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1/26. Autosomal dominantly inherited generalized basaloid follicular hamartoma syndrome: report of a new disease in a north carolina family.

    BACKGROUND: An 8-year-old girl presented with hundreds of milia, measuring 1 to 2 mm; comedone-like lesions; skin-colored and hyperpigmented papules on the face, scalp, ears, neck, upper trunk, and lower arms along with diffuse scalp hypotrichosis; and pinpoint palm/sole pits. Onset was in early childhood and the disease was historically present in 6 generations. OBJECTIVE: Our objectives were to delineate the clinical and histopathologic features and mode of inheritance as a base for gene studies. methods: Eighteen family subjects were studied. Twenty-six skin biopsy specimens were examined. A detailed pedigree was constructed. A complete literature search was done concerning diseases with generalized basaloid follicular hamartomas. RESULTS: The lesions were basaloid follicular hamartomas and other folliculocentric abnormalities. Inheritance was autosomal dominant. Extensive literature search confirmed the finding of a unique genodermatosis. CONCLUSION: A new genodermatosis termed dominantly inherited generalized basaloid follicular hamartoma syndrome was defined by delineating its clinical and histopathologic features and mode of inheritance and by extensive literature review.
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2/26. Pustular idiopathic recurrent palmoplantar hidradenitis: an unusual clinical feature.

    A 12-year-old boy had painful lesions on both soles. He had had a similar episode several months before that resolved spontaneously. physical examination showed erythematous, extremely tender nodules on both plantar surfaces and the toes. The second and third left toes had small pustules on top of the nodules. There was no palmar involvement. The clinical features, pathologic findings, and self-limited course suggested recurrent palmoplantar hidradenitis. This distinctive entity of unknown origin appears during childhood and is characterized histologically by a neutrophilic infiltrate affecting the eccrine glands. We report the case of a patient with unusual clinical features.
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3/26. methotrexate-induced acral erythema with bullous reaction.

    Chemotherapy-induced acral erythema (CIAE), a toxic reaction to a number of different chomotherapeutic agents, causes a symmetrical, painful erythema of both the palms and soles which is self-limiting. The association of this syndrome with methotrexate is unusual; only nine cases have been reported in the literature. We describe the tenth case of this syndrome associated with methotrexate, which is also the third case of the bullous variant of methotrexate-induced acral erythema. Our case is unusual in that the acral erythema was present only on the soles of the feet and in that it was associated with the presence of diffuse maculopapular lesions over the legs and trunk.
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4/26. Palmoplantar lichen planus presenting with vesicle-like papules.

    Palmoplantar lichen planus is a rare, localized variant of the disease that may create difficulty in diagnosis if it is present as an isolated finding. Although several morphological patterns may be seen, plaques or small papules with compact hyperkeratosis are usually observed. We present a 25-year-old woman with a one-month history of slightly pruritic, red papules on her palms and soles. Dermatologic examination revealed numerous, asymptomatic, unscaly, red papules on her palms and soles, some of them resembling vesicles and white reticulate plaques on both sides of the buccal mucosa. The diagnosis was established by the typical histopathological features of lichen planus. Although resistant to topical corticosteroids, she responded well to systemic corticosteroid therapy, and no recurrence was observed during the follow-up period of one year.
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5/26. Pustular acral erythema in a patient with acute graft-versus-host disease.

    Acral erythema is a well-known side-effect of chemotherapy treatment but it is not common in patients undergoing bone marrow transplant. We report a post-transplant patient with clinical and histological acute graft-versus-host disease (GVHD) who concurrently developed acral erythema presenting as painful, well-defined and self-limiting palmar erythema with pustules. A skin biopsy from the palm showed abnormal keratinocyte maturation and eccrine squamous syringometaplasia. This case illustrates the difficulties in establishing the differential diagnosis of cutaneous eruptions in patients undergoing bone marrow transplant.
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6/26. A case of adult T cell leukemia with bullae in the palmoplantar regions followed by a crisis.

    A 48-year-old Japanese female who had had chronic ATL for 4 years suddenly developed vesicles on the palms and soles. Histologically, these bullae were specific lesions of ATL. After a tendency toward improvement, a crisis appeared with increases in the following: peripheral white blood cells, atypical lymphocytes, CD25 positive cells, serum LDH, and soluble IL-2R. Palmoplantar bullae, a rather rare finding, may be indicative of a following crisis.
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7/26. phenytoin-induced linear IgA dermatosis mimicking toxic epidermal necrolysis.

    A 60-year-old woman developed a severe widespread blistering eruption that also involved the palms and soles, but spared the mucosae, approximately 7 days after starting phenytoin. phenytoin was commenced postoperatively after a craniotomy resection of a glioblastoma multiforme. The clinical features resembled that seen in toxic epidermal necrolysis. However, the patient was systemically well and the histology and immunofluorescence revealed linear IgA dermatosis. The skin lesions began to slowly heal 2 weeks after discontinuation of the phenytoin.
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8/26. Eosinophilic pustular folliculitis starting initially only with palmoplantar pustular lesions. Report of a case and review of the literature.

    We report a 23-year-old Japanese male with eosinophilic pustular folliculitis (EPF) that had started with palmoplantar rash. Only when follicular pustules appeared on the bilateral cheek 31 months later, we revised our initial diagnosis of pustulosis palmaris et plantaris (PPP) to EPF, and all the skin eruptions cleared mostly with indomethacin. A review of the Japanese literature for the past 20 years disclosed that in 207 cases of EPF so far reported, palmoplantar pustular lesion was noted in 38 (18%). Among them, in 16 cases (8%) the skin lesions started first from the palmoplantar region with the average period of 26 months until the appearance of other eruptions of EPF. None of them was diagnosed as EPF when skin lesions were localized only to the palmoplantar region. When we find pustules on the palmoplantar region, we should suspect the possibility of early lesions of EPF as well as PPP. Histopathologic demonstration of multilocular pustules located in the upper epidermis containing numerous eosinophils in the palmoplantar pustular lesions, together with the dramatic therapeutic response to indomethacin greatly favor the diagnosis of EPF.
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9/26. vancomycin-induced linear IgA disease manifesting as bullous erythema multiforme.

    BACKGROUND: vancomycin-induced linear immunoglobulin a (IgA) disease, an autoimmune, blistering disease in response to vancomycin administration, is characterized by a subepidermal, vesiculobullous eruption and linear IgA deposition along the basement membrane zone on direct immunofluorescence. CASE REPORT: We report the case of an 81-year-old man treated with vancomycin who developed diffuse erythema multiforme and tense bullae involving the palmoplantar surfaces. Discontinuation of vancomycin therapy resulted in complete resolution of this patient's cutaneous eruption. RESULTS: biopsy of a representative skin lesion demonstrated lichenoid interface dermatitis with focal subepidermal clefting, dyskeratosis, and prominent eosinophils. Direct immunofluorescence showed linear basement membrane staining with immunoreactants to IgA; indirect immunofluorescence demonstrated the presence of circulating IgG antibodies binding in an intercellular pattern. immunoprecipitation studies using the patient's serum revealed 210, 130, and 83 kDa target antigens. CONCLUSIONS: Presenting with an initial clinical picture suggestive of bullous erythema multiforme, this patient's subsequent clinical course and direct immunofluorescence confirm the diagnosis of linear IgA bullous disease (LABD). His indirect immunofluorescence findings and immunoprecipitation results suggest that circulating non-IgA antibodies may represent a newly recognized immunopathologic feature of vancomycin-induced linear IgA disease, underscoring the variable and unpredictable manifestations of this drug-induced cutaneous disease.
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10/26. Drug eruption due to peplomycin: an unusual form of stevens-johnson syndrome with pustules.

    A rare case of stevens-johnson syndrome (SJS) due to peplomycin in a 48-year-old man is described. The patient had squamous cell carcinoma on the scalp and underwent preoperative neoadjuvant chemotherapy with peplomycin. On the fifth day of the chemotherapy, he developed a fever and multiple dusky violaceous erythematous areas and pustules on his trunk, thighs, and palms. Erosive erythema and erosions also developed on his soles, scrotum, and oral mucosa. A biopsy specimen taken from the eruption on the thigh revealed marked liquefaction degeneration of the basal layer of the epidermis. Laboratory examinations demonstrated aggravation of liver function. Additionally, the patient developed conjunctivitis and corneal erosions. Although he had some subcorneal pustules, we diagnosed the case as an unusual form of SJS because of severe mucous membrane involvement. Oral prednisolone was administered, and the symptoms subsided. Then the patient underwent wide local excision. One month after surgery, we performed patch tests and a lymphocyte stimulation test with negative results. Then we re-administered peplomycin starting with 1/20 of a daily dose and gradually increasing the dose each day. After administration of the regular daily dose, the patient had a relapse of fever, eruptions, stomatitis, corneal erosions, and liver dysfunction. Therefore, a definite diagnosis of drug eruption due to peplomycin was made.
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