Cases reported "Alopecia"

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1/10. The cutaneous side-effects of hydroxyurea.

    The cutaneous side-effects of long-term hydroxyurea therapy are not widely known and only rarely reported. We report on a patient who developed widespread skin changes, including the recently recognized hydroxyurea dermopathy, during long-term treatment with hydroxyurea for polycythaemia rubra vera. The time course of the clinical changes suggests that they result from direct toxicity of hydroxyurea on the basal layer of the epidermis and mucosal surfaces. We aim to increase clinical awareness of this problem.
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keywords = epidermis
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2/10. Repair of wide coup de sabre without cutaneous excision by means of pericranial-galeal padding flap.

    A case of double linear scleroderma of the forehead (coup de sabre) is described. The histopathology of this rare lesion is now well known with a normal epidermis and a sclerotic dermis. The correction was done with an original two-stage procedure: the lesion with alopecia was first treated by excision-suture and a transfer of the involved subcutaneous tissue along the right inner canthus; 1 year later, by a hemicoronal incision, we transferred a galeal-pericranial flap beneath the wider forehead lesion. We think that the use of a filling flap to correct wide coup de sabre lesions without cutaneous excision can be a simple alternative to the classic treatment by complete excision and flap reconstruction. The subcutaneous fascial system of the scalp can provide a good donor site with minimal morbidity.
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keywords = epidermis
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3/10. Lichen planopilaris.

    P, a 20-year-old laborer displayed initial symptoms of the disease in question when he was 10 years old. Initially he had an asymptomatic progressive loss of hair on the scalp. A couple of years later he had mild to moderate pruritis, and the appearance of slate-blue eruptions on the scalp and elsewhere on the body. This resulted in a complete loss of hair on the vault of the scalp, which led him to seek specialist opinion. skin surface examination revealed the presence of grayish-blue acuminate follicular papules, disposed singly and in groups (plaques). The pilo-sebaceous orifices were conspicuously obliterated and filled by keratin plugs. Perifollicular erythema was a predominant feature on the scalp. The lesions were present over the scalp, around the neck, chest, back, axillae, groin and legs. Shiny atrophied scalp skin depicting scarring alopecia mimicking male-type baldness was a salient feature. In addition, it was studded with conspicuous acuminate papules in its center (Fig. 1a). The known nonhairy (glabrous) skin had classic lichen planus lesions (Fig. 1b). Hemotoxylin-eosin stained microsections prepared from typical lichen planus (LP) lesions over the abdomen and those of lichen planopilaris (LPP) of the scalp were simultaneously studied. The former revealed changes in the epidermis comprising of hyperkeratosis, increase in thickness of stratum granulosum, hydropic degeneration of the basal cell layer and band-like lympho-histiocytic infiltrate pressing against and invading the epidermis, while the latter revealed uniform atrophy of the epidermis and vacuolization of basal cells. The hair follicles were dilated and were filled with keratin plugs. In addition to fibrosis of the dermis, pigment laden microphages and lympho-histiocytic infiltrate was prominent. The follicles and the sebaceous glands were absent. However, arrectores pilorum and sweat glands were preserved (Fig. 2a,b).
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ranking = 3
keywords = epidermis
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4/10. Atrichia and papular lesions: report of a case.

    We report a case of atrichia with papular lesions in a 4-year-old girl. The scalp was completely hairless since birth, except for dark, shiny, coarse hair on the frontoparietal region. eyelashes and eyebrows were sparse. Numerous papular lesions developed on the hairless scalp, cheeks and neck during the second year of life. Teeth and sweating function were normal. The family history was negative. Histologic examination of a papular lesion showed the presence of a keratin-filled cyst in contact with the overlying epidermis. The pathology of the bald scalp showed the presence of tubular epithelial structures devoid of hair bulbs extending from the epidermis to the deep dermis and the superficial hypodermis. Sebaceous and outer root sheath differentiation was evident in most of the tubular structures that also frequently contained small ducts surrounded by two or three layers of flat epithelial cells. The superficial dermis contained horny cysts, similar to those present on the cheeks.
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keywords = epidermis
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5/10. Case study: fibrosing alopecia in a pattern distribution localized on alopecia androgenetica areas and unaffected scalp.

    A 54-year-old man with a 24-year history of androgenetic alopecia was referred to the Department of Dermatological Sciences with follicular inflammatory lesions leading to scleroatrophy in the vertex region (Figure 1) of 1-year duration.These lesions appeared a year ago. There was no previous history of this condition. On examination, the patient showed confluent infiltrative follicular lesions on the frontoparietal and occipital scalp (Figure 2). Some lesions evolved into erosions that developed in ivory white scleroatrophy within weeks. These lesions were localized both in and outside of are as affected by alopecia androgenetica and were associated with mild pruritus. Histopathologic examination, performed on an early lesion of the vertex, documented a mild thinning of follicular epithelium associated with an intense lymphohistiocytic perifollicular infiltrate. The damage of the basal cell layer was limited to the follicle, while epidermis was intact.In particular, follicular keratinocytes under the isthmus showed a very intense degeneration exactly where the infiltrate was the most prominent. The damage of the hair sheath was under the isthmus and involved the lower portions of the follicles (including the hair bulbs). The inflammatory infiltrate was exclusively represented by perifollicular lymphohistiocytes. Finally, a connective fibrotic shell with numerous fibroblasts formed a sheath around the atrophic follicle (Figure 3).Results of laboratory investigations (including complete blood cell counts, basal thyroid-stimulating hormone, c-reactive protein, serum ferritin levels, B and C hepatitis markers, antinuclear antibodies, and cultural examinations) were negative.We diagnosed the patient with fibrosing alopecia in a pattern distribution.
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keywords = epidermis
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6/10. Keratoma hereditaria mutilans (Vohwinkel's disease) with congenital alopecia universalis (atrichia congenita).

    A family is described in which a boy and two girls had features of keratoma hereditaria mutilans (Vohwinkel's disease) and congenital absence of hair. None of them had any hair at birth or developed any subsequently. They had congenital, nonscarring alopecia universalis. The skin over the palms and soles showed progressive thickening, which led to mutilating deformities of the hands and fingers. Histopathological examination of the scalp skin revealed no changes in the epidermis or dermis with normal sebaceous glands and identify hair structures; the skin on the palms showed a tremendously thickened horny layer. Both conditions remained unresponsive to various topical and systemic remedies used in the past. One of the girls had a meningocoele of the dorsolumbar region and died postoperatively. The simultaneous appearance of two rare hereditary diseases in siblings of one family is being reported for the first time.
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keywords = epidermis
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7/10. Tufted hair folliculitis.

    A case of scarring alopecia presenting as two circumscribed, tender and inflamed areas in the occiput with residual tufted follicles is reported. Each tuft comprised 10 to 15 normal-appearing hairs arising from individual hair follicles in the reticular dermis or subcutaneous fat, converging toward a single orifice in the epidermis. The initial pathologic finding was inflammation and scarring of the papillary and mid dermis with almost total sparing of the hair follicles in the subcutaneous fat. Successful treatment was achieved by surgical excision of the two areas. It is suggested that the areas of tufting represented nevoid lesions that underwent inflammation and scarring.
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ranking = 1
keywords = epidermis
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8/10. epidermolysis bullosa acquisita. Direct immunofluorescence and ultrastructural studies.

    A case of epidermolysis bullosa acquisita (EBA), associated with inflammatory bowel disease in which cicatricial alopecia was present, was studied by electron microscopy and direct immunofluorescence microscopy. Direct immunofluorescence studies were performed on both clinically normal and perilesional skin, with and without previous separation of the epidermis from the dermis by incubation with 1 M sodium chloride. We propose the use of this separation technique to identify the level of antibody deposition in patients with EBA in whom circulating antibodies are lacking. This technique may be particularly beneficial in delineating between EBA and the clinically similar scarring localized forms of bullous pemphigoid in which circulating antibodies are often absent.
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keywords = epidermis
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9/10. Iatrogenic benign lymphoplasia induced by allergic contact dermatitis from squaric acid dibutylester: immunohistologic study of cellular infiltrates.

    We report of a 62-year-old male patient with a dull red itchy nodule on the induction area of allergic contact dermatitis to squaric acid dibutylester, which had been used for the therapy of alopecia universalis. The excised biopsy specimen showed dense infiltration of lymphoid cells in the dermis and subcutaneous tissue, associated with the formation of lymphoid follicles. Immunohistologic analysis of the infiltrates indicated mixed proliferation of T- and B-cells. A biopsy specimen from the challenge area showed spongiosis in the epidermis and lymphoid cell infiltration in the upper dermis, while the infiltrates consisted mainly of T-cells. The following points are discussed: (i) the lesion had an iatrogenic origin and the causative agent was quite evident; (ii) the route of allergen application was only through the epidermis and not directly in the dermis; (iii) lymphoid cell infiltrates of the induction and challenge areas were different.
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ranking = 2
keywords = epidermis
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10/10. Cerebellotrigeminal and focal dermal dysplasia: a newly recognized neurocutaneous syndrome.

    A female child with brachycephaly, hypertelorism, convergent strabismus, interstitial keratitis, analgesia on both sides of the face, absent corneal reflexes, and focal congenital alopecia of a zone of the occipital and posterior parietal scalp is presented. The patient also had generalized hypotonia in early life, and at age 4 years 9 months, she was found to be moderately mentally subnormal and to have severe cerebellar deficit consisting of gait and truncal ataxia. There was no clinical evidence of other cranial nerves being affected. It is postulated that the patient has a cerebellotrigeminal and focal dermal dysplasia due to a development arrest of the ectoderm, which gives rise to the alar plate of the rhombencephalon, the overlying epidermis, the motor nucleus of V, and the trigeminal placodes.
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keywords = epidermis
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