Thomas N. Helm

Paraneoplastic pemphigus: A report of three cases including one long-term survivor

BACKGROUND Paraneoplastic pemphigus is a newly described autoimmune disease characterized by painful mucosal ulcerations and polymorphous skin lesions in association with an underlying neoplasm. All reported patients with an associated malignant neoplasm have had a poor prognosis. OBJECTIVE We present three new cases of paraneoplastic pemphigus associated with a malignant neoplasm and further characterize this disease. METHODS We used clinical criteria, histologic and immunopathologic examinations, and immunophenotyping to characterize this disease. In addition, we performed immunoprecipitation studies with extracts of radiolabeled human keratinocytes to characterize the antigens to which patient serum binds. RESULTS All three patients had clinical, histologic, and immunopathologic findings that were strongly suggestive of paraneoplastic pemphigus. Their sera immunoprecipitated a complex of four polypeptides from human keratinocyte extracts with molecular weights of 250, 230, 210, and 190 kd, confirming the diagnosis of paraneoplastic pemphigus. The 250, 230, and 210 kd antigens comigrated with desmoplakin I, the 230 kd bullous pemphigoid antigen, and desmoplakin II, respectively. Lymphocyte immunophenotyping revealed large populations of monoclonal CD19+, CD5+ B cells in two cases. Although two of the patients died, one patient is alive and well 2 years after the diagnosis of paraneoplastic pemphigus. CONCLUSION We report three cases of paraneoplastic pemphigus. One patient is alive and well 2 years after diagnosis, which suggests that a subgroup of patients may have a more benign course.

Indirect immunofluorescence on rat bladder transitional epithelium: A test with high specificity for paraneoplastic pemphigus

BACKGROUND Paraneoplastic pemphigus is a blistering disease with specific serum immunoprecipitation findings. Although immunoprecipitation studies allow accurate diagnosis, they are time-consuming, expensive, and not readily available. In contrast, indirect immunofluorescence (IIF) testing of serum on transitional rat bladder epithelium is a simple and inexpensive method available to any immunopathology laboratory. OBJECTIVE Our purpose was to determine the specificity of positive IIF on rat bladder epithelium for paraneoplastic pemphigus. METHODS The IIF findings in four index cases of paraneoplastic pemphigus were compared with the findings in 47 patients with a variety of malignant neoplasms and no associated blistering disease as well as 49 patients with vesiculobullous or lichenoid disease but no neoplasia. RESULTS IIF was negative in all patients with neoplasia and no blistering disease and negative in all but one of the patients with vesiculobullous or lichenoid disease without neoplasia (98.9% specificity). CONCLUSION IIF on transitional rat bladder epithelium appears to be a highly specific test for paraneoplastic pemphigus. Because of its simplicity and inexpensiveness, we suggest that IIF be performed on transitional epithelium in any suspected case of paraneoplastic pemphigus.

Malignant melanoma arising in a blue nevus with features of pilar neurocristic hamartoma

A 65‐year‐old man presented with a history of a giant blue plaque of the parietal scalp since childhood. Biopsy revealed a cellular blue nevus. The blue nevus was observed for 10 years and thought to be unchanged until a new adjacent lesion was noted. Biopsy of the new lesion revealed metastatic malignant melanoma. A wide excision was performed of the original lesion which revealed malignant melanoma arising in a blue nevus. Areas within the blue nevus were consistent with a pilar neurocristic hamartoma, whereas other areas were consistent with a common blue nevus. Subsequent satellite metastases developed, with early metastases resembling blue nevi except for the absence of a stromal component and the presence of hyperchromatic nuclei. Later metastases were typical of metastatic melanoma. This case illustrates the uncommon evolution of malignant melanoma from a blue nevus. The histological features and relationship between melanoma, blue nevus, and pilar neurocristic hamartoma are reviewed.

Contact urticaria and anaphylaxis to latex

Contact urticaria and anaphylaxis to latex is reported in two patients. One case was associated with oral and vaginal exposure to a condom and the other with rectal exposure to a latex glove. Both patients had positive reactions to scratch or prick tests to pieces of latex, as well as latex radioallergosorbent test (RAST). The diagnosis of contact urticaria to latex is difficult to make on the basis of history alone.

Case report and review of resolved fusariosis

Erythematous macules, nonpalpable and palpable purpura, and flaccid pustules developed in a 59-year-old man with acute lymphocytic leukemia 8 days after reinduction chemotherapy with cytosine arabinoside and daunorubicin. Tissue and blood cultures grew Fusarium proliferatum, and a skin biopsy specimen revealed fungal vasculitis. Anemia and muscle weakness accompanied the disseminated infection, for which the patient received granulocyte transfusions and amphotericin B, ketoconazole, rifampin, and griseofulvin. Skin lesions and fungemia resolved with recovery of the bone marrow, and 51 days after the completion of his chemotherapy he returned home. If promptly recognized and aggressively treated, disseminated fusariosis is responsive to therapy. Infection with Fusarium species should be suspected in profoundly neutropenic patients in whom disseminated palpable purpura and myositis develop concomitantly.

Lichen planus associated with neoplasia: A cell-mediated immune response to tumor antigens?

BACKGROUND Individual case reports have suggested an occasional association of lichen planus with internal malignancy. OBJECTIVE Our purpose was to describe five patients with a neoplastic disease in whom lichen planus developed. METHODS Serologic and immunopathologic studies were conducted. RESULTS No evidence of autoantibody production characteristic of paraneoplastic pemphigus was found, and antibodies reactive with basal cell keratinocytes were not detected. CONCLUSION Lichen planus may be rarely induced by neoplasia. A cell-mediated immune reaction possibly causes this phenomenon.

Ki-1-positive anaplastic large-cell lymphoma can mimic benign dermatoses

BACKGROUND Regressing atypical histiocytosis is a recently described disease characterized by recurrent nodules or ulcers. The cutaneous lesions appear abruptly and then regress only to return in a manner reminiscent of lymphomatoid papulosis. Immunophenotypic analysis has revealed that most cases are a form of anaplastic large-cell Ki-1-positive (CD30+) lymphoma. OBJECTIVE We describe two patients with Ki-1-positive anaplastic large-cell lymphoma that had clinical and pathologic features of regressing atypical histiocytosis and mimicked benign dermatoses (pyoderma gangrenosum and morphea), causing a delay in confirming the true diagnosis. A third case that was readily recognized as a lymphoma is also presented. METHODS The clinical and histopathologic findings were recorded. In addition, T-cell receptor gene rearrangement and immunophenotyping were determined in the index case. RESULTS The index patient and second patient were diagnosed as having Ki-1-positive anaplastic large-cell lymphoma by immunophenotyping and underwent cyclophosphamide, doxorubicin, prednisone, and vincristine (CHOP) chemotherapy with complete remission. The patient detected by chart review died of her disease without receiving antineoplastic therapy; disseminated lymphoma was diagnosed at autopsy. Studies on paraffin-embedded tissue were consistent with Ki-1-positive anaplastic large-cell lymphoma. CONCLUSION Regressing atypical histiocytosis may clinically resemble some benign dermatoses. Recent evaluation of these cases has shown that many represent a form of Ki-1-positive anaplastic large-cell lymphoma. Multiple skin biopsy specimens with immunophenotyping and gene rearrangement studies are required to arrive at the diagnosis.

Persistent Annular Erythema of Infancy

Abstract: Annular erythema of Infancy is an uncommon, nonpruritic, figurate erythema that begins in the first year of life. Biopsy specimens reveal a perivascular and interstitial lymphocytic infiltrate with numerous eoslnophils. The cause of the disorder is unknown, but a hypersensitivity response to unrecognized antigens is suspected. The disorder is selflimited, but may last for many months. In our patient the eruption continued 19 months after its onset.

Relapsing polychondritis: A case diagnosed by direct immunofluorescence and coexisting with pseudocyst of the auricle

A case of relapsing polychondritis with perichondrial IgG and C3, dermoepidermal IgG, and a coexisting pseudocyst of the auricle is reported. The use of direct immunofluorescence testing in establishing a diagnosis of relapsing polychondritis and the expected findings are reviewed. In some cases direct immunofluorescence may allow a diagnosis of relapsing polychondritis before clinical criteria or a routine histologic evaluation suggests a definite diagnosis.

Seborrheic keratoses with occult underlying basal cell carcinoma

vusat the site. The lesionwas treated with a singlepulse (7.0 J jcm2, 450 f.Lsec pulse duration) with a pulsed dye laser (Candela SPTL-l, Wayland, Mass.) and a 5 mm diameter treatment. The treatment area immediately became purpuric and then encrusted after 4 days. After 3 weeks the treatment area had totally reepithelialized, but a remnant of the original papule remained slightly palpable. The lesion was treated again as before. After an additional 3 weeks the lesion was nonpalpable. Fig. 1, B shows the clinical result 5 months after treatment.