Subcutaneous panniculitis-like T-cell lymphoma: an elusive case presenting as lipomembranous panniculitis and a review of 72 cases in the literature.

We present a remarkable case of subcutaneous panniculitic T-cell lymphoma (SPTL) that eluded diagnosis for 14 years and illustrates the importance of continued follow-up with repeat biopsy when SPTL is suspected. This case is unusual in that multiple biopsies demonstrated either a nonspecific panniculitis or lipomembranous panniculitis with calcified lipomembranes. A clinicopathologic review of 72 cases of SPTL from the English language literature is also presented, and approaches to diagnosis and treatment are reviewed.

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy): diagnostic skin biopsy changes determined by electron microscopy.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a familial vascular disorder associated with migraines, recurrent ischemic strokes, and early-onset multiinfarct dementia. The diagnosis of CADASIL is made ultrastructurally by finding characteristic granular, electron-dense, osmiophilic material attached to vascular smooth muscle cells. These changes have been found in brain, skeletal muscle, nerve, and skin. We describe a woman with CADASIL diagnosed on the basis of brain and skin electronmicroscopic findings. Dermatologists and dermatopathologists need to be aware of this disorder because characteristic electronmicroscopic changes on a skin biopsy specimen are diagnostic.

Intravascular lymphomatosis. A report of ten patients with central nervous system involvement and a review of the disease process.

The clinical, radiographic, and pathological findings in ten cases of intravascular lymphomatosis with central nervous system involvement seen at our institution over a 15-year period are presented. Nine patients presented with a subacute, progressive multifocal neurologic disorder. Most patients had fever, anemia, and elevation of the erythrocyte sedimentation rate. As the illness evolved, computerized tomography scanning and magnetic resonance imaging showed evidence of multifocal central nervous system disease. Angiography was nondiagnostic but suggested vasculitis in six cases. A response to empiric corticosteroid treatment was typical but usually transient. In six patients, the diagnosis was made antemortem by brain biopsy. The prognosis of patients was primarily dependent on early diagnosis and treatment, before massive central nervous system damage occurred. Treatment with chemotherapy, with or without radiotherapy, was associated with stabilization of the disease in three of five patients.

A study of the cutaneous manifestations of Behçet's disease in patients from the United States.

BACKGROUND: The type and frequency of different manifestations of Behçet's disease (BD) vary in different geographic areas. This variability could affect the ability to diagnose the disease in certain areas by using standardized criteria. The frequency of cutaneous lesions in patients from the United States, where the disease is less prevalent, is not known. OBJECTIVE: We sought to determine the frequency and type of skin lesions in a series of patients with BD from the United States and to identify methods of confirmation of these lesions as part of the disease process. RESULTS: Cutaneous manifestations were present in 64% of patients with BD. Clinicians most often relied on their clinical diagnosis to identify lesions as part of the spectrum of BD. Skin biopsy specimens were generally nonspecific. CONCLUSION: Cutaneous manifestations were common in patients with BD from the United States and usually were necessary to fulfill the diagnostic criteria of the disease in most cases.

Human herpesvirus 8 (HHV-8) in familial Kaposi's sarcoma.

Human herpesvirus 8 (HHV-8) has been detected in various epidemiological forms of Kaposi's sarcoma (KS). Since familial KS cases are exceedingly rare and the occurrence of familial KS in siblings has thought to depend rather on genetic factors than on a viral factor, familial KS has not been investigated for the presence of HHV-8. To investigate whether HHV-8 is present also in this rare form of KS, we examined tumor biopsies of 2 siblings with familial KS for the presence of HHV-8 specific DNA sequences by a nested PCR protocol. HHV-8 DNA sequences could be detected in KS specimens of both patients. Sequence analysis revealed an identical DNA sequence of HHV-8 in KS tissue of both siblings, but the sequence in our cases differs in one base pair at position 67 from the previously published HHV-8 KS330Bam fragment. The findings indicate that besides the yet poorly defined genetical factors involved in the pathogenesis of KS, HHV-8 may act as a cofactor also in familial KS. In addition, our data demonstrate that HHV-8 is found in all epidemiological forms of KS, including the rarely occurring familial KS. Familial KS may act as a further model to study the interaction of an oncogenic virus with genetic host factors in the context of a neoplastic disorder.

Calcifying basal cell carcinomas.

BACKGROUND: Dystrophic calcification refers to calcium deposition occurring in association with local tissue injury or abnormality. Little is known about dystrophic calcification occurring in malignant cutaneous neoplasms. OBJECTIVE: The purpose of this study was to determine the frequency and pattern of dystrophic calcification occurring in basal cell carcinomas and squamous cell carcinomas. METHODS: Consecutive cases (200) of basal cell carcinoma and squamous cell carcinoma were reviewed for evidence of dystrophic calcification. Histologic subtype, location of calcification, and tumor site were noted. RESULTS: Of 200 basal cell carcinomas, 41 (21%) showed evidence of dystrophic calcification, compared with 6 (3%) squamous cell carcinomas. Calcifying basal cell carcinomas were more likely to have a more aggressive histologic subtype and to be located on the trunk. CONCLUSION: Skin calcium binding protein may be responsible for the calcium deposition and account for the discrepancy seen between the frequency of calcification occurring in basal cell carcinomas and squamous cell carcinomas. As with other tumors with follicular differentiation, calcium deposits were located in keratin-filled cysts. This pattern of dystrophic calcification may be a marker of follicular differentiation.

Fingertip dermatitis in an ophthalmologist caused by proparacaine.

PURPOSE: To report the late diagnosis of allergic response to proparacaine hydrochloride in an ophthalmologist. METHOD: Case report. In a 49-year-old practicing ophthalmologist, the history, clinical findings of fingertip dermatitis, skin pathology, and skin patch testing are described. RESULTS: Proparacaine, as the initiating agent, took almost 3 years to identify because of the unusual pattern of allergy. We explored numerous treatment options before identifying and removing the offending agent. CONCLUSIONS: Ophthalmologists may be exposed to proparacaine on a daily basis. This unique report heightens the awareness of this rare work-related complication.

Apoptosis with positive direct immunofluorescence findings in a patient with necrolytic migratory erythema.

A 45-year-old man with a glucagonoma and necrolytic migratory erythema is described. Clinical, radiographic, and histologic features were typical for this syndrome. However, a skin biopsy specimen for direct immunofluorescence examination revealed apoptotic keratinocytes that stained positive with immunoglobulins, fibrinogen, and C3. These immunofluorescence findings were initially interpreted erroneously as showing erythema multiforme or a related disorder. We present a unique case of necrolytic migratory erythema with positive direct immunofluorescence findings reflecting immunoglobulin and complement deposition within dyskeratotic epithelial cells.

Exaggerated arthropod-bite lesions in patients with chronic lymphocytic leukemia: a clinical, histopathologic, and immunopathologic study of eight patients.

BACKGROUND: Unusual papulovesicular lesions resembling arthropod bites have been described in patients with chronic lymphocytic leukemia (CLL). OBJECTIVE: Our purpose was to describe and characterize further the clinical, histopathologic, and immunopathologic features of these lesions. METHODS: Eight patients were identified retrospectively who had CLL and characteristic skin lesions. Clinical and histologic features were recorded. Skin biopsy specimens were analyzed immunohistochemically for eosinophil granule major basic protein, eosinophil-derived neurotoxin, neutrophil elastase, and mast cell tryptase. RESULTS: The clinical features, including the lesional distribution, suggested arthropod bites, although most patients could not recall having been bitten. Mixed T- and B-cell lymphoid cell infiltrates were present within lesions, along with prominent eosinophil infiltration and eosinophil granule protein deposition. CONCLUSION: Exuberant papulovesicular lesions develop in patients with CLL apparently as an exaggerated response to arthropod bites. Prominent eosinophil infiltration and degranulation within these lesions likely contribute to the severity of symptoms.

Asteroid bodies and other cytoplasmic inclusions in necrobiotic xanthogranuloma with paraproteinemia.

BACKGROUND: Necrobiotic xanthogranuloma (NXG) with paraproteinemia is a distinctive palisading granuloma of the skin. Extracutaneous lesions are rarely present. OBJECTIVE: The purpose of this study was to confirm the presence and significance of giant cell asteroid bodies and other cytoplasmic inclusions in NXG with paraproteinemia. METHODS: Skin biopsy specimens from 24 patients with NXG with paraproteinemia were reviewed; autopsy and lung biopsy specimens from two patients were stained for iron, calcium, and polysaccharide. RESULTS: Giant cell asteroid bodies were observed in skin biopsy specimens of 8 (33%) of the 24 patients. In addition, large acidophilic polygonal cytoplasmic inclusions were observed in myocardial tissue of one of the autopsy cases. Iron and calcium were not found. CONCLUSION: Asteroid bodies and other inclusions can be present in the giant cells of NXG with paraproteinemia. They are as frequent as, or more frequent than, in other granulomatous diseases and should be considered in the diagnosis of NXG with paraproteinemia.

Granuloma annulare: radiographic demonstration of progressive mutilating arthropathy with vanishing bones.

Granuloma annulare, a relatively common cutaneous disorder of unknown origin, is named for its predominantly papular lesions, which tend to group and coalesce in an annular fashion. Clinically distinct subtypes are divided into localized, generalized, perforating, and subcutaneous forms. Only the superficial granulomata of the extremities in subcutaneous granuloma annulare have been radiographically depicted in the literature. We describe and illustrate what we believe is the first radiographic presentation of bone and joint involvement with advanced, generalized granuloma annulare.

Phenytoin-induced linear IgA bullous disease.

Drug-induced linear IgA bullous disease most commonly occurs after exposure to vancomycin, but other medications may also trigger the eruption. We describe a 78-year-old man with linear IgA bullous disease related to treatment with phenytoin.

Multinucleate cell angiohistiocytoma.

Multinucleate cell angiohistiocytoma (MCA) is a benign fibrohistiocytic vascular tumor affecting predominantly the acral sites in elderly women. The disorder is often confused clinically and histologically with Kaposi's sarcoma. We report the case of a 63-year-old woman with MCA and describe the main clinical and histopathologic features of this disorder.

Dermatopathologic variants of malignant melanoma.

An appropriate biopsy is the pivotal procedure that facilitates accurate histopathologic diagnosis of a pigmented skin lesion. Excisional skin biopsy is the method of choice for removing a suspected malignant melanoma. More than 95% of malignant melanomas that involve the skin belong to one of the four most common clinicopathologic categories: superficial spreading, nodular, lentigo maligna, and acral lentiginous melanoma. A small but important group of cutaneous melanomas can be classified as unusual variants. Many of these unusual variants have a distinct histopathologic appearance; they include desmoplastic melanoma, neurotropic melanoma, pedunculated melanoma, metastatic melanoma, amelanotic melanoma, melanoma arising within a benign nevus, regressing ("invisible") melanoma, and balloon cell melanoma. Other lesions may simulate malignant melanoma histopathologically. Immunohistochemical stains, such as S-100 protein, vimentin, keratin, and HMB-45, are useful for distinguishing these lesions from true melanoma.

Cutaneous extravascular necrotizing granuloma (Winkelmann granuloma): confirmation of the association with systemic disease.

BACKGROUND: An unusual palisading granuloma has been described in patients with immunoreactive diseases. Multiple names have been given to this lesion. OBJECTIVE: Our aim was to verify whether a distinct palisading granuloma can be used as a marker for systemic disease. We also propose unifying nomenclature. METHODS: Thirty-four biopsy specimens from 22 patients were selected for study on the basis of histologic criteria. The medical histories of these patients were subsequently reviewed for clinical information. RESULTS: At least 21 of the 22 patients with cutaneous extravascular necrotizing granuloma had evidence of an underlying immunoreactive systemic illness. In each, the systemic disease preceded or was diagnosed concurrently with the cutaneous lesions. CONCLUSION: The cutaneous extravascular necrotizing granuloma has unique clinical and histologic features. In a great majority of cases, a systemic immunoreactive disease is present.

Familial Kaposi's sarcoma.

Familial cases of Kaposi's sarcoma are exceedingly rare. We identified an 85-year-old man with Kaposi's sarcoma. Multiple lesions were present on the feet. Previously, the man's 84-year-old sister presented with similar lesions on the lower extremity. Both siblings were lifelong residents of the United States and were of English/German descent. Neither had risk factors for infection with human immunodeficiency virus (HIV). On histologic examination, lesions in both patients demonstrated vascular slits, hemorrhage, and dense aggregates of spindle cells within the dermis.

Unusual cutaneous lymphomas.

BACKGROUND: New variants of cutaneous lymphoma other than mycosis fungoides and Sezary syndrome continue to be described. OBJECTIVE: A practical classification of cutaneous lymphomas is presented with detailed discussions of angiocentric T cell lymphoma, subcutaneous T cell lymphoma, and malignant angioendotheliomatosis. METHODS: The recent pertinent medical literature relating to unusual cutaneous lymphomas is reviewed. RESULTS/CONCLUSION: The utilization of immunohistochemistry in the dermatopathology laboratory has greatly enhanced the ability to recognize and categorize cutaneous lymphoma. Some illnesses previously believed to be non-neoplastic diseases are malignant lymphomas.

Clinical and histologic spectrum of human T-cell lymphotropic virus type I-associated lymphoma involving the skin.

BACKGROUND: Adult T-cell leukemia/lymphoma (ATL) is associated with infection with human T-cell lymphotropic virus type I (HTLV-I). OBJECTIVE: The objective was to describe the clinical, histopathologic, and immunologic features in three patients with ATL involving the skin. METHODS: Clinical histories and skin biopsy specimens were reviewed. Immunophenotypic studies were performed on peripheral blood lymphocytes (three patients) and on skin biopsy specimens (one patient). RESULTS: Serologic testing in each patient was positive for HTLV-I. Specific cutaneous lesions of ATL were diverse. Histologic features included markedly epidermotropic lymphoid infiltrates and dermal aggregates of lymphocytes and macrophages resembling granulomas. One patient died 3 months after diagnosis; the other two are alive with residual lymphoma. CONCLUSION: A spectrum of clinical, histologic, and immunophenotypic features are seen in ATL involving skin. Those cases with a chronic course may resemble mycosis fungoides clinically and histologically. Serologic testing for HTLV-I is recommended in all patients with cutaneous lymphoma from endemic areas and in those with other risk factors for HTLV-I infection.