Trichoblastoma with sebaceous and sweat gland differentiation.
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Biomedical subjects
Publications and source records attributed to S N Chang.
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In an attempt to develop a safer pertussis vaccine, we successfully purified 3 pertussis protective antigens-pertussis toxin, filamentous hemagglutinin, and a 69-kDa outer membrane protein (also named pertactin), from Bordetella pertussis strain ATCC 9340. The toxicity of pertussis toxin could be effectively reduced by the treatment with formaldehyde 0.07% while preserving of a high degree of immunogenicity. By mixing purified pertussis antigens with diphtheria and tetanus toxoids (DT), we have formulated a DT acellular pertussis (DTaP) vaccine. Toxicity studies on body-weight gain in mouse, histamine sensitization, lymphocyte promoting, and Chinese hamster ovary cell clustering tests suggested that this DTaP vaccine is safer than a whole cell vaccine produced in France (DTP[F]). The formulated vaccine elicited high levels of anti-pertussis toxin antibodies in both mice and monkeys. In mice, a 2-fold neutralization of anti-pertussis toxin antibodies was produced by DTaP compared with DTP(F) vaccine and an acellular vaccine manufactured in Japan (DTaP[J]). More importantly, in intracerebral challenge assay in mouse, this vaccine also provided a better protection than DTaP(J).
Cutaneous chemotherapy reactions are more likely to be toxic than allergic. Epidermal dysmaturation refers to histologic changes that may be observed in the epidermis after any significant cytoreductive therapy. We report a case of epidermal dysmaturation occurring in a patient with acute myelogenous leukemia after antineoplastic chemotherapy.
Two cases showing changes of sclerotic fibroma developed in association with an inflammatory process, especially folliculitis. The lesion in the first case showed a well-circumscribed, nonencapsulated nodule in the dermis, which consisted of a perifollicular fibrotic area and a peripheral sclerotic area. In addition to the usual findings of sclerotic fibroma, spindle cells were heavily infiltrated in a storiform and fascicular pattern around the degenerated hair follicle, suggestive of dermatofibroma. The lesion in the second case showed the typical findings of sclerotic fibroma in association with folliculitis and hair follicle remnants. Our observations suggest that solitary sclerotic fibroma of the skin may be a degenerated or sclerotic end stage of other fibrous conditions, such as dermatofibroma, and that it may be induced by inflammation, especially folliculitis.
Primary cutaneous actinomycosis is very uncommon because of the exclusively endogenous habitat of the organism. We report a case of primary cutaneous actinomycosis characterized by histopathological "sulfur granules". The patient had a well-defined subcutaneous nodule on the left thigh without any other lesion and was treated with surgical excision and subsequent oral ampicillin for six weeks. There has been no recurrence during a 1-year period of follow-up.
Acinic cell adenocarcinoma is a rare salivary gland tumor which predominantly occurs in the parotid gland. A 46-year-old man presented with a left preauricular mass of one month duration; the pathologic report showed acinic cell adenocarcinoma. He was transferred to the oral and maxillofacial surgery department, and a total parotidectomy was performed. We present this interesting case of acinic cell adenocarcinoma from the parotid gland with cutaneous involvement.
Chronic actinic dermatitis is a rare disease worldwide and also in Korea. However there has been no clinicohistologic and photobiological analysis of chronic actinic dermatitis in Korea. We examined 11 patients who were diagnosed as chronic actinic dermatitis and the results of this study were compared with previous reports. Most patients were elderly men who had erythematous papules or lichenified plaques on sun-exposed areas such as the face, neck, and dorsum of the hands with severe itching sensation. All patients had severe sensitivity to UVB and biopsied specimens showed findings of chronic eczema. Five patients had positive photopatch test materials. The patients were treated with systemic and topical steroid, cyclosporine and antihistamine.
We report a case of epidermolysis bullosa simplex, Dowling-Meara type (EBS-DM), which was associated with congenital pyloric atresia (PA) and various urologic abnormalities, a diagnosis confirmed by immunofluorescence mapping and electron microscopic findings. Immunofluorescent mapping showed the serum from a patient with bullous pemphigoid faintly binding to the floor of the blister, and monoclonal antibodies against type IV and VII collagens were also stained on the floor of the blister. Electron microscopy showed epidermolytic cleavage and prominent clumping of tonofilaments in the basal and suprabasal keratinocytes. An abdominal radiograph and barium swallow showed a complete obstruction at the pyloric channel level. The widespread bullae healed without any scar formation and the bullae formation was localized on the extremities after 3 months of age without any specific treatment. Multiple urologic abnormalities such as bilateral hydronephrosis, hydroureter and a distended bladder with trabeculation were observed at 12 months of age. Currently, with the patient at 4 years of age, bullae still appear on the hands and feet and nail shedding can be observed. The patient's father, a paternal uncle and a paternal aunt had had similar bullous eruptions in infancy, all of which had improved spontaneously by the age of one.
Chronic recurrent pruritic papular eruptions in which a specific diagnosis cannot be established becomes a baffling experience to the dermatologist. We have met adult male patients with chronic recurrent pruritic papular eruptions, but their clinicopathological features are not described in English language textbooks. Our purpose was to study the clinical and histological features of this entity and review the various existing taxonomy. We conducted a study of 20 patients over a six year period by taking histories, performing skin biopsies, screening patch tests, and doing immunofluoresence studies. The eruptions occurred exclusively in male adults and had a predilection for the trunk and proximal extremities. The lesions were characterized by severely pruritic, nonfollicular, monomorphic, erythematous urticarial papules. There was no evidence of atopic diathesis or history of insect bite. Most patients had normal levels of serum eosinophils and IgE. The predominant histopathologic finding was a presence of perivascular infiltration of mononuclear cells with eosinophils. The patients followed a chronic course of at least six months with waxing and waning; systemic corticosteroids were the only effective treatment. Finally, all other pruritic erythematous papular dermatoses were ruled out. These cases comprise a distinct entity that has previously been mentioned in a few reports. Clear definition of this entity with an appropriate designation is in order to avoid confusion among dermatologists, and we propose the disease name "chronic papular dermatitis in adult men" as a variant of prurigo.
Isolated Kaposi's sarcoma (KS) of the glans penis is rare; only 37 cases are reported in the literature. Treatment of KS consists mostly of old modalities, some old methods in updated improved versions, and some new and experimental therapies. We report a case of isolated classical Kaposi's sarcoma involving multiple sites of the penis in a 54-year-old man who was treated with high-energy pulsed carbon dioxide (CO2) laser therapy, resulting in complete resolution of the lesions and no evidence of recurrence.
We describe a 19-year-old female patient who developed recurrent ulcerations limited to the orogenital mucosa for the last 3 years. She also developed dyspnoea 5 months after the onset of the orogenital lesions. Castleman's tumour of the retroperitoneum was found incidentally during routine physical examination. The diagnosis of paraneoplastic pemphigus (PNP) was made by pathological and immunological studies. The orogenital ulceration responded well to corticosteroid therapy, but severe bronchiolitis obliterans progressed despite intensive care. The patient eventually died from respiratory failure. This case demonstrates the diversity of clinical features of paraneoplastic pemphigus.
A 26-year-old Korean man exhibited generalized lentigines, a slight prominence of the chin, short stature, and a familial history consistent with the autosomal dominant mode of inheritance. The multiple lentigines appeared at the age of 1 and spread over his entire body including the scalp, palms, and soles by the age of 4. There were several large dark pigmented spots on the trunk, but no other skin abnormalities were found. We performed histologic studies of the normal skin and the lentigo by both electron and light microscopy with immunohistochemical stains. Routine hematoxylin-eosin staining of the pigmented skin revealed heavily pigmented basal and spinous layers and prominent rete ridges. Fontana-Masson stain showed enormous black pigmentations on the basal and spinous layers of the pigmented macule. S-100 protein stain of the pigmented lesion demonstrated an increased number of melanocytes per unit skin area. Ultrastructural studies showed the presence of extensive aggregated melanosomes within the keratinocytes in the epidermis of lentigo, but not the presence of giant melanosomes in either normal skin or lentigines.
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Although the 210 and 190-kDa proteins are the most frequently detected antigens reacting with sera of patients with paraneoplastic pemphigus (PNP) in immunoblot analysis, there is still uncertainty as to the nature of these PNP antigens. To isolate and characterize a cDNA clone encoding the 210-kDa PNP antigen, we screened a human keratinocyte lambda gt 11 cDNA expression library by the immunoperoxidase method with serum IgG from a PNP patient. The IgG used for the immunoscreening of a keratinocyte cDNA expression library recognized 210- and 190-kDa antigens by immunoblotting. A single clone, called here the PNP clone, producing a fusion protein that reacted strongly with the patient's IgG, was further characterized. Only the PNP patient's IgG, but not IgG from a normal control, pemphigus foliaceus, or pemphigus vulgaris patients, bound the plaques of this positive clone. Furthermore, PNP IgG affinity purified on plaques of this clone, but not unrelated clones, bound to keratinocyte cell surfaces by immunofluorescence and reacted with the 210-kDa PNP antigen by immunoblotting. EcoRI digestion of the clone's cDNA insert demonstrated a 1.4-kbp fragment. This cDNA insert was placed into a M13 mp 18 vector and sequenced. Sequence analysis revealed that the cDNA insert of the PNP clone encodes a part of the central rod domain and the COOH-terminal C domain of envoplakin, a newly defined precursor of the cornified envelope that is homologous to desmoplakin. This result demonstrates that the 210-kDa PNP antigen is envoplakin and PNP is an autoimmune disease that produces autoantibodies against intermediate filament-associated proteins in desmosomes and hemidesmosomes, desmoplakin, bullous pemphigoid antigen 1 (BPAG 1), and envoplakin.
Nevoid hypertrichosis is an uncommon congenital disorder consisting of terminal hair growth in a localized distribution on flesh-colored skin; it is not associated with any other systemic abnormalities. A 21-year-old male patient had a circumscribed area of depigmented terminal hairs on his chest which had been present since 1 year of age. At the age of 18, similar lesions developed on his left shoulder and upper arm. The underlying skin was slightly hypopigmented, and no other systemic abnormalities were found. Histologic examination demonstrated terminal hair follicles in otherwise normal skin. The number of HMB 45 positive melanocytes was markedly decreased in the affected skin. This case of multiple nevoid hypertrichosis is reported with a review of previously reported cases.
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Multinucleate cell angiohistiocytoma is a rare benign fibrohistiocytic vascular condition that mainly affects middle-aged women. Grouped reddish-brown papules are found in the extremities. We describe a case of multinucleate cell angiohistiocytoma occurring in a 24-year-old man who presented with multiple papules on the trunk and extremities. This is the first report of generalized multinucleate cell angiohistiocytoma.