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BACKGROUND: Coma-induced bullae and sweat gland necrosis is a rare clinicopathological entity often associated with drug-induced coma. SUBJECT: We report a case with clinical and histopathologic findings characteristic of blisters and sweat gland necrosis occurring in a non-comatose patient. CONCLUSIONS: Skin blisters with underlying sweat gland necrosis is an entity previously reported to occur in comatose patients, our findings open new questions about the role of the drugs in the pathogenesis of those conditions.

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Due to diverse clinical and histopathological presentations, diagnosis of secondary syphilis can occasionally prove challenging. This is especially true in the setting of human immunodeficiency virus (HIV) infection. Variable clinical presentations of secondary syphilis in HIV disease may result in an incorrect diagnosis and an inappropriate treatment regimen. Similarly, the histology of secondary syphilitic lesions may show considerable variation, depending on the clinical morphology of the eruption. We report 2 cases of secondary syphilis in HIV-1-infected patients with cutaneous lesions of variable clinical presentation and an unusual lymphoid infiltrate simulating mycosis fungoides.

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We report the unique case of a 50-year-old African American female with pulmonary sarcoidosis who presented with a new ichthyosiform eruption symmetrically located on the anterior shins and surrounded by red, translucent, intradermal papules. A skin biopsy of a new red papule showed features consistent with granuloma annulare (GA) with positive mucin staining, and an older hyperpigmented papule showed classic dermal noncaseating granulomas consistent with sarcoidosis. Recent reports have clearly demonstrated GA occurring in association with sarcoidosis, but this is the first report that suggests that a GA lesion may develop into a sarcoidal granuloma. We propose that GA may act as a precursor lesion to the more mature sarcoidal granuloma. This case further underscores the importance of careful clinicopathologic correlation.

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We describe an unusual case involving the simultaneous occurrence of segmental neurofibromatosis (Type V NF) in a patient with a large nevus sebaceus of Jadassohn in the same physical distribution. Causative mechanisms of development of these 2 genetic disorders have not been definitively linked. Factors producing these diseases probably involve similar tissues at the same point in development because both have been reported in association with central nervous system anomalies and have been classified among the neurocutaneous syndromes. This is a case of a nevus sebaceus occurring in association with and in the same physical distribution as segmental NF. These disorders most likely represent a spectrum of disease within the phakomatoses.

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We describe a 53-year-old white woman with dermatomyositis (DM) who had additional clinical findings of pityriasis rubra pilaris (type Wong dermatomyositis) with histopathologic features of both pityriasis rubra pilaris (PRP) and porokeratosis. Type Wong dermatomyositis was originally described in 11 patients by Wong in 1969 and has been reported in 5 additional patients. This is a rarely described phenomenon in which patients with DM develop cutaneous hyperkeratotic lesions that resemble PRP and histologically show follicular hyperkeratosis and hair follicle destruction. Arrector pilorum muscles also show degenerative findings and myositis. We believe that this is the first reported case of a patient with type Wong DM who also has clinical and histologic features suggestive of porokeratosis. This is important because of the association of adult-onset dermatomyositis with internal malignancy and the well-documented association of porokeratosis with immunosuppression. These clinical and histologic findings serve as markers for malignancy in patients with DM. These patients warrant a complete review of systems and investigation for age-appropriate neoplasms as well as close long-term follow-up by dermatologists to ensure that these cutaneous eruptions are not overlooked.

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Cowden syndrome (CS) is an autosomal dominant inherited disorder characterized by hamartomas in a variety of tissues including the skin, thyroid, breast, endometrium, and the brain. Individuals with CS are predisposed to development of malignancy in these organs, especially the breast and the thyroid. We describe 3 unrelated individuals with CS associated with germline PTEN mutations. While the frameshift (375insTTTA) and the missense (Gly69Arg) mutations reported herein are novel in CS, the nonsense (Arg130stop) mutation has been described in 2 families with CS and in a single family exhibiting both CS and Bannayan Zonana phenotype.

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A 22-year-old black man developed fever, chills, fatigue, night sweats, tender lymphadenopathy, and a generalized, pruritic, macular eruption 3 weeks after starting minocycline therapy for acne. His illness was also characterized by a palpable spleen tip, marked lower extremity and scrotal edema, and generalized lymphadenopathy. The patient had leukocytosis with a large percentage of atypical lymphocytes on peripheral smear and liver dysfunction. Titers for Epstein-Barr virus, hepatitis B, toxoplasmosis; and cytomegalovirus were all negative. Human immunodeficiency virus-1 viral load and antibodies were also negative. Marked improvement was noted after the discontinuation of minocycline and the use of systemic corticosteroids. With the negative viral serologies, the clinical picture was most consistent with an infectious mononucleosis-like syndrome produced by the minocycline ingestion.

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We present a rare case of metastatic adenocarcinoma of the parotid gland to the skin. Reviewing the histologic features of the primary parotid gland and comparing the microscopic sections and immunohistochemical studies, we concluded the skin tumor to be metastases from the parotid adenocarcinoma. By histologic examination alone, it is difficult to distinguish an eccrine sweat gland carcinoma from a metastatic carcinoma of the salivary gland. Immunohistochemical analysis may not be conclusive. Therefore, clinical history and clinicopathologic correlation are essential in arriving at an accurate diagnosis in these cases.

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Eccrine angiomatous hamartoma (nevus) is a rare form of congenital tumorous malformation with proliferation of eccrine secretory coils and ducts, surrounding capillary angiomatous channels and occasionally other minor elements. To date, there have been only about 24 cases reported in the literature. We report an additional case with more detailed description of the microscopic findings, including immunohistochemical observations. The patient was a 28-year-old female who presented with painless, flesh- to reddish brown-colored, violaceous or bluish subcutaneous nodules on the extremities and trunk. The tumors did not show sweating following exertion. The histologic features were comparable to the previously reported cases. The hamartomatous eccrine sweat glands and ducts and a few apocrine glands demonstrated qualitatively diminished antigens commonly found in the eccrine sweat apparatuses, such as carcinoembryonic antigen (CEA) and S-100 protein. The findings of CD34, CD44, human nerve growth factor receptor and Ulex europaeus antigens have not been previously reported. The histologic features suggested a "hamartomatous" growth rather than a true neoplastic process.

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Unilateral nevoid telangiectasia syndrome can be classified into two major categories: congenital and acquired. There have been reports showing an increase in skin estrogen and progesterone receptors in patients with this syndrome. We report a case of acquired unilateral nevoid telangiectasia syndrome related to pregnancy with no estrogen and progesterone receptors. Results of more studies are needed to demonstrate the correlation of systemic estrogen levels with the presence of estrogen and progesterone receptors in the skin.

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BACKGROUND: Substance P, an undecapeptide neurotransmitter, has been implicated in the pathophysiology of psoriasis and pruritus. OBJECTIVE: Safety and efficacy of topical capsaicin, a potent substance P depletor, were evaluated in patients with pruritic psoriasis. METHODS: Patients applied capsaicin 0.025% cream (n = 98) or vehicle (n = 99) four times a day for 6 weeks in this double-blind study. Efficacy was based on a physician's global evaluation and a combined psoriasis severity score including scaling, thickness, erythema, and pruritus. RESULTS: Capsaicin-treated patients demonstrated significantly greater improvement in global evaluation (p = 0.024 after 4 weeks and p = 0.030 after 6 weeks) and in pruritus relief (p = 0.002 and p = 0.060, respectively), as well as a significantly greater reduction in combined psoriasis severity scores (p = 0.030 and p = 0.036, respectively). The most frequently reported side effect in both treatment groups was a transient burning sensation at application sites. CONCLUSION: Topically applied capsaicin effectively treats pruritic psoriasis, a finding that supports a role for substance P in this disorder.

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A case of periorbital and eyelid edema in an eighteen-year-old student is presented as the initial manifestation of acute infectious mononucleosis occurring one week before the typical prodrome. Although periorbital and eyelid edema have been reported in about 50 percent of patients with early infectious mononucleosis, its occurrence is much less frequent in clinical practice. Physicians, particularly those specializing in the treatment of cutaneous and ocular diseases, should now include acute infectious mononucleosis in the differential diagnosis of periorbital and eyelid edema.

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Four cases of rare familial multiple eccrine spiradenomas showing features of dermal cylindromas and associated with epithelioma adenoides cysticum of Brooke are reported. Skin biopsy specimens were obtained from three generations of this family and routine histochemical and immunoperoxidase stains were used. The eldest affected family member had multiple disfiguring facial and scalp tumors, which precipitated episodes of depression. Unlike other cutaneous genetic disorders, such as neurofibromatosis and tuberous sclerosis, the cutaneous adnexal tumors occurring in these patients continue to erupt and grow during their lifetimes.

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Ultrastructural studies of Kaposi's sarcoma (KS) from skin biopsies of 24 patients (eight with acquired immunodeficiency syndrome (AIDS) and 16 without) were performed to delineate the nature of hyaline globules and vascular slits. These structures have been regarded as one of the important criteria for the recognition of KS under light microscopy. Histochemical and immunochemical studies were also performed to correlate with the electron microscopic (EM) observations. The most remarkable EM findings of KS were the intracytoplasmic lumen formation and erythrophagocytic activities of the neoplastic cells, particularly in the mature nodular, or neoplastic stage. The spindle-shaped or ovoid neoplastic cells frequently contained one to several intact and fragmented red blood cells. The intracellular and extravasated erythrocytes were often arranged in single files, giving these vascular slits an elongated appearance on longitudinal sections. The phagocytic activities of the neoplastic cells were demonstrated by the presence of membrane-bound lysosomes containing phagocytized erythrocytes and their partially digested forms (erythrophagosomes) adjacent to pinocytotic vesicles, prominent rough endoplasmic reticulum, and Golgi apparatus, as well as scattered, small, membrane-bound lysosomal granules, some of which were attached to the erythrophagosomes. The erythrophagosomes underwent various stages of disintegration. The partially digested red cells varied from 0.4 to 10 microns in diameter. The results of histochemical and immunochemical findings also strongly suggested that erythrophagosomes were most likely the hyaline globules (bodies) seen in light microscopy. The exact mechanism of erythrophagocytosis is uncertain. However, its consequences, erythrophagosomes, and intracytoplasmic lumen formation, particularly in the nodular or neoplastic stage in patients with and without AIDS, are among the important histologic features of KS.

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Focal acantholytic dyskeratosis (FAD) is a distinctive histologic pattern characterized by suprabasilar clefts surrounding dermal papillae (villi), acantholytic and dyskeratotic cells at all levels of the epidermis, hyperkeratosis, and parakeratosis. The features of FAD are typically seen in Darier's disease, warty dyskeratoma, and transient acantholytic dermatosis; they are also present in a variety of cutaneous neoplastic and nonneoplastic lesions. FAD, however, has not been previously described in lesions of inflammatory dermatoses. We report a case of FAD occurring in lesions of pityriasis rubra pilaris (PRP). To the best of our knowledge, this is the first reported case of this kind. We also review the pertinent literature.

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Squamous-cell carcinoma may arise in scars of chronic discoid lupus erythematosus. Although there have been 19 cases reported previously, detailed histopathologic features of this entity have not been recorded. We report a patient with extensive chronic discoid lupus erythematosus involving the scalp with subsequent development of multiple squamous-cell carcinomas. The tumors were locally aggressive with recurrences and invasion into the underlying skull and dura. The patient died of respiratory failure 4 1/2 years after initial surgical treatment. There was no clinical evidence of metastasis. Squamous carcinoma arising in discoid lupus erythematosus can be regarded as a low-grade carcinoma. Although about 20% of patients developed local recurrences and metastasis developed in about 30%, fatality occurred in only two patients (10.5%). Pertinent literature is reviewed, and the histopathologic findings, differential diagnosis, and biologic behavior of this tumor are discussed.

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