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Ichthyosiform sarcoidosis.

Ichthyosiform sarcoidosis is a rare specific cutaneous manifestation of sarcoidosis; it clinically and microscopically appears as acquired ichthyosis. We report a 68-year-old black man with a 10-year history of chronic obstructive pulmonary disease who presented with a 2-month history of acquired ichthyosis. His skin biopsy specimen showed both noncaseating granulomas in the dermis, consistent with sarcoidosis, and ichthyosis vulgaris. Ichthyosiform sarcoidosis is an uncommon presentation of cutaneous sarcoidosis that has been previously described in 19 nonwhite patients whose lesions were located on the legs. The skin lesions appeared either concurrently with or preceded the diagnosis of systemic sarcoidosis in 76% of patients; 95% of the patients eventually developed systemic involvement of their sarcoidosis. The onset of acquired ichthyosis should prompt evaluation for an associated malignancy, connective tissue disease, endocrine abnormality, nutritional deficiency, drug reaction, or sarcoidosis. A skin biopsy specimen consistent with acquired ichthyosis may point to the presence of cutaneous sarcoidosis.

Abdomen↗

[Anthrax: an unusual escharotic lesion].

BACKGROUND: Anthrax is basically an animal disease rarely found in humans. We report a case occurring in a young boy who had minimal signs and a suggestive skin lesion. CASE REPORT: A 16-year-old boy living in the steppe pastures in western Algeria was hospitalized for unexplained long-duration fever. Physical examination and laboratory tests did not suggest any infectious disease among those common in our area: typhoid fever, brucellosis, meningitis. Anthrax was suggested by presence of an escarrotic black plaque measuring 2 to 3 cm localized on the left leg. The lesion was very adherent and surrounded by an inflammatory rim. The diagnosis was confirmed bacteriologically at direct examination and by culture. Pathology found epidermal necrosis without dermal inflammatory infiltration nor vascular thrombosis. Penicillin was effective in 10 days. DISCUSSION: Bacillus anthracis, the causal agent in anthrax, is a Gram positive rod. The disease is generally seen in herbivorous animals contaminated by plant or animal products. Human anthrax is very rare but should not go unrecognized. In humans, skin contamination is the rule, causing first a vesiculopustulous lesion which progresses to an escarrotic plaque with peripheral inflammation localized on exposed areas. In our case, the hidden localization retarded diagnosis.

Adolescent↗

[Pretibial epidermolysis bullosa and hypothyroidism].

BACKGROUND: We report a case of primary non-autoimmune hypothyroidism causing pretibial epidermolysis bullosa. CASE REPORT: A 70-year-old man with primary non-autoimmune hypothyroidism developed blisters of different ages on the lateral aspect of both legs. Pathology reported blisters with subepidermal cleavage. Direct immunofluorescence was negative. Electron microscope examination showed a variable cleavage level and diffuse infiltration of a granulous and amorphous microfibrillar substance. After hormone replacement therapy, euthyroidism was associated with a reduction in the number of bullae and finally complete remission. After 12 months follow-up, the patient has not experienced recurrence. DISCUSSION: Recurrence-free clinical improvement after hormone replacement therapy suggests the diagnosis of hypothyroidism pretibial epidermolysis bullosae. Mochizuki et al. described a similar case which rapidly regressed after hormone therapy but where the electron microscope showed a different cleavage level. These bullae appear to result from a mechanical mechanism due to their localization in areas exposed to friction and also to the presence of bullae of different ages. This hypothesis is confirmed by the presence of a variable level of cleavage and a substance dense to electrons at electron microscopy as well as by the skin weakness. Our case confirms the reality of hypothyroidism pretibial epidermolysis bullosa. Thyroid hormones should be assayed in patients presenting pretibial bullae.

Aged↗

[Linear radiodermatitis following total body electron beam therapy].

BACKGROUND: We describe herein a peculiar clinical presentation of a linear overlapping radiodermatitis, localized on the internal side of the limbs, following a total body electron beam therapy. CASE REPORT: A 68-year-old-man was treated in April 1998 by a total body electron beam therapy, for a stage I mycosis fungoides. Few days after the last irradiation, the patient suffered from a linear eruption localized on the internal side of the limbs and the external side of the abdominal wall. DISCUSSION: Total body electron beam therapy can be proposed in early stage mycosis fungoides with localized or generalized cutaneous lesions. Because penetration depth of electron is well controlled and is limited to the skin, usual side effects of total body electron beam therapy do not concern internal organs (bone marrow,.). We report here a peculiar clinical presentation of a linear radiodermatitis in frontal plane which has not been reported to date, to our knowledge. This radiodermatitis corresponds to overlapping of posterior and anterior fields of irradiation.

Aged↗

Calciphylaxis.

Calciphylaxis is a rare, life-threatening condition of widespread metastatic calcification most commonly seen in the setting of end-stage renal disease. The etiology of calciphylaxis is not well described, though there are several hypotheses. Cutaneous lesions are characteristically found on the abdomen, buttocks, or thighs as reticulated, painful, purple plaques that often undergo ulceration and may serve as a portal of entry for potentially life-threatening infectious agents. Histology reveals medial calcification with intimal proliferation involving small vessels in the subcutaneous fat, associated with a lymphohistiocytic infiltrate of the affected lobules. Treatment, including phosphate binders and parathyroidectomy, is not universally effective. We present one case of calciphylaxis and discuss the clinical features, pathophysiology, histology, and treatment of the condition.

Adult↗

Pyoderma gangrenosum and myelodysplastic syndrome.

Pyoderma gangrenosum (PG) is a painful, often rapidly progressive, ulcerating skin disorder frequently associated with systemic diseases. We report the case of a patient with PG and an anemia. A bone marrow biopsy showed changes consistent with one of the myelodysplastic syndromes, refractory anemia with ringed sideroblasts. Patients with PG and anemia should have bone marrow biopsy if no cause of anemia is readily apparent.

Anemia, Refractory↗

Lichen striatus: clinical features and follow-up in 12 patients.

Lichen striatus (LS) is an uncommon disease of unknown origin characterised by a linear inflammatory papular eruption with spontaneous regression. We here review a series of 12 consecutive cases of LS. Diagnosis was supported by histological examination. Ten of our 12 patients were children aged 6 months to 12 years. The male gender predominated by 9:3. The lower limb was involved more often than the upper limb and trunk. The duration of the disease until regression ranged from 4 months to 4 years (median, 12 months). Postinflammatory hypopigmentation was noted in 5, and hyperpigmentation in 4 patients. Two patients showed nail involvement (onychodystrophy, longitudinal ridging) which appeared simultaneously with the skin lesions and resolved completely. A personal history of atopic disorders was found in 7 of 12 patients. From this series we can confirm that LS mainly affects children. Both skin and nail lesions disappear completely even if they last longer than one year. Compared with other studies, our series showed differences with regard to sex ratio, predilection sites and after effects.

Arm↗