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1/8. tuberous sclerosis with macrodactyly.

    tuberous sclerosis with macrodactyly is a very uncommon presentation. We report a 15-year-old girl with a thick, loose hyperpigmented area on the dorsum of the left hand with macrodactyly. A skin biopsy specimen from the dorsum of the left hand revealed dense collagenization in the dermis. Radiographs showed marked irregular thickening of the cortex of the metacarpals and phalanges of the left index and middle fingers.
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2/8. Pachydermodactyly: a forme fruste of knuckle pads?

    A 16-year-old boy presented with painless swellings localized to the radial and ulnar aspects of his second through to the fifth fingers on the left hand, with more subtle changes affecting two fingers on the opposite hand. This had developed in the absence of mechanical trauma. Investigations for an arthropathy were negative, while a biopsy showed marked epidermal hyperplasia and an expanded dermis. These features are typical of pachydermodactyly, a benign dermatosis of uncertain aetiology. The interesting feature of this case is the presence of knuckle pads in the father of the patient, which raises the possibility that these two similar entities are related.
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3/8. Disabling pansclerotic morphea: clinical presentation in two adults.

    Disabling pansclerotic morphea involves all layers of the skin, extending through the dermis and subcutaneous tissues to involve muscle, tendon, and bone. It is distinguished from generalized scleroderma by its lack of systemic involvement. Onset usually occurs before the age of 14 years. We describe adult-onset disabling pansclerotic morphea in two previously healthy young men. In both cases, the onset of disease was explosive, with rapid progression, widespread cutaneous involvement, and severe disablement caused by mutilating contracture deformities. Increased susceptibility of sclerodermatous tissue to recalcitrant ulceration and malignant transformation with development of nonmelanoma skin cancers was also observed. Treatment of this disease continues to present a therapeutic dilemma with only sporadic remission despite multimodality therapy.
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4/8. Destructive granuloma annulare of the skin and underlying soft tissues--report of two cases.

    Two cases of extensive cutaneous granuloma annulare (GA) associated with severe deformity and impairment of function in the involved extremity are described. In the first patient a mutilating, destructive arthritic condition and infiltrative oedema of upper extremities developed over the years along with florid disseminated GA of the tendons and overlying skin. In the second patient disseminated GA of one lower extremity resulted in diffuse induration and chronic lymphoedema. In both cases, histology showed palisading granulomas typical of GA at all levels of the dermis, subcutaneous tissues, fascia, and synovium. The unique clinical features of GA in both presented cases emphasize the local destructive potential and, as yet, poorly understood progression of GA to a soft-tissue disease.
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5/8. Surgical correction of the hand in epidermolysis bullosa dystrophica.

    epidermolysis bullosa dystrophica (polydysplastic type) is a rare congenital skin anomaly which, in the hands, because they are exposed to repeated trauma, results in a severe "mitten"-like deformity. Functional benefit was obtained in three patients by separation of the digits and application of split-thickness grafts. Wolfe grafts or "split-off" (epidermis) grafts. arthrodesis of the interphalangeal joints and filleting of the little finger to provide a flap which could be turned in to the palm, resulted in an improvement in hand function.
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6/8. Diabetic scleredema and scleroderma-like changes in a patient with maturity onset type diabetes of young people.

    A 21-year-old housewife with maturity onset type diabetes of young people developed scleredema diabeticorum, scleroderma-like skin thickness on forearms and dorsum of hands, digital sclerosis and cheiroarthropathy. She had diabetes mellitus since the age of 11 years. Her grandfather on the mother's side, her mother and 3 of 5 her mother's brothers and sisters have diabetes mellitus. blood glucose was 295 mg/dl. Urinary glucose was 5.3 g/day. Nail fold capillary microscopy revealed a progressive systemic sclerosis pattern. Histologically, hematoxylin and eosin sections from back and forearm skin demonstrated broad collagen bundles separated by widened clear spaces throughout the thickened dermis.
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7/8. Two cases of pachydermodactyly.

    We report two cases of pachydermodactyly. Case 1 was a 16-year-old girl who complained of asymptomatic, bulbous, firm swellings which developed insidiously on both sides of the proximal interphalangeal (PIP) joint of her right middle finger. Case 2 was a 14-year-old boy with similar lesions on the sides of the PIP joints of the index and middle fingers of both hands. They both had histories of mild, repetitive mechanical trauma of the fingers. Radiologic findings showed soft tissue swellings without any bony or articular abnormalities. Histopathologic findings from the bulbous swellings revealed marked hyperkeratosis, slight epidermal hyperplasia, and a markedly thickened dermis with a deposition of mucinous material among the collagen fibers. Ultrastructural examinations of both cases showed decreased diameters of collagen fibrils. The lesions temporarily improved with intralesional injection of triamcinolone acetonide. Pachydermodactyly is more commonly found in boys and the affected fingers are more numerous in boys. Both of the present cases had the habit of rubbing and gripping their fingers unconsciously. Mechanical trauma of the fingers around puberty may play an important role in pachydermodactyly.
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8/8. Expression of heat shock protein 27 in chromomycosis.

    We report on a 58-year-old woman with long-lasting (36 years) chromomycosis on the foot and secondary self-inoculation from foot to hand 4 years ago. Mycological classification was performed after culture on Sabouraud glucose agar. We used haematoxylin and eosin and Giemsa staining and an antibody to heat shock protein (HSP) 27 (Stress Gen, Clone G3.1) on paraffin-embedded and cryostat specimens of chromomycosis. The mycological culture revealed the fungus Fonsecaea pedosoi. Histopathology revealed dermal fibrosis with persistent fungi (Medlar bodies), numerous mast cells and pseudoepitheliomatous hyperplasia. Immunohistochemically, HSP 27 was positively identified in F. pedrosoi. Moreover, in differentiating keratinocytes in the pseudoepitheliomatous lesions of chromomycosis, HSP 27 was increasingly expressed from basal layers to stratum spinosum in the epidermis but not in keratinocytes directly bordering Medlar bodies. In chromomycosis, HSP 27 is expressed, in accordance with its role as a marker of differentiation and proliferation, in keratinocytes and also in F. pedrosoi. It remains unknown if these results might explain the therapeutic efficacy of hyperthermic treatment.
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