Search

In several families, multiple endocrine neoplasia type 2A (MEN 2A) has been found in association with cutaneous lichen amyloidosis. It has been debated, however, whether the skin amyloidosis found in MEN 2A families, localized exclusively in the interscapular area, represents the same anomaly as that found in autosomal dominant familial cutaneous lichen amyloidosis, which is more generalized. We screened two MEN 2A families with associated skin amyloidosis for germline mutations in the RET gene responsible for the MEN 2A cancer syndrome, and found the same mutation characteristic of MEN 2A in both families. We also screened probands from three pedigrees with familial cutaneous lichen amyloidosis for RET mutations. In none of the RET coding and flanking intronic sequences was a mutation detected. This most probably indicates that skin amyloidosis found in some MEN 2A families and familial cutaneous lichen amyloidosis are different conditions. Consequently, patients with apparent familial cutaneous lichen amyloidosis do not appear to be at risk for MEN 2A.

A clinical and histopathological description is given of a number of lesions in 4 patients, mainly caused by picking at the skin. The name dermatotillomania is proposed for this group of lesions.

Squamous cell carcinoma antigen (SCC), formerly referred to as TA-4, is a tumor marker for SCC of the uterine cervix. Based on the findings in a patient with complete remission after treatment for cervical carcinoma, the authors decided to analyze the sera from patients with benign dermatoses. It was found that 83% (25/30) of the patients with psoriasis and 80% (12/15) of the patients with eczema had SCC levels in excess of the cut-off value of 2.5 ng/ml. In psoriasis the serum SCC level correlated positively with the body surface area affected by the disease (r = 0.64). Seven patients with miscellaneous skin disorders, all with an inflammatory component, showed high serum SCC levels as well. Thus the existence of an inflammatory skin disease or a hyperkeratotic skin disease with an inflammatory component interferes with the usefulness of the SCC antigen as a tumor marker in SCC of the uterine cervix.

In a case of epidermodysplasia verruciformis with impaired cell-mediated immunity and multiple skin cancers human papillomavirus type 5 (HPV5) DNA sequences were demonstrated in a cutaneous squamous cell carcinoma. HPV5 and HPV8 were detected in the benign disseminated skin lesions together with three newly characterized HPVs: HPV17, HPV19 and HPV24. A chronic infection with hepatitis B virus resulting in macronodular cirrhosis associated with a primary hepatocellular carcinoma was also acquired by this patient. This case provides an example of the circumstantial evidence which suggests that certain types of HPV are potentially oncogenic and stresses the importance of immune surveillance in the protection against virus-associated tumors.

This article discusses omenn syndrome in a male patient aged one and half year.In the first months of life this baby showed a skin eruption of the scalp suggestive of a seborrhoeic dermatitis. From the age of i year a widespread pruritic skin disorder developed with fever, lymphadenopathy, anaemia and cosinophilia. Topical steroids were not effective. From the age of 14 months chronic diarrhoea was present. Biopsy from a nodule showed acanthosis, an oedematous papillary layer and in the lower part of the dermis a dense infiltrate consisting of histiocytes, lymphocytes, eosinophils and mast cells.

This article discusses linear IgA dermatosis in a male patient aged 35. For 4 years this patient had suffered from a pruritic eruption. The individual lesions healed spontaneously in about 2-4 weeks. Mucosal lesions were not noticed. Sparse solitary vesicles or small bullae were seen, mostly on an erythematous base, disseminated over the entire skin. Direct IF of lesional and normal skin both show linear deposits of IgA along the basement membrane. Deposits of other immunoglobulins are not seen. In direct IF using guinea-pig oesophagus as substrate linear deposits of IgA, not of IgG, are found along the epithelial basement membrane.

The article presents a case report of a person suffering from epidermolysis bullosa acquisita. Since 1976, the patient has had periodic pretibial bullae following minor mechanical trauma. Tense bullae with surrounding erythema and erosive lesions were present on his chin, nostril, nipple, dorsum of two fingers and shins. Electron microscopy showed that there were abnormal deposits beneath the lamina densa zone. The erosions were dressed with gauze but repeated dressings impaired epithelialization.

Describes a case of a 49-year-old male presented with cutaneous immunoblastic T cell lymphoma. History of the patient's medical case; Clinical manifestations; Treatment.

This article discusses cutaneous malignant epidermotropic lymphoma in a male patient aged 53. In July 1980 the first cutaneous lesion was observed on the right upper leg. Subsequently an increasing number of new lesions spread over the entire skin. This revealed an apparently healthy man apart from the fact that disseminated over the entire skin, infiltrated erythematous and erythemato-squamous, partly annular plaques were observed. Cutaneous biopsies showed a dense infiltration of the epidermis with blast-like cells. In tumours, infiltration of the dermis was also seen. A biopsy of the tumour on the tongue also showed an epidermotropic infiltrate with these atypical cells.

This article discusses subacute cutaneous lupus crythematosus with polyneuropathy in a female aged 53. Since 1974 this patient has had a skin disorder, initially on the face but since 1977 also on the trunk and extremities. There are spontaneous remissions and exacerbations. Sunlight aggravates the disorder. The patient complains of a loss of sensitivity of both feet. The trunk, especially the back, the limbs and the face present a symmetrical erythematous slightly raised eruption consisting partly of papules, partly of annular and polycyclic lesions, which become scaly and disappear without scarring.

The article presents a case report of a 73-year old man having porokeratosis disseminata with cutaneous amyloidosis. From the age of 67 years, he started developing disseminating, slightly itchy skin eruption. Biopsies from the legs and buttocks showed acanthosis and hyperkeratosis and multiple parakeratotic columns in each lesion. The person was treated with topical fatty ointments, since etretinate made the hyperkeratotic fissuring worse.

The article presents a case study of an infant female suffering from junctional epidermolysis bullosa letalis. At the time of birth, her several fingers and toes showed onycholysis. Multiple small and large bullae were seen, especially on pressure sites, some tense and intact, others leaving eroded areas. A biopsy from perilesional skin confirmed the site of bulla formation to be within the lamina lucida. The child was nursed and treated according to the protocol of the Dutch Genodermatoses Study Group, but failed to thrive and died at the age of 4 months.

The article presents a case report of a 37-year old woman suffering from red itchy skin lesions. There were light brown lesions. There was a dermal perivascular infiltrate consisting of neutrophils and eosinophils and mononuclear cells. The woman was treated with topical steroids and hydroxychloroquine, but there was no significant effect.

The article presents a case report of a 54-year old male having annular elastolytic giant cell granuloma. He had non-itchy skin lesions from 3 months of age. The back and the extensor side of his arms showed red. Histology. Biopsies from the border of annular lesions on the arms and back showed a granulomatous infiltrate in the upper and mid-dermis consisting of lymphocytes, histiocytes and numerous multinucleate giant cells.

The article presents a case study of a women suffering from pemphigoid gestationis. The woman developed itchy lesions around the umbilicus soon after the birth of her child by Caesarian section. These lesions became bullous after a week. For the treatment, a complete clinical remission was produced with oral prednisolone initially at a dosage of 40 milligrams daily. There was no C3c deposition in the skin after eighteen months. There was some itching premenstrually which occurred due to the use of an oral contraceptive.