Relevance of cellular infiltrate and cytokines in polymorphic eruption of pregnancy (PEP).
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Biomedical subjects
Publications and source records attributed to Samantha Berti.
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We report a 7-year-old girl who presented with erythematous-infiltrated, figurate, well-defined lesions over sun-exposed skin and antinuclear and Ro/SSA antibodies. Lupus band test revealed granular IgM and microgranular C3 deposits at the dermoepidermal junction. Histopathologic examination of lesional skin showed orthohyperkeratosis, epidermal atrophy, widespread hydropic degeneration of the epidermal basal cell layer, and a dermal perivascular lymphohistiocytic infiltrate. The clinical, immunopathologic, and histopathologic findings were consistent with the diagnosis of annular-polycyclic variant of subacute cutaneous lupus erythematosus (SCLE). Treatment with hydroxychloroquine cleared the cutaneous lesions. We report this patient because SCLE is extremely rare in childhood, and discuss the two previous cases reported in the literature.
We describe a 5-year-old boy with red-pink, firm, nodular lesions, with central resolution and prominent borders, localized to the face, backs of the hands, extensor surfaces of the arms and legs, and the buttocks. He also had recurrent bacterial respiratory infections. Quantitative immunoglobulin levels revealed hypogammaglobulinemia and increased IgM levels. Histopathologic examination of a nodular lesion revealed perivascular and periadnexal granulomas composed of epithelioid cells surrounded by a mantle of lymphocytes; three cultures for fungi and acid-fast bacilli were negative. Clinical, histopathologic, and immunologic studies supported a diagnosis of hyper-IgM (HIM) syndrome. The boy was treated with pulsed-dose antibiotics and intravenous gamma globulin every 3 weeks, with improvement of clinical symptoms. Skin lesions were treated with topical corticosteroids, with immediate recurrence upon cessation of therapy. To the best of our knowledge, this is the first description of cutaneous granulomas in HIM syndrome.
We present an 11-year-old girl, with celiac disease, and a 9- month history of itchy and erythemato-edematous lesions with vesicles and exudation on her nipples and areolas. No other lesions or signs of scratching were present on her face or folds. She had no specific lesions of atopic dermatitis in typical sites nor in other body surface during her life. Patch tests showed a positive reaction to nickel and thimerosal that was not significantly related to the clinical appearance. This presentation documents the clinical relevance of atopic dermatitis minor diagnostic criteria. We discuss the importance of nipple eczema in AD and its differential diagnosis.
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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.
A 35-year-old woman at 30th gestation week of her second pregnancy presented to our department with a 2-month history of intense and generalized pruritus. She had a spontaneous abortion 1 year earlier. Itching initially presented during nighttime and localized on lower limbs and after became continuous, diffuse, and associated with excoriations due to scratching. The patient was previously treated with oral corticosteroids (25 mg/d) in a gynecological department with temporary response. On our examination, she presented linear excoriations with hemorrhagic crusts localized on the trunk, buttocks, and upper and lower limbs. Biopsy specimen from the lesional area of the right buttock submitted for routine histology documented a mild perivascular and interstitial infiltrate of lymphocytes and monocytes with rare eosinophils on superficial dermis. Indirect and direct immunofluorescence (performed on perilesional skin) were negative. Laboratory investigations revealed microcytic anemia (hemoglobin 7.5 g/dL; medium corpuscular volume 61.7 fl), erythrocyte sedimentation rate (21 mm) and serum bile acid levels (18.3 nmol/L; normal values 1.00-8.90) increase. On the basis of clinical, serological, and histological findings, we diagnosed an itching dermatosis during an intrahepatic cholestasis of pregnancy. We treated the patient with ursodeoxycholic acid (600 mg) and topical corticosteroids with gradual resolution of itching. Furthermore, she delivered a healthy boy at 39th gestation week with normal birth weight and normal Apgar score.
A 41-year-old woman presented with a 3-year history of purpuric lesions followed by superficial, painful ulcers and development of lesions on the lower legs and on the dorsa of the feet, particularly in the summer. The patient was asymptomatic during the winter months. On physical examination she had irregular, scleroatrophic, white-ivory, coalescent lesions on a livedoid basis, with purpuric and, in some lesions, pigmented borders with numerous telangiectatic capillaries. These lesions were localized on the medial sides of the lower legs and on the dorsa of the feet (Figure 1). Laboratory investigations were normal or negative, including complete blood cell count, platelets, coagulation indexes, erythrocyte sedimentation rate, serum immunoglobulins, antinuclear antibodies, anti-double-stranded DNA, anticardiolipin, antiphospholipids, antineutrophilic cytoplasmic antibodies, circulating immunocomplexes, complement fractions (C3, C4), cryoglobulins, rheumatoid factor, and Rose-Waaler reaction. The only laboratory abnormality was an elevated fibrinogen level (472 mg/dL). Doppler velocimetry excluded a chronic venous insufficiency. Thoracic x-ray and abdominal ultrasound were normal. A digital photoplethysmograph revealed functional Raynaud's phenomenon. A biopsy specimen taken from a purpuric lesion showed an atrophic epidermis with parakeratosis and focal spongiosis. An increased number of small-sized vessels were observed within a sclerotic dermis. Most of the vessels in the upper dermis were dilated and showed endothelial swelling; some were occluded due to amorphous hyaline microthrombi (Figure 2). There were fibrinoid deposits around the vessels with thickening of the vessel walls. Extravasated erythrocytes were found throughout the upper and mid-dermis. There was a sparse perivascular lymphocytic infiltrate but no vasculitis. Direct immunofluorescence showed a perivascular microgranular deposit of IgM (+), C3 (++), and fibrinogen/fibrin (+++). On the basis of clinical, serologic, histopathologic, and immunopathologic findings, a diagnosis of idiopathic atrophie blanche was made. The patient was treated with dapsone (50 mg p.o. q.d.) and pentoxifylline (400 mg p.o. t.i.d.) with pain relief and complete resolution of the ulcerations after 6 weeks of therapy.