M. Pippione

The clinicopathologic spectrum of rhinophyma

We report the results of a clinicopathologic study of 17 patients with rhinophyma in different stages of evolution, with particular attention paid to the severe form of this disease. On the basis of clinical features, we identified 2 groups of patients: the first group (12/17 patients) included patients with the common form of rhinophyma, whereas the second one (5/17 patients) included patients with the severe form of the disease. There was no link between the clinical aspect and the duration of the disease. Microscopic examination of specimens obtained from the classic type of rhinophyma substantially showed the histopathologic features of fully developed rosacea, except for the presence of prominent sebaceous hyperplasia. The second group showed a very different histologic pattern displaying marked dermal thickness, absence of folliculosebaceous structures, sclerotic collagen bundles with large amounts of mucin, and spreading telangiectasia. The inflammatory infiltrate was inconspicuous, with numerous interstitial spindle and bizarre cells. Most of the interstitial cells were reactive to factor XIIIa. The severe form of rhinophyma shares many histologic characteristics with elephantiasis caused by chronic lymphedema.

Interstitial granulomatous dermatitis with plaques

We report on the clinical and histopathologic findings of four patients who had asymptomatic, erythematous to violaceous plaques symmetrically distributed on the upper aspect of the thighs, lateral chest, and in two cases also on the abdomen and flexor surface of the elbows. All of the patients were women; two of them had arthritis, which in one case was associated with an autoimmune disorder, and another had autoimmune thyroiditis. Histopathologically, all cases showed similar changes consisting of an interstitial granulomatous dermatitis involving mostly the lower reticular dermis. Histiocytes were the predominant cellular component, arranged interstitially and in small palisades around foci of degenerated collagen bundles in concert with large numbers of neutrophils and eosinophils. Interstitial granulomatous dermatitis can present different clinical expressions, including linear cords, papules, and, as in our cases, plaques. This peculiar histopathologic pattern falls into the spectrum of cutaneous extravascular necrotizing granuloma, a condition that is often associated with systemic autoimmune disease.

Melanocytic nevi of the breast: a histologic case‐control study

Background:  Melanocytic nevi in the genital, acral, and flexural sites often display clinical and histologic features that may simulate melanoma. We verified whether this is the case also for nevi of the breast.

Agminated Spitz nevi occurring within a congenital speckled lentiginous nevus

A 40-year-old woman had a speckled lentiginous nevus on her thigh since birth. During her first pregnancy, additional papules and nodules appeared within the preexisting hyperpigmented area, histologic examination of which showed features of both junctional and compound Spitzs nevi accompanied by simple lentigolike changes. In this particular case, speckled lentiginous nevus may have constituted a particular environment for the production of multiple Spitz nevi.

Identification of granzyme B-expressing CD-8-positive T cells in lymphocytic inflammatory infiltrate in cutaneous lupus erythematosus and in dermatomyositis

Background.  Lupus erythematosus (LE) and dermatomyositis (DM) are interface dermatitis, histologically characterized by formation of colloid bodies and a CD4+ CD8+ lymphocyte infiltrate. Colloid bodies are examples of intraepidermal apoptosis. Granzyme (Gr)B, synthesized by activated cytotoxic lymphocytes, is a serine protease able to prime apoptosis. GrB+ lymphocytes have been previously found in infiltrates in skin lesions from other types of interface dermatitis.

Angioendotheliomatosis in a woman with rheumatoid arthritis

Reactive angioendotheliomatosis (RA) is a rare self-limited skin condition characterized histopathologically by a proliferation of endothelial cells within vascular lumina, usually as a result of different stimuli such as systemic infections, cryoproteinemias, monoclonal gammopathies, allergic conditions, severe peripheral vascular atherosclerotic disease, and iatrogenic arteriovenous fistulas. We report on a 67-year-old woman with a 20-year history of seropositive rheumatoid arthritis who presented with violaceous swelling of her left forearm. A skin biopsy revealed the histopathologic finding of RA with focal glomeruloid features and deposition of periodic acid-Schiff-positive material. In this systemic disorder, cutaneous manifestations may occur secondary to an immune complex-mediated vasculitic mechanism.

Folliculosebaceous cystic hamartoma with perifollicular mucinosis

An 8-year-old boy had a congenital, solitary verrucous lesion on his ear that histologically showed numerous maloriented infundibulocystic structures from which radiated immature sebaceous lobules as well as hair germs with rudimentary papillae. The surrounding stroma was fibrillary, containing large quantities of mucin. We conclude that perifollicular mucinosis, considered to be specific to Carneys syndrome, can also be observed in solitary folliculosebaceous hamartoma.

Histological features used in the diagnosis of melanoma are frequently found in benign melanocytic naevi

Aims: The histological features used in the diagnosis of melanoma may be present in benign naevi, but quantitative data are not available. The aim of this study was to establish the real prevalence of such features in naevi. Methods: Ten dermatopathologists, from nine Italian institutions, studied a series of naevi. Eleven histological parameters currently used in melanoma diagnosis were analysed: asymmetry, poor circumscription, predominance of single melanocytes, irregular confluent nests, suprabasal melanocytes, hair follicle involvement, absence of maturation, cytological atypia, dermal lymphocytic infiltrate, mitoses, and necrosis. Results: Ninety one naevi were examined: 22 junctional, 59 compound, and 10 intradermal. None of the studied parameters was seen in 22 of the benign naevi studied. One or more investigated features were found in 69 naevi. Poor circumscription was found in 49 cases, single melanocytic predominating in 42, asymmetry in 41, irregular confluent nests in 16, cytological atypia in 14, suprabasal melanocytes in seven, and hair follicle involvement in seven; absence of maturation, mitoses and necrosis were not found. Conclusions: The histological features used for the histological diagnosis of melanoma are often present in benign melanocytic naevi. This suggests a critical, non-mechanical use of them in melanoma diagnosis.

Cutaneous Angiosarcoma Arising in an Irradiated Breast

Angiosarcoma (AS) is a rare, aggressive tumour of endothelial origin occurring in various clinical settings, including idiopathic AS on the head and neck in elderly people, lymphoedema-associated AS, post-irradiation AS, soft-tissue AS, and various others. Despite the widespread use of radiation therapy in the treatment of breast carcinoma, AS developing in the wake of a radiation therapy is extremely infrequent. Although there is little doubt that radiation in therapeutic doses can induce sarcomas, quantification of that risk is complicated by many variables, among them chronic lymphoedema. We describe a 70-year-old woman in generally good health who presented with a 2-year history of a maculo-papular eruption on the skin of her right breast. There was no lymphoedema of the thoracic area. The lesions developed 3 years after she had undergone ipsilateral quadrantectomy for an invasive ductal carcinoma followed by 25 tangent field radiotherapy sessions on the breast. The oncological follow-up did not disclose local recurrence of the tumour or metastases of breast carcinoma. Histopathologic examination of a papule was diagnostic for AS. In addition, signs of chronic radiation dermatitis were found in the biopsy specimens. The patient underwent monthly cycles of chemotherapy with intravenous doxorubicin with partial remission of the affected area after 24 months, followed by the occurrence of liver metastases and exitus 30 months after diagnosis. From the review of the literature, it appears that post-irradiation mammary AS mainly affects women over 60 who have undergone breast-sparing surgery and that it is usually associated with axillary lymphadenectomy. Whereas the role of lymphoedema does not seem relevant to the pathogenesis of this malignancy, the association with chronic radiation dermatitis in our case reinforces the supposed role of radiation in the development of this tumour. Onset of AS should be taken into consideration when treating patients who develop multiple lesions on the skin above the irradiated area, even many years after the therapy.

Immature myeloid precursors in chronic neutrophilic dermatosis associated with myelodysplastic syndrome.

Sweet syndrome (SS) associated with myeloproliferative disorders has been considered an inflammatory process mediated by neutrophils in which immunologic mechanisms are operative. The authors report the case of a 68-year-old man suffering from a myelodysplastic syndrome, who presented with a relapsing skin eruption resembling SS. Histopathologically, the skin infiltrates showed prominent neutrophilic features masking the underlying malignant process. Extensive immunophenotypic studies of skin revealed the presence of a few immature myeloid cells intermingled with an overwhelming infiltrate of neutrophils. The atypical cells in the skin had a phenotype identical to that of leukemic cells in the peripheral blood and bone marrow. Whether or not immature myeloid cell precursors constitute a specific infiltrate of leukemia cutis or are a result of recruitment of circulating leukemic cells to this area of inflammation is discussed.