Cutaneous ganglioneuroma is rare. Only five cases have been reported, and in all patients the lesions developed after birth. We describe a congenital cutaneous ganglioneuroma. The differential diagnosis includes well-differentiated cutaneous metastases of neuroblastoma and ganglion cells entrapped by a neurofibroma in patients with neurofibromatosis.

Diagnosis, Differential↗

PubMed1996

Tumor vascularity as a prognostic indicator in intermediate-thickness (0.76-4 mm) cutaneous melanoma. A quantitative assay.

Tumor vascularity has been reported to be a prognostic factor in solid tumors. We studied the prognostic value of tumor vascularity in 19 primary stage I skin melanomas. Only intermediate-thickness melanomas (0.76-4.00-mm thick) were studied. They were treated surgically to provide two groups of patients. The first group of 11 patients had no evidence of metastases after a follow-up of a mean period of 72.36 months, whereas the second one developed metastases in a mean period of 46.87 months. The two groups were matched for important prognostic factors including tumor thickness, sex, and age. Vascularity was quantified by a morphometric stereological analysis on paraffin sections stained with anti CD31 monoclonal antibody. The percentage of vascular area was significantly higher in the metastasizing group than in the nonmetastasizing one. Our study suggests that increased vascularity may have a prognostic significance in intermediate-thickness melanoma.

Female↗

PubMed1996

Mossy leg with eccrine syringofibroadenomatous hyperplasia resembling multiple eccrine syringofibroadenoma.

Eccrine syringofibroadenoma (ES) is a histological entity with a polymorphous clinical presentation. We describe a patient who had multiple nodules on both of his legs resembling lymphoedematous keratoderma (mossy leg or elephantiasis) in the absence of any vascular or lymphatic incompetence. The histological features were those of ES. A surgical resection was effective. Our case is probably an eccrine sweat duct reactive hyperplasia rather than a neoplasia or hamartoma.

Adenoma, Sweat Gland↗

PubMed1996

Solitary eccrine syringofibroadenoma (or eccrine syringofibroadenomatous hyperplasia?) and diabetic polyneuropathy.

A 70-year-old diabetic woman with sensory polyneuropathy presented with osteonecrosis of the toes and a plaque-like lesion on the dorsum of the ipsilateral foot. Histological diagnosis of eccrine syringofibroadenoma (ES) was made. A review of the literature reveals several cases of solitary ES of the foot in diabetic patients with peripheral neuropathy. This variant of ES seems to be an eccrine sweat duct hyperplasia during the restoration of skin structures damaged by traumas in a situation of peripheral neuropathy. Diabetes and polyneuropathy should be searched for in patients with ES, particularly in acral locations.

Adenoma, Sweat Gland↗

PubMed1995

Multinucleate cell angiohistiocytoma. A review and report of four cases.

Multinucleate cell angiohistiocytoma (MCA) was first characterized by Smith & Wilson-Jones. Although only a few cases have been published, this very characteristic benign tumor is probably not rare. The clinical pictures are firm circumscribed papules, mainly of the hands, which progress slowly over the years. The histologic features show an increase of capillaries and venules in the reticular dermis, overlaid by epidermal hyperplasia. Bizarre-shaped multinucleate cells are present between the vessels, and factor XIIIa-positive interstitial cells are increased in number. MCA can be easily recognised if the pathologist is aware of the diagnosis. We present here the clinical, histologic and immunopathologic features of 4 new cases and review the literature.

Adult↗

PubMed1995

Lichen planus induced by interferon-alpha-2a therapy for chronic active hepatitis C.

OBJECTIVE: To report the development of hepatitis C virus (HCV)-positive chronic active hepatitis with lichen planus in a patient during interferon treatment. DESIGN: Case report and literature review. PATIENT: A 64-year-old anti-HCV and HCV-RNA-positive woman. INTERVENTIONS: The patient received interferon-alpha-2a treatment for histologically proven chronic active hepatitis. RESULTS: Four months after the start of treatment the patient developed multiple cutaneous lesions on her hands, feet and back. A skin biopsy led to the diagnosis of lichen planus. The withdrawal of interferon was followed by a marked improvement in the cutaneous lesions, but not complete regression. CONCLUSION: This case shows that HCV-positive patients with chronic active hepatitis may develop lichen planus during interferon therapy.

Female↗

PubMed1995

[Lichen planus and thymoma. A case].

INTRODUCTION: Lichen planus is a chronic inflammatory disease of the skin, rarely associated with a thymoma. CASE REPORT: A 72-year-old woman with erosive buccal lichen, hypertrophic lichen planus of the lower limbs, severe myasthenia and acquired hypogammaglobulinaemia associated with thymoma. CD8 lymphocyte count was increased. In the months following surgical ablation of the thymoma, the clinical examination and laboratory findings progressively returned to normal. Two years later, the patient was in good health. DISCUSSION: To our knowledge, this is the first report of favourable outcome after surgical treatment despite the aggressive symptomatology. The particular outcome allow confirmation that the thymoma plays a causal role in this syndrome and emphasizes the effects a thymoma can have on the immune system.

Agammaglobulinemia↗

PubMed1995

[Desmoplastic and neurotropic subungueal melanoma].

INTRODUCTION: Desmoplastic and neurotropic melanoma is rare and involvement of the subungual skin is an exceptional finding. OBSERVATION: We report the second case of desmoplastic and neurotropic subungual melanoma in the literature occurring in a 71-year-old woman. DISCUSSION: In our patient, the histological diagnosis of desmoplastic melanoma was particularly difficult because of the exceptional involvement of the subungual skin. Microscopic neurotropism and immunohistochemical studies were the clues in diagnosing desmoplastic melanoma.

Aged↗

PubMed1994

Cathepsin D and E co-expression in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and Langerhans' cell histiocytosis: further evidences of a phenotypic overlap between these histiocytic disorders.

Nosological classification of sinus histiocytosis with massive lymphadenopathy (SHML; Rosai-Dorfman disease) is difficult, and the normal cellular counterpart of Rosai-Dorfman (RD) cells is uncharacterised. The peculiar S-100+ phenotype of RD cells suggests a relationship with the dendritic cell family. Recent investigations have revealed cathepsin E to be selectively concentrated in antigen-presenting cells, whereas cathepsin D was found to be expressed in cells of macrophage lineage. Cathepsin D and E distribution was investigated by immunohistochemistry in a series of SHML biopsies and in two types of dendritic cell proliferative lesions: dermatopathic lymphadenitis (DL) and Langerhans' cell histiocytosis (LCH). In SHML biopsies, RD cells and monocyte-related elements of the sinuses and pulp coexpressed cathepsin D and E. LCH cells also stained for both these aspartic proteinases. Conversely, in DL cathepsin E and D were localised to separate cells that resembled Langerhans' cells (LC) or macrophages, respectively, in morphology and distribution. Our data outline the peculiar immunophenotype of RD and LCH cells and suggest that caution should be exercised in the identification of their normal cellular counterpart. The common expression of cathepsin D and E and of S-100 protein suggests some phenotypic overlap between SHML and LCH cells, despite their striking morphological divergence.

Cathepsin D↗

PubMed1994

Self-healing localized cutaneous mucinosis.

A 54-year-old woman presented a lesion on the dorsum of the right hand. Routine laboratory tests were normal or negative. Histological examination revealed mucin deposits in the reticular dermis. The lesion cleared within 1 year without any treatment but biopsy. This case does not fit the diagnostic criteria of any known cutaneous mucinoses.

Female↗

PubMed1994

Mucin deposits in morphea and systemic scleroderma.

BACKGROUND: Though rarely reported, mucin deposition may be observed in scleroderma. OBJECTIVE: To verify the frequency of significant amounts of mucin in the biopsy specimens. METHODS: Biopsies from 20 patients with scleroderma were reviewed and stained to verify the presence of mucin. RESULTS: Mucin deposits were found in all of the 20 specimens. CONCLUSION: Mucin deposition is probably a constant feature in both morphea and systemic scleroderma. Its relevance in differential diagnosis between scleredema and scleroderma is debatable.

Adolescent↗

PubMed1994

Herlitz junctional epidermolysis bullosa keratinocytes display heterogeneous defects of nicein/kalinin gene expression.

Previous studies have correlated the Herlitz junctional epidermolysis bullosa (H-JEB) to an altered expression of the basement membrane component nicein/kalinin. This heterotrimeric glycoprotein appears to be present in H-JEB tissues in an abnormal form, because a number of antibodies specific to the protein either do not react with or weakly stain the epidermal basement membranes of most of the patients. With cDNA probes encoding each subunit of nicein and polyclonal antibodies raised against bacterial fusion polypeptides corresponding to the individual chains of the protein, we have molecularly analyzed the expression of nicein in H-JEB tissues and cultured keratinocytes. By immunohistochemistry, Northern blot, and protein analysis, we show a defective synthesis of one of the nicein subunits in six cases of H-JEB from five different consanguineous families. In two patients, the disease correlates with an impaired synthesis of the nicein B2 (nic B2) chain, in three others with that of the B1 (nic B1) chain, and in a sixth patient with that of the heavy A (nic A) chain. In this report, we thus demonstrate that H-JEB is a genetically heterogeneous disease and we provide strong evidence that the genes of nicein are the candidates for this genodermatosis.

Blotting, Northern↗

PubMed1994

[Necrotizing vasculitis induced by cytomegalovirus in a woman with acquired immunodeficiency syndrome].

INTRODUCTION: Skin lesions induced by cytomegalovirus are rare and usually non-characteristic. CASE REPORT: A 32-year-old women with AIDS developed about twenty unpainful ulceronecrotic lesions on the extension aspect of the members and the trunk. Histology examination and in situ hybridization favoured cytomegalovirus infection of the skin. DISCUSSION: Despite the exceptional nature of this case, this particular clinical presentation should be recognized as it could be useful for early diagnosis of cytomegalovirus infection in immunodepressed subjects.

AIDS-Related Opportunistic Infections↗

PubMed1994

Recurrent Spitz nevus. Case report and review of the literature.

The recurrence of Spitz nevus (SN) both after partial and full excision is a rare but benign event that may simulate metastatic melanoma. We describe a 5-year-old girl in whom the tumor, which had histopathologic features of SN, recurred twice. The first recurrence was after electrodessication, whereas the second was after full excision. A review of the available studies on recurrent Spitz nevus is also performed.

Child, Preschool↗