A case of a 21-year old female with Maffucci's syndrome is presented where the patient developed multiple cutaneous spindle-cell hemangioendotheliomas. She had multiple pea to walnut sized, non-tender, normal skin colored or slight bluish nodules and bony mass-like lesions on the left upper extremity and hand. A histological examination of the lesions showed that they were composed of irregularly dilated, thin walled cavernous blood spaces containing phleboliths and collapsed vascular spaces separated by spindled fibroblastic cells. Radiologically, the bony lesions showed radiolucent densities with calcified spots within the second proximal phalanx and metacarpal bone of the left hand, which were consistent with enchondromas.
Chondroma ; Female ; Fibroblasts ; Hand ; Hemangioendothelioma* ; Humans ; Juglans ; Peas ; Skin Pigmentation ; Upper Extremity

Cutis marmorata telangiectatica congenita(CMTC) is a rare congenital cutaneous vascular anomaly. The major skin findings are persistent, fixed cutis marmorata, telangiectasia, and phlebectasia. In approximately 50 % of patients, the disorder tends to be associated with various con-genital anomalies. We describe in this report a 4-month-old male infant with CMTC. The patient had red or violet marbled patches, telangiectases, and atrophy on the right arm present at birth. During the follow up period of 12 months, there was no change in the cutis marmorata pattern and telangiectases, whereas the atrophy has been less prominent than at the initial visit.
Arm ; Atrophy ; Follow-Up Studies ; Humans ; Infant ; Male ; Parturition ; Skin ; Telangiectasis ; Viola

BACKGROUND: The keratinocyte growth factor(KGF) is a recently identified mitogen for epithelial cells produced by nomal stromal fibroblasts. ln the skin, KGF has been shown to stimulate keratinocyte proliferation and differentiatian. Dendritic epidermal T cells(DETC) are skin-specific members of the epithelial y 8 T-cell family that reside normally in the murine epidermis. The DETCs recognize antigen expressed by damaged or diseased neighboring keratinoctyes and consequently secrete cytokines sueh as IFN- y, lL-2, IL-4, GM-CSF. OBJECTIVE: The purpose of this study was to observe the expression of KGF mRNA in keratinocyte and DETC as well as to investigate the cytokine-mediated intercellular communication between kerati- nocyte and DETC. METHODS: Using a RT-PCR(reverse transcription-polymerase chain reaction), we examined the expression of KGF mRNA in keratinocyte and DETC, and compared the level of KGF mRNA between resting and activated DETC with Con-A (concanavalin A).
Cytokines ; Epidermis ; Epithelial Cells ; Fibroblast Growth Factor 7* ; Fibroblasts ; Granulocyte-Macrophage Colony-Stimulating Factor ; Humans ; Interleukin-4 ; Keratinocytes* ; RNA, Messenger* ; Skin ; T-Lymphocytes

Twenty-nail dystrophy is an idiopathic nail dystrophy in which all twenty nails are uniformly and simultaneously affected with excess longitudinal ridging and loss of lustre. The pathogenesis is controversial, and the treatment is unsuccessful. It is thought to have a self-limiting and reversible nature when it develops in childhood, but in adults, it is unusual and exists persistently. We re-port here two cases of adult patients with dystrophy of all twenty nails, whose ages were 58 and 55. The disease had been present for one year and may be associated with alopecia areata in the 58 year old and an idiopathic condition in the 55 year old. Negative results were obtained on mycological studies. Biopsies taken from the nail bed revealed marked hyperkeratosis consistent with nail dystrophy.
Adult ; Alopecia ; Alopecia Areata ; Biopsy ; Humans

No abstract available.
Child* ; Humans ; Tuberculosis, Renal*

No abstract available.
Aspirin* ; Epithelial Cells*

We report a case of infantile digital fibromatosis in a 34 month-old boy, who presented with a painless subcutaneous tumor on the medial aspect of the left third toe. A histological examination showed scattered small, round eosinophilic inclusion bodies in the cytoplasm of the tumor cells, which was consistent with infantile digital fibromatosis. A'immunohistochemical study revealed that desmin, a-smooth muscle actin, and vimentin were clearly positive in the cytoplasm of the tumor cells, but the inclusions themselves showed negative staining, thus indicating a hollow-like staining pattern. Electron microscopy showed either well-defined or ill-demarcated dense bodies in the cytoplasm of the tumor cells. In some areas, small vesicles and intracellular organells were observed' in the inclusions. In the course of conservative treatment, a new lesion developed on the lateral aspect of the left third toe, seven months after the appearance of the initial lesion.
Actins ; Cytoplasm ; Desmin ; Eosinophils ; Fibroma* ; Humans ; Inclusion Bodies ; Male ; Microscopy, Electron ; Negative Staining ; Toes ; Vimentin

We report a case of porokeratosis of Mibelli coexistence with linear porokeratosis in a 28-year old male. He had multiple discrete reddish to brownish annular plaques with peripheral keratotic ridges on the left side of the neck and axilla, and such lesions extended in a linear fashion on the left side of the upper arm and posterior aspect of the lower extremity. The cutaneous lesions started on the left neck and axilla about 6 years prior to consultation. Two years after that, new lesions developed on the left side of the upper arm and lower extremity in a linear fashion. There was no family history of similar skin disorders. A histological examination showed cornoid lamellae in the horny layer. Hypogranulosis, dyskeratotic cells and perivascular mononuclear in-filtrations were also seen beneath the cornoid lamella.
Arm ; Axilla ; Humans ; Lower Extremity ; Male ; Neck ; Porokeratosis* ; Skin

No abstract available.
Aneurysm*

No abstract available.
Follow-Up Studies* ; Prostheses and Implants*