Immunopathogenesis of folliculitis decalvans: clues in early lesions.

Folliculitis decalvans (FD) is a rare variant of primary cicatricial alopecia, for which the etiopathogenesis remains unclear. Our purpose was to evaluate whether certain immunologic mechanisms might have a significant role in the pathogenesis of FD. Lesional scalp biopsy specimens from 7 patients with FD, 7 with lichen planopilaris, and 4 with alopecia areata were studied immunohistochemically by using monoclonal antibodies to CD1a, CD3, CD4, CD8, CD20, CD25, HLA-DR, interleukin (IL)-1beta, IL-4, IL-8, interferon gamma, tumor necrosis factor alpha, basic fibroblast growth factor (b-FGF), transforming growth factor (TGF)-beta, endothelial leukocyte adhesion molecule 1, intercellular adhesion molecule (ICAM)-1, and vascular cell adhesion molecule. We showed that early FD lesions are characterized by an infiltration of activated T-helper cells, featuring mixed TH1/TH2 polarization. IL-8 and ICAM-1 may contribute to the infiltration of neutrophils, whereas b-FGF and TGF-beta may represent important mediators of the fibrosis that characterizes late-phase FD.

Idiopathic atrophie blanche.

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.

An itching and excoriated dermatosis during intrahepatic cholestasis of pregnancy.

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.

Atopic dermatitis exclusively localized on nipples and areolas.

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.

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.