Serologic profiles as immunologic markers for different clinical presentations of lupus erythematosus.

The clinical and laboratory features of 55 patients with lupus erythematosus (LE), grouped on the basis of six nuclear immunofluorescent pattern results commonly encountered in this disease were examined. Serologic profiles of antinuclear antibodies (ANA), anti-DNA and anti-ENA results can serve as immunologic markers in LE for a benign subset and two other groups with a different incidence of certain clinical characteristics. The large speckle-like thready pattern without antibodies to DNA or ENA is an immunologic marker for a benign LE subset, with generalized skin lesions with or without joint involvement only. Significant levels of the anti-DNA antibodies with the shrunken peripheral, peripheral, or leukocyte-specific ANA with a particulate pattern are markers for severe systemic involvement. The thready pattern with antibodies to ENA (Sm antigen) and leukocyte-specific ANA without a particulate pattern, with or without antibodies to DNA or ENA, indicate less severe systemic disease.

Bleomycin-lidocaine mixture reduces pain of intralesional injection in the treatment of recalcitrant verrucae.

Intralesional bleomycin has been effective treatment of recalcitrant verrucae since 1970, but one major drawback is the moderate to severe pain associated with the injection. To minimize procedural discomfort, bleomycin can be reconstituted in lidocaine. It is effective, associated with minimal morbidity, and well tolerated by most patients.

Ichthyosiform sarcoidosis. Report of two cases and a review of the literature.

Although acquired ichthyosis has been associated with a number of systemic illnesses, an association with sarcoidosis has rarely been reported. We report two patients with acquired ichthyosis of the lower extremities whose diagnosis of cutaneous sarcoidosis was established by histologic examination. Systemic involvement in both patients included ocular and pulmonary disease. A diagnosis of sarcoidosis must be considered when a patient presents with acquired ichthyosis.

Chrysiasis following gold therapy for rheumatoid arthritis: ultrastructural analysis with x-ray energy spectroscopy.

Chrysiasis is a rare, permanent, pigmentation of the skin caused by the parenteral administration of gold preparations followed by subsequent exposure to ultraviolet light. We report a case of chrysiasis following gold therapy for rheumatoid arthritis, wherein ultrastructural analysis combined with x-ray energy spectroscopy afforded precise identification and localization of the gold pigment in skin biopsy specimens.

Tinea nigra masquerading as acral lentiginous melanoma.

Tinea nigra is a superficial mycosis that may mimic serious pigmentary lesions. A lesion recently encountered on the foot was suspected of being a malignant melanoma. Histologic and mycologic studies, done after a biopsy was obtained, demonstrated Exophilia werneckii in the stratum corneum. Tinea nigra should be considered in the diagnosis of pigmented lesions of the hands and feet. A KOH examination is a simple and rapid means of demonstrating this entity.

Large speckle-like thready and thready antinuclear antibody patterns as markers for different clinical presentations in lupus erythematosus.

Fifty-one patients with lupus erythematosus were studied retrospectively. They were chosen on the basis of their antinuclear antibody (ANA) immunofluorescent pattern. Only those with the thready or the large speckle-like thready patterns were studied. Autoantibody profiles consisting of ANA, anti-single-stranded deoxyribonucleic acid (ssDNA) antibody, and anti-extractable nuclear antigen (ENA) antibody determinations were obtained. The patients with the thready ANA pattern and anti-ENA (Sm) antibodies had a significantly higher incidence of pulmonary, joint, and renal involvement than the anti-ENA negative patients with the large speckle-like thready pattern. There was also a significantly higher incidence of Raynaud's phenomenon in patients with the thready pattern than in those with the large speckle-like thready pattern. Photosensitivity was seen significantly more frequently in the patients with the large speckle-like thready pattern than in those with the thready pattern.

Rippled-pattern trichomatricoma. Histological, immunohistochemical and ultrastructural studies of an immature hair matrix tumor.

A hair matrix tumor showing an unusual tumor cell arrangement was found at the base of a solitary trichoepithelioma. Coexisting with solid epithelial islands and immature hair follicle-like stroma resembling the Verocay bodies of neurilemmoma or "ripplemarks" on waves were found. In other areas myxomatous degeneration of the stroma changed the rippling into a cribriform pattern. In some parts of the tumor there was a dense melanin pigment associated with MEL5 stained melanocytes. S-100 and CD1 (OKT6) antigen stains demonstrated Langerhans cells scattered in the parenchyma and less frequently in the stroma. The majority of tumor cells were considered immature pilar cortical cells because of the following: 1. HKN-6 was strongly positive; 2. a large number of melanocytes were associated with tumor cells in some foci; 3. ultrastructurally immature tumor cells, which had electron-dense tonofilaments and many desmosomes, were transformed without production of trichohyalin granules into semikeratinized cells which showed nuclear degeneration and loss of electron density in tonofilaments. This tumor, however, has not attained the degree of differentiation observed in trichoblastoma (1) another example of an immature cortical cell tumor. Squamous eddy-like or horn pearl-like foci of incomplete keratinization and large keratin-filled cysts were also present within the immature parenchyma, indicating that some immature cells were differentiating toward non-cortical cells, as found in the outer sheath. We would like to designate this tumor "rippled pattern trichomatricoma", a new entity.

Characterization of apoA- and apoB-containing lipoprotein particles in a variant of familial apoA-I deficiency with planar xanthoma: the metabolic significance of LP-A-II particles.

This study describes a variant of familial apoA-I deficiency associated with a moderate risk for premature coronary artery disease. The proband, a 25-year-old man of Philippine origin, and his 62-year-old maternal aunt had peripheral corneal opacification, xanthelasma, and planar xanthoma; the aunt had coronary artery bypass surgery at 61 years of age. Proband's parents and three brothers were asymptomatic and apparently healthy. The characteristic apolipoprotein features of affected patients were the immunochemically and chemically undetectable apoA-I, reduced levels of apoA-II, apoC-II, apoC-III, and apoD, and normal levels of apoB and apoE; except for negligible levels of high density lipoprotein (HDL)-cholesterol (2-3 mg/dl), their plasma lipid profile was normal. The apoA-I levels in all five unaffected relatives were more than one SD below the normal mean values for their age and sex; the HDL-cholesterol levels of proband's unaffected brothers were below the 10th percentile of normal control values. Patient's very low density lipoprotein (VLDL), low density lipoprotein (LDL), and HDL contained 1.4, 80.4, and 18.1%, whereas those of control subjects contained 2.7, 28.8, and 68.1% of the total apolipoprotein mass, respectively. In unaffected relatives, the levels of LP-A-I, but not LP-A-I:A-II, were significantly lower than in controls. Neither of the two patients had detectable concentrations of LP-A-I or LP-A-I:A-II. Their HDL only consisted of LP-A-II particles, the levels of which (7-13 mg/dl) were similar to those of unaffected relatives or controls. There was no difference in the lipid composition of LP-A-II between patients and their relatives. However, LP-A-II from patients contained substantial amounts of apoC-peptides and apoE (0.40-0.98 mg/mg apoA-II), whereas those from unaffected relatives were free of these minor apolipoproteins. In patients, among all four major apoB-containing lipoproteins, only the levels of LP-B and LP-B:C were slightly higher than those in controls. Results of this study suggest a genetic cause for this variant of apoA-I deficiency characterized most probably by autosomal recessive inheritance. It appears that patients are likely to be homozygous for a gene present in single dose in the parents and brothers of the affected proband.(ABSTRACT TRUNCATED AT 400 WORDS)

Cutaneous granulomatous lesions in patients with ataxia-telangiectasia.

Ataxia-telangiectasia is a genetic syndrome with progressive cerebellar ataxia, oculocutaneous telangiectasias and other skin manifestations, variable immune system defects, chronic progressive sinopulmonary disease, and a high incidence of cancer. Cutaneous granulomas developed and persisted in eight patients with ataxia-telangiectasia, despite treatment with intravenously administered immune globulin, topical antibiotic therapy, and potent topical corticosteroid therapy. We were unable to identify an infectious agent; the granulomas may develop in an attempt to localize antigen in the presence of a dysfunctional immune system.