[Trichilemmal horn: a new case and review of the literature]. / Cuerno tricolémico: presentación de un nuevo caso y revisión de la literatura.

Trichilemmal horn, or trichilemmal keratosis, is an uncommon benign neoplasm of follicular lineage with trichilemmal differentiation. The essential characteristics of this tumor are its clinical presentation in the form of a cutaneous horn with trichilemmal keratinization apparent in the histology study (with a hyperplastic epithelium giving rise to dense, orthokeratotic eosinophilic keratin). We present a new case of this type of tumor in an 82-year-old woman who developed a solitary lesion on her scalp after surgical removal of a trichilemmal cyst. On the basis of the pathology report, the tumor was diagnosed as trichilemmal horn. We review the 33 reports of this tumor in the literature to date.

Glomeruloid haemangioma is not always associated with POEMS syndrome.

Glomeruloid haemangioma is considered a specific marker of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome and it is usually but not always associated with multicentric Castleman's disease. We report a 78-year-old man who presented with a single, red-violet soft nodule with superficial telangiectases on the scalp. Histopathologically, the lesion consisted of lobules of coiled aggregated capillaries that involved the lumina of dilated vascular structures, mimicking renal glomeruli. A collagenous stroma separated the capillary lobules, and eosinophilic, periodic-acid-Schiff positive globules of varying sizes and shapes were seen within the cytoplasm of endothelial cells. Immunohistochemical studies with antibodies against IgA and IgG, and against the kappa and lambda light chains of immunoglobulins showed immunoreactivity within the eosinophilic globules. Results of complete blood count, liver, renal and thyroid function tests, fasting blood sugar measurement, serum levels of oestradiol, testosterone, prolactin and cortisol, serum protein electrophoresis, immunoelectrophoresis and immunofixation yielded normal or negative results. No Bence-Jones proteinuria was found in a sample from a 24-h urine collection. To our knowledge, only two cases of glomeruloid haemangioma have been previously reported in patients without POEMS syndrome. We describe the third case of glomeruloid haemangioma in a patient without features of POEMS syndrome.

[Superficial spreading capillary hemangioma: case report]. / Hemangioma capilar de extensión superficial: descripción de un caso.

Superficial spreading capillary hemangioma has been described for the first and sole time by Mihara and collaborators in 1986, as a proliferation of capillaries in the dermal papillae, separated by thin elongated rete ridges. Clinically it appeared as an asymptomatic, isolated, small and long-standing red plaque of slow growth located in the plantar surface of a 57-year-old Japanese woman. We report a 23-year-old Caucasian woman with an asymptomatic, long-standing, brownish plantar lesion of stable size. In the pathological study we observed a capillary proliferation in tangles with slack stroma located in the papillary dermis. We report, therefore, the second case of superficial spreading capillary hemangioma published in the literature.

Epithelioid angiosarcoma of the breast involving the skin: a highly aggressive neoplasm readily mistaken for mammary carcinoma.

BACKGROUND: Angiosarcomas are malignant neoplasms of endothelial cells. Angiosarcoma of the breast is a rare neoplasm that behaves in a highly malignant fashion. It must be differentiated from benign vascular proliferations and from mammary carcinoma. METHODS: We report on a 49-year-old-woman who presented with a large mass involving the left breast. RESULTS: The lesion had an erythematoviolaceous hue and livedoid pattern at the periphery. Histopathologic study showed an epithelioid malignant neoplasm, and immunohistochemical studies demonstrated that neoplastic cells expressed immunoreactivity for endothelial cell markers. CONCLUSIONS: A diagnosis of epithelioid angiosarcoma of the breast was established. The patient was treated with radical mastectomy, but she refused any other additional therapy.

Cutaneous metastases from Ewing's sarcoma: report of two cases.

Ewing's sarcoma is a malignant osseous neoplasm that affects mostly children and young adult males. Clinically, the neoplasm presents with oedema, swelling, and pain of the involved area. Histopathologically, Ewing's sarcoma consists of solid sheets of small round cells, with vesicular nuclei and scant cytoplasm, arranged in irregular masses separated by strands of fibrous tissue, with areas of necrosis en masse intermingled with intratumoural haemorrhage. Ewing's sarcoma is an extremely aggressive neoplasm and metastases to sites such as lung, pleura, other bones, central nervous system, liver, and regional lymph nodes frequently develop in early stages of the disease. Surprisingly, despite the highly aggressive biological behaviour of this neoplasm, cutaneous metastases from Ewing's sarcoma are very uncommon. We report two patients with Ewing's sarcoma of the bone who developed cutaneous metastases. As in other internal malignancies, the onset of cutaneous metastases in patients with Ewing's sarcoma indicates a poor prognosis.

Drug eruption secondary to aciclovir with recall phenomenon in a dermatome previously affected by herpes zoster.

Classically, recall dermatitis refers to chemotherapy-induced reactivation of skin damage caused by radiotherapy months, or even years, earlier. The concept of recall dermatitis has now been extended to include radiation recall dermatitis induced by other drugs, ultraviolet radiation, extravasation of drugs, and allergic contact dermatitis. We now describe recall dermatitis along the residual cutaneous lesions of a previous thoracic herpes zoster in a patient who developed a drug eruption after oral administration of aciclovir. The most striking feature consisted of confluent linear erythema along the dermatomes previously involved by the herpes zoster episode. Histopathologic study demonstrated small foci of spongiosis, vacuolar changes involving the basal layer of the epidermis and single necrotic keratinocytes scattered within the epidermis. The papillary dermis appeared oedematous and with dilated blood capillaries surrounded by a sparse inflammatory infiltrate composed mainly of lymphocytes. Serial sections failed to demonstrate cytologic changes of herpes varicella zoster infection. We interpreted this case as an example of recall dermatitis because the widespread cutaneous eruption secondary to aciclovir was more intense in skin previously compromised by herpes varicella zoster infection. To the best of our knowledge, recall dermatitis has not been described before at the site of previous involvement by herpes zoster.

Olmsted syndrome: report of a case with study of the cellular proliferation in keratoderma.

Olmsted syndrome is a rare disorder that consists of sharply marginated keratoderma of the palms and soles, constriction of digits and toes that may result in spontaneous amputation of the distal phalanges, hyperkeratotic plaques around the body orifices, onychodystrophy, and other less common cutaneous and extracutaneous anomalies. Although some patients had other affected family members, most cases of Olmsted syndrome seem to be of sporadic occurrence. We describe a patient with the characteristic features of Olmsted syndrome. The symptoms consisted of diffuse transgrediens palmoplantar keratoderma and keratotic plaques around the mouth and nose. Our patient also had the associated anomalies of hyperhidrosis of the palms and soles and congenital deaf-mutism. Histopathologic study of the keratoderma demonstrated epidermal hyperplasia with acanthosis, papillomatosis, and orthokeratotic hyperkeratosis. Immunohistochemical study showed more basal and suprabasal keratinocytes of the epidermis with immunoreactivity for Ki-67 marker when compared with the keratinocytes of the epidermis of the adjacent non-involved skin. These results support the notion that Olmsted syndrome is a hyperproliferative disorder of the epidermis.

Multiple hereditary infundibulocystic basal cell carcinomas: a genodermatosis different from nevoid basal cell carcinoma syndrome.

BACKGROUND: Infundibulocystic basal cell carcinoma is a recently described distinctive clinicopathologic variant of basal cell carcinoma. Histopathologic differential diagnosis among infundibulocystic basal cell carcinoma, trichoepithelioma, and basaloid follicular hamartoma has generated controversy in the literature. OBSERVATIONS: Members of 2 families with multiple infundibulocystic basal cell carcinomas are described. Each patient showed multiple papular lesions, mostly located on the face. No patient showed palmar pits or jaw cysts. Forty-two cutaneous lesions from 5 patients were studied histopathologically. Thirty-nine lesions were infundibulocystic basal cell carcinomas. This clinicopathologic variant of basal cell carcinoma consists of a relatively well-circumscribed basaloid neoplasm composed of buds and cords of neoplastic cells arranged in anastomosing fashion and with scant stroma. Some of the neoplastic cords contain tiny infundibular cysts filled by cornified cells with abundant melanin. Linkage analysis in family 2 was performed using polymorphic markers (D9S196, D9S280, D9S287, and D9S180), and the affected members shared the same haplotype. Loss of heterozygosity analysis was performed in 2 affected members of this family from whom tumoral DNA was available, and although these individuals were constitutively heterozygous for D9S196, they did not show loss of heterozygosity for this marker in their neoplasms. CONCLUSIONS: Multiple hereditary infundibulocystic basal cell carcinomas represent a distinctive genodermatosis different from multiple hereditary trichoepitheliomas and nevoid basal cell carcinoma syndrome. We propose clinical and histopathologic criteria to distinguish infundibulocystic basal cell carcinoma from trichoepithelioma, basaloid follicular hamartoma, and folliculocentric basaloid proliferation.

Papular-purpuric "gloves and socks" syndrome: polymerase chain reaction demonstration of parvovirus B19 DNA in cutaneous lesions and sera.

We report a typical case of papular-purpuric "gloves and socks" syndrome (PPGSS) in which primary infection by parvovirus B19 was demonstrated by seroconversion to this virus; parvovirus B19 DNA was also identified by polymerase chain reaction (PCR) methods in the sera of the patient and in the cutaneous biopsy specimen, both taken 4 days after the onset of clinical manifestations. To our knowledge, this is the fourth published case in which parvovirus B19 DNA has been recovered from the skin by PCR. Serologic studies and PCR investigations in cutaneous biopsy for other viruses including herpes simplex virus types 1 and 2, varicella zoster virus, Epstein-Barr virus, cytomegalovirus, and human herpesvirus 6, 7, and 8 were negative. Clinically, our case presented some additional features, which have not been previously described in cases of PPGSS, namely dysuria with vulvar edema and erythema, and unilateral petechial rash on the breast. The histopathologic findings of our case were nonspecific and consisted of an interface dermatitis with slight vacuolar degeneration at the dermoepidermal junction and a superficial perivascular inflammatory infiltrate mostly composed of lymphocytes, with numerous extravasated erythrocytes. We review the cases of PPGSS published in the literature with respect to the different viruses that have been proposed as etiologic agents and conclude that acute infection by parvovirus B19 is the only one that has been adequately proved.

Sebaceous carcinoma of the vulva.

Extraocular sebaceous carcinoma is an uncommon neoplasm usually localized on the head and neck. Sebaceous glands are abundant on the vulva, but vulvar sebaceous carcinoma is an uncommon neoplasm. To our knowledge, there are only five previously reported cases of sebaceous carcinoma on this location. We report an additional case of vulvar sebaceous carcinoma associated with Bowen's disease in the overlying epidermis. The patient also had bowenoid papulosis involving the skin of labia majora. We analyzed by immunohistochemistry, Southern blot hybridization, and polymerase chain reaction (PCR) techniques for the presence of DNA of human papilloma viruses (HPVs) in the specimen of sebaceous carcinoma and in lesions of bowenoid papulosis. Immunohistochemistry, Southern blot hybridization, and PCR studies in specimens of bowenoid papulosis lesions and sebaceous carcinoma did not detect DNA of HPVs. A significant increase in intranuclear p53 staining was demonstrated in several areas of neoplastic aggregations of sebaceous carcinoma.

Panniculitis mimicking lupus erythematosus profundus: a new histopathologic finding in malignant atrophic papulosis (Degos disease).

We present the case of a 57-year-old woman with a 1-year history of a cutaneous eruption clinically and histopathologically characteristic of Degos disease. In one of the two cutaneous biopsy specimens taken from the trunk lesions, the histopathology consisted of necrosis and sclerosis of the subcutaneous lobules, a finding that to our knowledge has not been previously described in the cutaneous lesions of Degos disease. Two months after cutaneous biopsies were taken, the patient developed ptosis and an episode of acute abdominal pain; intestinal perforation and many characteristic lesions of visceral Degos disease involving the entire small bowel were noted at laparotomy.

Intravascular and diffuse dermal reactive angioendotheliomatosis secondary to iatrogenic arteriovenous fistulas.

Reactive angioendotheliomatosis is a rare benign process that has been mainly described in patients with systemic infections, such as subacute bacterial endocarditis or tuberculosis, and in association with intravascular deposition of cryoproteins. Histopathologically, it is characterized by a proliferation of endothelial cells within vascular lumina resulting in the obliteration of the involved vessels. Another rare variant of reactive angioendotheliomatosis has been described in the lower extremities of patients with severe peripheral vascular atherosclerotic disease. It consists of violaceous and purpuric plaques histopathologically characterized by diffuse proliferation of endothelial cells interstitially arranged between collagen bundles of the reticular dermis. This second variant has been named diffuse dermal reactive angioendotheliomatosis. We report two patients with reactive cutaneous angioendotheliomatosis appearing distally to arteriovenous fistulas used for hemodialysis because of chronic renal failure. The first patient showed intravascular reactive angioendotheliomatosis, while the second one had purpuric plaques that were characterized histopathologically by diffuse dermal angioendotheliomatosis. Both patients showed an arteriovenous "steal" syndrome with distal ischemia, and it is possible that a local increase of vascular endothelial growth factor, as is the case in hypoxia situations, induces the endothelial proliferation. To the best of our knowledge, cutaneous reactive angioendotheliomatosis has not been previously described in association with arteriovenous shunts.

Pyodermatitis-pyostomatitis vegetans: report of a case and review of the literature.

Pyodermatitis-pyostomatitis vegetans is a benign, rare disorder characterized by a pustular eruption in the oral mucosa and vegetating plaques involving the groin and axillary folds. Its association with inflammatory bowel disease is well established. We report the case of a 49-year-old-white man with ulcerative colitis who manifested a vegetating, annular plaque in the left inguinal region of 2 months' duration. Oral examination disclosed an erythematous mucosa with multiple painful pustules involving the labial and gingival mucosa. Histopathologic study demonstrated epidermal hyperplasia and an inflammatory infiltrate composed mostly of neutrophils and eosinophils, grouped into microabscesses within the epidermis and with a bandlike configuration in the upper dermis. Results of direct and indirect immunofluorescence studies were negative. We discuss the differential diagnosis between pyodermatitis-pyostomatitis vegetans and pemphigus vegetans.

Histopathology of cutaneous reaction to granulocyte colony-stimulating factor: another pseudomalignancy.

Granulocyte colony-stimulating factor (G-CSF) is a hematopoietic growth factor (HGF) with many applications in cancer therapy. The most important applications are reduction in the incidence of febrile neutropenia, acceleration of neutrophil recovery after chemotherapy or bone marrow transplantation, and mobilization of progenitor cells. Many cutaneous adverse reactions associated with HGF have been reported in recent years, including injection site reactions, pyoderma gangrenosum, Sweet's syndrome, cutaneous leucocytoclastic vasculitis, and widespread folliculitis. The presence of large histiocytes on the dermis between collagen bundles has been proposed as a characteristic histopathologic finding in cutaneous eruptions secondary to granulocyte colony-stimulating factor and granulocyte-macrophage colony-stimulating factor. We report on a patient with a high-risk ductal infiltrating carcinoma of the breast who received high-dose chemotherapy (HDC) with peripheral blood progenitor cell (PBPC) rescue. The patient received G-CSF after PBPC for a faster granulocyte recovery. She developed a cutaneous eruption located on back, buttocks, axillae, groin and sites where electrocardiography electrodes had been placed. From the histopathological point of view, the eruption was characterized by the presence of numerous large, atypical histiocytes in the dermis with several mitotic figures, mimicking involvement of the dermis by a malignant process.

Acquired hypertrichosis lanuginosa: case report and review of the literature.

Acquired hypertrichosis lanuginosa is a rare cutaneous disorder usually associated with internal malignancy that consists of the development of abnormal hair growth of the lanugo type, often confined to the skin of the face and neck, although other areas also may be involved. We report on a 66-year-old woman with a metastatic ductal infiltrating carcinoma of the breast who developed growth of fine lanugo type hair on her face and progressive growth of the hair of eyebrows and eyelashes. We review the literature on this uncommon paraneoplastic cutaneous disorder emphasizing the pathogenic mechanisms that have been proposed to explain the striking overgrowth of lanugo type hair.

Pigmented and nested sebomatricoma or seborrheic keratosis with sebaceous differentiation?

Recently, the term sebomatricoma has been proposed as the most appropriate denomination to avoid controversial and confusing terms and to encompass all benign neoplasms with sebaceous differentiation. We report on two specimens that may be interpreted as examples of two new histopathologic patterns in sebomatricoma, namely, pigmented and nested sebomatricoma, or as seborrheic keratosis with sebaceous differentiation. The first case consisted of a neoplasm composed of basaloid immature cells with features of sebaceous differentiation in the form of clusters of sebocytes and sebaceous ducts, in addition to the proliferation of dendritic melanocytes arranged as solitary units scattered through the neoplastic aggregations of immature germinative sebaceous cells. The second case, in addition to the proliferation of single dendritic melanocytes as the first case, showed well-defined nests of basaloid and heavily pigmented germinative sebaceous cells connected to the skin surface and numerous sebaceous ducts. In the first case, the pigmented nature of the lesion was the most striking histopathologic characteristic, whereas the nested pattern was remarkable in the second one. Immunohistochemical investigations demonstrated positivity of cytokeratin MNF116 in neoplastic aggregations of germinative sebaceous cells, whereas the clusters of mature sebocytes and sebaceous ducts expressed epithelial membrane antigen positivity. S-100 protein made evident the scattered dendritic melanocytes, but neoplastic cells resulted negative.

Spinulosis of the face as a manifestation of demodicidosis.

We describe a 78-year-old woman with polycythaemia rubra vera who had multiple tiny follicular hyperkeratotic spicules on the cheeks. She was receiving treatment with oral hydroxyurea, but no topical agents had been applied to her face. Histopathological study demonstrated numerous Demodex folliculorum mites within dilated follicular infundibula, and we consider that the mites were playing a part in the aetiology of the skin lesions.

Cutaneous manifestations of infection by nontuberculous mycobacteria.

Cutaneous infections by nontuberculous mycobacteria (NTM) are not usual but their relative importance has changed during the last few years and still further changes are expected. This study comprised 13 patients from whom NTM were recovered from skin biopsy specimens, sinus exudates or cutaneous abscesses. All samples were processed according to standard methods, and the isolates were identified by biochemical testing. Skin biopsy specimens, when available, were processed for histopathological study. The clinical records of the patients were reviewed, and the relevant clinical, microbiological and epidemiological data collected. The clinical manifestations were noted to be relatively nonspecific and consisted of draining sinuses, abscesses, ulcers and nodules with multicentric or sporotrichoid patterns. Tissue culture isolated Mycobacterium fortuitum complex in nine patients, M. avium in three, and M. marinum in one. In the nine patients studied by histopathology, various patterns were observed. These included dermo-hypodermal abscesses, suppurative granulomas, tuberculoid granulomas and granulomas with a perifollicular distribution. Cutaneous lesions can thus be the first and the only sign of NTM disease, and culture still remains the definitive diagnostic procedure.

Clear-cell porocarcinoma: another cutaneous marker of diabetes mellitus.

The relationship between clear-cell syringoma and diabetes mellitus is well established. We present a case of clear-cell porocarcinoma in a diabetic patient. The lesion consisted of a 5-cm nodule on the lateral aspect of the left leg. Histopathologically, the neoplasm was composed of irregular aggregations of neoplastic cells with striking clear-cell appearance, showing features of ductal differentiation. The clear-cell appearance of neoplastic cells was due to glycogen accumulation within their cytoplasm. Immunohistochemistry and ultrastructural studies also supported the diagnosis of a neoplasm with sweat ductal differentiation. Enzyme histochemical reactions for phosphorylase immunoreactivity on fresh, unfixed sections of the neoplasm demonstrated that this immunoreactivity was remarkably decreased. Some adnexal neoplasms of the skin mostly composed of clear cells may be cutaneous markers of diabetes mellitus. Phosphorylase activity deficiency in diabetic patients may be responsible for glycogen accumulation in neoplastic cells resulting in clear-cell appearance of these neoplasms.