Margot S. Peters

Drug-induced linear IgA bullous dermatosis: report of six cases and review of the literature.

BACKGROUND Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease that may be associated with drug exposure. OBJECTIVE Our purpose was to compare the clinical, pathologic, and immunofluorescence findings in drug-induced LABD with those in the idiopathic type. METHODS Six patients with an acute drug eruption were identified who had linear IgA deposition at the basement membrane zone (BMZ). Lesional tissue was examined by brightfield microscopy, and perilesional tissue was examined by direct immunofluorescence (DIF). The presence of circulating BMZ antibody was assayed by indirect immunofluorescence (IIF) on monkey esophagus (ME) and salt-split human skin (SS). RESULTS Histopathologic examination showed subepidermal bullae with varying numbers of inflammatory cells. DIF showed linear IgA at the BMZ; three of the patients also had weak deposition of C3 at the BMZ. Serum from five patients was studied by IIF. One patient had circulating IgA BMZ antibodies in a titer of 1:80 on ME, localized to the dermal side on SS. All patients were free of lesions within 5 weeks after discontinuation of the drug. CONCLUSION Drug-induced LABD is a self-limited eruption characterized by linear deposition of IgA without IgG at the BMZ. Most patients lack circulating antibodies. The distribution of lesions and the course of the disease differ from those of idiopathic LABD.

Pacemaker contact sensitivity

In a patient who had 4 cardiac pacemakers implanted and removed, pruritus, redness, and swelling of the skin overlying the pacemaker developed at intervals of 6 weeks to 17 months after insertion. Patch testing showed a 2 + reaction to titanium. The positive result of this test, the titanium case of the generator, and the history of multiple local reactions around the generator site pointed toward contact sensitivity to the pacemaker. Although a review of the literature indicates that this problem is rare, it is of extreme importance to the patient with pacemaker contact dermatitis.

Follicular mucinosis: Clinical and histopathologic study

Fifty-nine patients with the histologic diagnosis of follicular mucinosis (alopecia mucinosa) were evaluated retrospectively. Thirty-seven were male and 22 were female; ages ranged from 10 to 76 years. Of 19 patients with mycosis fungoides, 16 had initial lesions of follicular mucinosis on the trunk or extremities. Two patients had Hodgkins disease and follicular mucinosis; both were younger than 20 years of age. Seven other patients were 20 years of age or younger. Two of the nine adolescent patients had persistent plaques of follicular mucinosis up to 18 years after diagnosis. Evaluation of biopsy specimens for lymphocytes, eosinophils, nonlymphoid cell infiltration, epidermal lymphocytic exocytosis, mucin deposition, and epidermal hyperplasia revealed no predominant feature that differentiated the group with benign disease from the group with mycosis fungoides. We conclude that no single clinical or histopathologic observation predicts which patients with follicular mucinosis will have a benign course and that evaluation of multiple clinical and histologic variables is necessary.

T-cell receptor gene rearrangement analysis: cutaneous T cell lymphoma, peripheral T cell lymphoma, and premalignant and benign cutaneous lymphoproliferative disorders.

T-cell receptor gene rearrangement analysis is a useful technique to detect clonality and determine lineage of lymphoid neoplasms. We examined 103 patients with mycosis fungoides, Sézary syndrome, peripheral T cell lymphoma, potentially malignant lymphoproliferative disorders including pre-Sézary syndrome, large plaque parapsoriasis, lymphomatoid papulosis and follicular mucinosis, and various benign inflammatory infiltrates. A clonal rearrangement was detected in skin samples in 20 of 24 patients with mycosis fungoides and in peripheral blood samples in 19 of 21 patients with Sézary syndrome. A clonal population was also detected in seven of eight cases classified as peripheral T cell lymphoma. The potentially malignant dermatoses tended to have clonal rearrangement, with the exception of large plaque parapsoriasis, and further follow-up is needed to correlate clonality with the disease course. These studies demonstrate the value of molecular genetics as an adjunct to morphology in the examination of patients with cutaneous lymphoproliferative disease.

Purpura simplex (inflammatory purpura without vasculitis): A clinicopathologic study of 174 cases

Purpura simplex manifests clinically as macular purpuric and petechial pigmented, golden, annular, or lichenoid lesions. These subtypes have been termed Schambergs purpura, lichen aureus, Majocchis purpura, and Gougerot-Blum purpura. Histologically, there is inflammation and hemorrhage without fibrinoid necrosis of vessels. One hundred seventy-four cases were retrospectively reviewed. In some patients, the eruption was related to medications. Treatment was usually of limited benefit. Fifty-eight of 87 patients (67%) who had follow-up data did eventually have clearing of lesions. Because of the clinical and histologic similarities among the subtypes, the inclusive term purpura simplex is favored.

Mycosis fungoides in children and adolescents

The clinical and histologic findings in five young patients with mycosis fungoides are reviewed. The skin specimen from a 16-year-old boy had an infiltrate predominantly Leu-1+, Leu-4+, Leu-3a + 3b+, OKT6+, Leu-2a-, Leu-8-, and Leu-9-, and rearrangements were noted in T cell receptor gene (beta chain [constant region probe] and gamma chain [J region probe]); no rearrangements were found in a histologically normal lymph node or peripheral blood. The skin specimen from an 11-year-old girl contained predominantly Leu-4+ and Leu-9+ lymphoid cells. No T cell receptor gene rearrangements were found in the skin or in an involved lymph node. The variations in clinical, histologic, and immunopathologic features suggest that mycosis fungoides in young patients represents a heterogeneous subgroup of cutaneous T cell lymphoma.

Lupus Erythematosus Panniculitis

LE panniculitis is an uncommon but distinctive subset of LE. It may develop in patients with discoid LE or SLE or may occur as an isolated phenomenon. The typical clinical presentation is that of multiple indurated nodules or plaques (or both), often associated with lipoatrophy, there being a predilection for the proximal extremities and trunk. Because the clinical and histologic findings of LE panniculitis overlap with those of other connective tissue diseases, evaluation of patients suspected of having LE panniculitis should include a complete history and physical examination as well as serologic studies, determination of peripheral blood counts, and tests of renal function. A deep excisional biopsy rather than punch biopsy should be performed for diagnosis. The characteristic histologic pattern includes hyaline necrosis of fat; lymphoid nodules, often with germinal centers; and lymphocytic lobular panniculitis. Direct immunofluorescence testing of skin may help confirm the diagnosis in patients who have less than classic histologic features. LE panniculitis tends to have a chronic course marked by recurrent nodules or plaques (or both). Antimalarial agents, with or without courses of systemic steroids, are beneficial in most patients.

Necrotizing fasciitis: A clinical, microbiologic, and histopathologic study of 14 patients

We studied the clinical, microbiologic, and histopathologic findings from 14 patients with necrotizing fasciitis; also tissue obtained at autopsy was available from six patients. In all cases, material cultured was positive for multiple organisms, including Clostridia and fungi; organisms were identified by histochemical staining of tissue sections in 12 of 14 cases. The histologic pattern comprised edema, necrosis, and inflammation of skin, subcutaneous fat, and fascial tissue. Hyalin necrosis of sweat glands was observed in five patients. Thrombosis of vessels at all levels was a prominent feature, suggesting that study of coagulation factors may be important. Diagnosis may be confirmed by the histologic picture; however, microbiologic material is essential to guide therapy.

Immunohistochemical stains in extramammary Paget's disease

The histologic and immunohistochemical characteristics of 49 skin biopsy specimens from 49 patients with extramammary Pagets disease were studied. Patients with extramammary Pagets disease with and without underlying malignant disease were identified. Associated malignant lesions, present in 16 patients (33%), were transitional cell carcinoma of the bladder (n = 8), adenocarcinoma underlying the skin (n = 3), adenocarcinoma of the anus (n = 1), adenocarcinoma of the vulva (n = 1), apocrine carcinoma (n = 1), prostate carcinoma (n = 1), and carcinoma metastatic to the lung (n = 1). The main histologic feature was the presence of Pagets cells, predominantly at the base of the epidermis. In 6% of the cases, well-defined nests of large Pagets cells mimicked melanocytic nests. Carcinoembryonic antigen and Cam 5.2 (a monoclonal antibody that stains 40-kDa, 45-kDa, and 52.5-kDa low molecular weight keratins) were localized to the Pagets cells in 42 of 45 (93%) and 29 of 41 cases (71%), respectively. Forty-four of 46 lesions (96%) were mucin positive, as determined by Hales colloidal iron stain. Absence of staining for colloidal iron and carcinoembryonic antigen occurred somewhat more frequently in patients with underlying malignant disease than in patients without tumors (13% vs. 0% mucin negative and 13% vs. 3% carcinoembryonic antigen negative, respectively). Although immunohistochemical staining for low molecular weight keratin may be used to confirm the diagnosis of extramammary Pagets disease, Cam 5.2 is not as sensitive as the colloidal iron or carcinoembryonic antigen stain.

Kimura's disease and angiolymphoid hyperplasia with eosinophilia: New observations from immunohistochemical studies of lymphocyte markers, endothelial antigens, and granulocyte proteins

Kimuras disease (KD) typically presents as large subcutaneous masses in young Oriental males. It is characterized by deep inflammation with vascular proliferation, lymphocytic nodules with subcutaneous germinal centers, fibrosis, and edema. In comparison, angiolymphoid hyperplasia with eosinophilia (ABLE) occurs in all races and the lesions usually are smaller and more superficial. The causes of diese two diseases are debated.