The various clinical patterns of xanthoma disseminatum. Considerations on seven cases and review of the literature.

BACKGROUND: Xanthoma disseminatum (XD) is a rare mucocutaneous xanthoma nowadays classified as a benign non-Langerhans cell histiocytosis. OBJECTIVE: We studied 7 cases of XD with a long follow-up and reviewed the literature in order to compare and discuss previous reports. METHODS: We studied clinical manifestations, course, histopathologic, ultrastructural and immunohistochemical findings of 7 cases of XD followed for 1-20 years. RESULTS: We report our findings and compare our cases with those described in the literature. CONCLUSIONS: XD and Langerhans cell histiocytosis were distinguished by clinical differences, as well as ultrastructural and immunohistochemical methods. Three clinical patterns of XD appeared to emerge in our group of patients and in the cases reviewed from the literature.

[Vater Paccini's neurofibroma. Report of 3 cases]. / Neurofibroma de Vater Paccini. Reporte de tres casos.

The authors study three clinical cases of Pacinian neurofibromas. All of them were located in the fingers. A bibliographic review was performed. The morphologic, clinical and histological features are reviewed and stressed its benign behavior and special localization.

[Eosinophilic migratory nodular panniculitis (human gnathostomiasis)]. / Paniculitis nodular migratoria eosinofílica (gnathostomiasis humana).

The case of a patient with thoracic abdominal and left arm, migratory erythema-edematous plates over a space of three years is described. The histopathologic features reveal a eosinophilic panniculitis due to probable larva migrans (gnathostomiasis). This appears to be the first report on the subject because we have not found any reference to the subject in Argentina bibliographic search.

[Trichodiscomas associated with fibrofolliculomas and acrochordons]. / Tricodiscomas asociados a fibrofoliculomas y acrocordones.

A case of trichodiscomas associated to fibrofolliculomas and acrochordons is presented. The histological and histogenic characteristics are discussed. Bibliographic revision is made. It is possible to consider the case above mentioned as a Birt-Hogg-Dubé syndrome.

Palmar subcutaneous lipoma.

We report the case of a patient with a large palmar subcutaneous lipoma. The unusual site of the lesion and the importance of a differential diagnosis to rule out angiolipoma are stressed.

Two cases of infantile porphyria cutanea tarda: successful treatment with oral S-adenosyl-L-methionine and low-dose oral chloroquine.

In a 7-year-old girl and a 12-year-old boy, with photosensitivity and hypertrichosis, the diagnosis of familial porphyria cutanea tarda was confirmed by the characteristic pattern of urinary porphyrin excretion, diminished erythrocyte uroporphyrinogen decarboxylase and elevated plasma porphyrin index with emission maxima at 617-618 nm. The patients were treated with a combination of low-dose oral chloroquine and oral S-adenosyl-L-methionine (SAM); in one case alkalinization of urine was also applied. Complete clinical and biochemical recovery was achieved within 3 months. No adverse ophthalmological or other side-effects were observed. We propose that the treatment of choice should be oral SAM (12 mg/kg/day) for 3 weeks and oral chloroquine (2 X 100 mg weekly) for about 120-150 days or until improvement of clinical and biochemical abnormalities is attained. So far no relapses have occurred. This combined therapy appears to be safe, simple, effective and very convenient for both patients and physicians.

[Arnozán-Dubreuilh depilating folliculitis]. / Foliculitis depilante de Arnozán y Dubreuilh.

Male patient, aged twenty-six years. Having an eight-and-a-half-year- old dermatosis of localized evolution on scalp, under arms, thighs, pubis and legs, characterized by inflammatory papules and follicular and extra-adnexial pustules, tiny scars and lack of hair in the affected areas. The authors present a representative case of "depilating folliculitis" of (Arnozán and Dubreuilh) stressing the following: Uncommon frequency in our country but frequent in warmer climates where it is observed mainly among peasants and sugar-cane workers. Wide-depilated areas principally in folds. Seasonal outbreak. Good response to treatment. A revision of the theme is made emphasizing the histopathologic pathogenesis.

[The hypertrophic scar]. / La cicatriz hipertrófica.

The authors briefly review the literature about hypertrophic scars, emphasizing the etiopathogenesis and treatments, taking in account, the good therapeutic results of pressure-treated scars.

Multiple disseminated pyogenic granuloma.

A 15-month-old baby girl with extensive skin burns developed within the burned areas multiple lesions with the clinical and histological characteristics of granuloma pyogenicum. These lesions seem to differ from those reported by others as pyogenic granuloma with multiple satellites.

Squamous cell carcinoma of the nail bed.

A case is presented of squamous cell carcinoma developed from carcinoma in situ of the nail bed of the first finger in a 79-year-old patient. Stress is laid upon the importance of the differential diagnosis. Surgery is considered the treatment of choice for this uncommon condition.

[Keratodermic genodermatosis with hydrocystomas, miliary cysts, xanthelasmas, nail and dental dystrophies, and basal cell epitheliomas]. / Genodermatosis queratodérmica con hidrocistomas, quistes de milium, xantelasmas, distrofias dentarias y ungulares y epiteliomas basocelulares.

Two cases of a condition including palms and soles keratoderma, ectodermal dysplasias and basal cell epitheliomas are reported by the authors, with clinical, genetical and pathological data. The condition is called Keratodermal Genodermatose with hydrocystomas, milium cysts, xanthelasma, dental and nail dysplasias and basal cell epitheliomata (Borda's syndrome).

[Scleroatrophying and degenerative keratodermic genodermatosis of the extremities]. / Genodermatosis escleroatrofiante y queratodérmica de las extremidades frequentemente degenerativa.

A case of a picture described by Huriez et al. under the title of scleatrophying and keratodermic genodermatosis of the limbs usually degenerative is presented. In the genealogic study it was observed that seven out of the sixteen members that belonged to this primary family group were affected or probably affected and it is stressed that one of them died from metastasis of spinocellular epithelioma.

[Benign circinated pityriasic erythema in children]. / Eritema circinado pitiriásico benigno del niño.

The case is report of a boy aged 6 months whose dermatosis had set in suddenly 15 days before in the form of large round circinate patches on the face, thorax, abdomen, dorsum and extremities. These patches were limited by a slightly elevated erythematous border, the inside of which was constituted by collarette-like epidermal desquamations with central parakerotosis. The patches impairment was observed in the child's general condition. A histopathological study was carried out.

Acquired pigmentation of skin folds with the histological picture of seborrhoeic wart.

The case history is presented of a woman with acquired lesions in the axillary, submammary, intermammary, inguinal, genitocrural and perianal skin folds. These lesions were confluent pigmented macules, giving rise to extensive (en nappe) reticulate patches. History revealed changes typical of seborrhoeic wart. The name patchy (en nappe) seborrhoeic wart is suggested for this condition.

[Basal and spinous cell epitheliomas]. / Epiteliomas baso y espinocelulares

A study on 502 epithelial cutaneous cancers was carried out by the authors. The study included 377 basal cell carcinomas (57,5% in males and 42,4% in females) and 125 squamous cell carcinomas (78,4% in males and 21,6% in females). The basal cell carcinomas in both sexs had an earlier onset than the squamous cell carcinomas.