Félix Contreras

Pilomatrix carcinoma: a clinicopathologic study of six cases and review of the literature.

Pilomatrix carcinoma, the malignant variant of pilomatrixoma, is a rare entity. The authors report on six patients with pilomatrix carcinoma and review the pertinent literature. The lesions showed a predilection for elderly individuals (mean age, 61 years) with a male:female ratio of 5:1, and they presented as dermal or subcutaneous tumors located on the head and neck (5 neoplasms) and chest (1 neoplasm). Tumors varied in size from 0.6 cm to 2.5 cm (mean, 1.78 cm). None of the lesions recurred after wide local excision. On scanning magnification, all tumors showed the architectural features of a malignant neoplasm (asymmetry and poor circumscription, presence of several markedly sized and variably shaped basaloid aggregations, and ulceration). The tumors were composed of pleomorphic basaloid cells with prominent nucleoli and frequent atypical mitoses accompanied by central areas with keratotic material, shadow cells, and foci of necrosis. The tumor nests were surrounded by a desmoplastic stroma and infiltrated the adjacent tissues. Vascular or perineural infiltration was not observed. In one case, the basaloid cells contained abundant melanin pigment in their cytoplasms. Pilomatrix carcinoma is a neoplasm of low-grade malignancy that should be distinguished from the conventional pilomatrixoma and its variants (aggressive pilomatrixoma and proliferating pilomatrixoma), matricoma, and basal cell carcinoma with matrical differentiation. Clinicians and pathologists should be aware of the occurrence of pilomatrix carcinoma because of its potential for distant metastases.

High incidence of porokeratosis in renal transplant recipients

Immunosuppression is a well–documented precipitant of porokeratosis (PK). However, PK is not considered among the most common cutaneous disorders in immunosuppressed patients. We studied prospectively a series of 103 renal transplant patients and found 11 cases (10.68%) of PK. Our series represents the highest incidence of PK in transplant patients reported so far. Our findings suggest that PK in transplant recipients may be more frequent than previously thought.

Multiple self-healing indeterminate cell lesions of the skin in an adult

We report a case of multiple cutaneous indeterminate cell proliferative lesions in an adult without any other organ involvement. All lesions regressed spontaneously over 5 years without recurrence over a 4-year period of follow-up. The electron-microscopic and immunohistochemical features of the cellular infiltrate were those of the indeterminate cells. This case supports the hypothesis that indeterminate cell proliferative disorder is a wide spectrum of conditions with variegated clinicopathologic presentation and with different biological behavior.

Syphilis in an HIV infected patient misdiagnosed as leprosy

A 42‐year‐old man with uveitis and a widespread cutaneous eruption, histopathologically characterized by dermal granulomatous infiltrates with perineurial invasion, was incorrectly diagnosed and treated as having borderline leprosy. Further studies demonstrated secondary syphilis and human immunodeficiency virus (HIV) inlection. Standard penicillin therapy resolved his cutaneous and ocular lesions. Reports on clinical and pathological findings of active syphilis in HIV infected patients are scarce but this case and isolated previous case reports suggest that granulomatous infiltrates might be a common feature in secondary syphilis with short evolution in HIV infected palients.

Neonatal Lupus Erythematosus with Multisystem Organ Involvement Preceding Cutaneous Lesions

Here we report a case of neonatal lupus erythematosus syndrome presenting with multisystem organ involvement, including anemia, thrombocytopenia, purpura, bloody diarrhea, enzymatic liver abnormalities, splenomegaly and pneumonitis. These findings preceded the cutaneous rash that was the clue for the diagnosis. The patients mother had an undiagnosed subacute cutaneous lupus erythematosus. The various forms of onset of neonatal lupus erythematosus syndrome are emphasized.

Two Cases of Segmental Multiple Glomangiomas in a Family: Type 1 or Type 2 Segmental Manifestation?

Several autosomal dominant skin diseases may manifest cutaneous mosaicism. Two types of segmental arrangement can be distinguished: type 1 is characterized by segmental lesions with similar severity to that observed in the diffuse phenotype, the remaining skin being normal; type 2 is characterized by segmental lesions showing a major degree of severity and milder lesions diffusely arranged. Multiple glomus tumours have recently been included in the group of genodermatoses showing type 2 segmental involvement. A family with 2 cases of multiple glomangiomas arranged in a segmental fashion is reported. A 12-year-old girl presented multiple nodular glomangiomas on her right buttock and thigh, in a band-like distribution. A sister of her paternal grandfather showed plaque-like multiple glomangiomas on her left thigh and various glomangiomas on her right buttock and arm. No other family members were known to be affected. Two new cases of familial segmental multiple glomangiomas are reported, with the particularity that one of these exhibited type 2 segmental manifestation and the other type 1 from a clinical point of view.

Multibacillary leprosy: lesions with macrophages positive for S100 protein and dendritic cells positive for Factor 13a

In the defense against Mycobacterium leprae, macrophages play an essential part in the mechanism of bacterial lysis but require the presence of cytokines such as interleukin 2 and gamma interferon from lymphocytes in order to effectively kill the organisms in any number. While there have been many studies of the lymphocytes in lesions of leprosy, less attention has been given to the immunohistochemical characterization of the macrophage populations. In this study, the cutaneous lesions of 69 patients with leprosy (42 lepromatous, 5 mid‐borderline, and 22 tuberculoid) were evaluated by immunohistochemistry for the expression of S100 protein, CDla, CD68, muramidase, HLA‐DR, and Factor 13a. The macrophages from lesions of polar, subpolar, and borderline Iepromatous leprosy patients expressed S100 protein intensely and constantly. In contrast, the lesions of polar and subpolar tuberculoid leprosy had very few cells that were immunoreactive for S100 protein (‘S1000+’) in the granulomas in the dermis. The macrophages in all lesions were reactive for CD68 and muramidase. In paraffin sections, macrophages of lepromatous lesions failed to stain for HLA‐DR, whereas in tuberculoid lesions, they were strongly positive for HLA‐DR. Three patients with histoid leprosy (relapse lesions) had lesions that were strongly positive for Factor 13a and were negative for S100 protein (‘S100−’). Given the possible chemotactic and migration inhibition effects of the calcium‐binding proteins of the S100 family, these data suggest a possibly important role for S100 protein in the accumulation of macrophages in lepromatous leprosy, and also reveal infection of Factor 13a+ dermal dendritic cells in histoid leprosy.

Verrucous carcinoma of the skin associated with syringadenoma papilliferum: a case report

We report the case of a 67‐year‐old man with a granular, cauliflower‐pink lesion on the skin of the thigh. Histopathological study showed a typical verrucous carcinoma associated with a syringadenoma papilliferum. To our knowledge this association has not been previously reported.

p53 protein expression in viral warts from patients with epidermodysplasia verruciformis

Summary The p53 protein is the product of a tumour suppressor gene, which is implicated in many human malignancies. p53 expression was investigated by immunohistochemistry in a series of viral warts (n = 12) from five patients with epidermodysplasia verruciformis (EV), using a monoclonal anti‐p53 antibody (D07). p53 expression was also investigated in a series of common warts (n = 8), flat warts (n= 8), and penile bowenoid papulosis (n = 6) from non‐EV patients. Immunostaining was positive in 11 of 12 (92%) EV warts, whereas p53 reactivity was negative in most cases of warts from non‐EV patients. Exons 5–8 of the p53 gene were screened by the polymerase chain reaction‐single strand conformation polymorphism technique in four EV warts, which were strongly stained for p53, and p53 mutations were not detected. These results suggest an association between p53 accumulation (probably of wild type) and EV warts.

Enfermedad de Fox-Fordyce

Fox-Fordyce disease is an infrequent inflammatory disorder characterized by the appearance of pruritic papules with follicular distribution, localized in areas where apocrine glands are present. Its etiopathogenesis is not clearly known, and it involves the obstruction of the apocrine excretory duct as an early phenomenon in the process. We present a case of histologically confirmed Fox-Fordyce disease with high clinical expressivity in a 16-year-old female.