diary scores of daily itching and frequency, number, size, and duration of hives. Total clearing of the pruritus and urticarial lesions occurred in 43% of the patients while receiving doxepin and in only 5% while receiving diphenhydramine (p less than 0.001). Partial or total control of the pruritus and hives was noted in 74% of the patients receiving doxepin and in only 10% of those receiving diphenhydramine (p less than 0.001). Doxepin induced markedly less sedation (22%) than diphenhydramine (46%) (p less than 0.05). Dermatopathologic categories included (1) urticaria simplex, (2) lymphocytic urticaria, and (3) leukocytoclastic urticaria. Patients with urticaria simplex had a more favorable response to doxepin than the two other groups.     

The therapeutic uses of topical vitamin A acid

Topical vitamin A acid (VAA) has various mechanisms of action which may be responsible for its therapeutic success in many different disorders. Although the absorption, metabolism, and excretion of VAA are not completely understood, VAA appears to remain mainly on the skin surface. The question of carcinogenicity is unresolved, and more research is needed to clarify this problem. This article reviews the literature regarding the therapeutic uses of VAA and summarizes various investigators' experiences with VAA.     

Malignant pyoderma

Malignant pyoderma is a destructive, ulcerating skin disease that occurs chiefly in young adults. Only eight cases of this rare disease have been reported. The head and neck have been involved in all cases, and a predilection for the periauricular region has been noted in several of the cases. Although the disease is progressive and chronic, responses to high-dose systemic corticosteroids have been noted, but usually a flare is associated with a reduction in dose. An additional case is described in which therapy with isotretinoin and sulfapyridine led to complete remission.     

Genital Paget's disease and urinary tract malignancy

Eight cases of Paget's disease of genital mucosa with malignancy of the lower urinary tract are described. In five it was apparent that there was concurrence of two separate malignancies. In one patient with long-standing Paget's disease of the genital mucosa, carcinoma of the urethra and bladder developed subsequently in continuity with the genital lesion; the two lesions were indistinguishable histologically, suggesting extension of the Paget's disease into urothelium. In two patients with bladder malignancy, there was histologic evidence of outward pagetoid extension of this process along urothelium and onto the genital mucosa. The significance of genital Paget's disease is discussed in the light of these findings, and the possible origins of Paget cells within the epidermis are reviewed.     

Factitial traumatic panniculitis

We report five cases of factitial panniculitis produced by blunt trauma. Localized forms include Secrétan's syndrome and l'oedeme bleu, which are usually unilateral and involve the hand and forearm, respectively. When the lesions are more generally distributed, the self-induced nature may be less apparent. These patients may have been diagnosed as having Weber-Christian disease, pancreatic fat necrosis, or superficial thrombophlebitis. The microscopic picture of organizing hematoma may be supplemented by the appearance of amorphous polysaccharide masses commingled with iron pigment.     

Eosinophilic histiocytosis: A variant form of lymphomatoid papulosis or a disease sui generis?

Five patients are reported on whose clinical skin disease consisted of polymorphous papulonodular lesions healing with a depigmented scar. Although all cases had been termed lymphomatoid papulosis after clinical or histologic examination, the lesions consisted principally of masses of histiocytes and eosinophils. Individual lesions healed spontaneously or with minimal treatment, but the chronic course of disease was not altered by any therapy used. Follow-up 3 to 17 years later indicated persistent or recurrent disease, and one patient died of histiocytic malignancy. Eosinophilic histiocytosis is the microscopic picture of an unusual group of patients with chronic papulonodular necrotic skin disease that may deserve to be considered a disease pattern per se.     

Evaluation of isotretinoin treatment of hidradenitis suppurativa

Eight patients with a long-standing hidradenitis suppurativa were treated with isotretinoin, 0.71 to 1.2 mg/kg/day, as a single agent for 4 months and have had follow-up of at least 2 months. The clinical status was judged as cleared in one patient, almost cleared in three patients, improved in one patient, not changed in two patients, and worse in one patient.     

Pre-Sézary syndrome

Eighteen patients with erythroderma, recurrent cycles of circulating Sézary cells of less than 1,000 cells/mm3, and a chronic course were followed for a mean time of nearly 5 years and were diagnosed as having pre-Sézary syndrome. Only one patient died, and none developed lymphoproliferative disease. All ten patients who underwent patch testing showed positive results. The elevation of IgE was striking when this group was compared with a group with Sézary syndrome. Most patients achieved partial or complete remission on low-dose chlorambucil and prednisone therapy. Some patients had lymphocytic or lymphomatoid bands on skin biopsy specimens and were like previously reported patients with pre-Sézary syndrome whose condition progressed to Sézary syndrome. A nontoxic chemotherapy or an anti-T cell treatment program can control this chronic erythroderma state.     

Psoralens and ultraviolet A therapy of pityriasis lichenoides

Three patients with long-standing pityriasis lichenoides, which was resistant to other forms of therapy, were successfully treated with PUVA (psoralens and ultraviolet light of wavelength A). One patient had complete clearing of all lesions, and the other two had marked improvement. PUVA is being used to treat increasing numbers of patients with pityriasis lichenoides, and the results have been very good.     

Scleredema: A review of thirty-three cases

A review of thirty-three cases of scleredema, with particular reference to clinical and histologic findings, revealed that the disease was often of insidious onset and the course was usually prolonged. A preceding respiratory tract infection was uncommon and usually did not indicate a short course. The patients were divided into two groups according to the presence or absence of diabetes. Diabetes, when present, was typically the late-onset, insulin-dependent type and difficult to control. In many of these patients, the onset of scleredema was so subtle that it went unnoticed by the patient until pointed out by an examining physician.     

Olmsted syndrome--congenital palmoplantar and periorificial keratoderma

We are reporting the third case of the Olmsted syndrome, an entity that has received little attention in the medical literature. The syndrome consists of congenital diffuse, sharply marginated keratoderma of the palms and soles, onychodystrophy , constrictions of digits, and periorificial keratoses. Our patient, in addition, had congenital universal alopecia, absence of a premolar tooth, leukokeratosis of oral tissue, and hyperlaxity of the joints. The differential diagnosis includes hidrotic ectodermal dysplasia of the Clouston type, pachyonychia congenita, acrodermatitis enteropathica, keratoma hereditarium mutilans of Vohwinkel , mal de Meleda , and other forms of palmoplantar keratodermas . This patient had originally been reported as having acrodermatitis enteropathica, a condition with which the Olmsted syndrome can easily be confused.     

Inflammatory macular atrophy: immunofluorescent and ultrastructural findings.

In a patient with primary inflammatory macular atrophy, immunofluorescence studies disclosed both C'3 and IgM in a granular and fibrillar pattern in the dermis between the coagen fibers and at the basement membrane zone. Localization of C'3 to autofluorescent elastic fibers was demonstrated. Ultrastructural examination showed the presence of activated macrophages enveloping fragmented elastic fibers. Immunologic mechanisms may play a participatory role in some forms of macular atrophy.