AN ACQUIRED FORM OF ACRODERMATITIS ENTEROPATHICA DUE TO LONGTERM LACTOSE-FREE MILK ALIMENTATION

A 4-month-old boy, fed on lactose-free milk for the treatment of intractable diarrhea for about 3 months, developed acrodermatitis enteropathica-like skin lesions. All the symptoms dramatically disappeared 2 weeks after switching from this milk to a general cow's milk formula, and his low serum zinc level also rapidly returned to normal. The zinc deficiency seen in this case was presumed to be closely related to longterm lactose-free milk alimentation. Acrodermatitis enteropathica, a human zinc deficiency, should be classified into two types, hereditary and acquired. The latter form can develop from intravenous hyperalimentation, longterm lactose-free milk alimentation and longterm penicillamine administration.     

ON INFANTILE PAPULAR ACRODERMATITIS (GIANOTTI DISEASE) AND INFANTILE PAPULAR-SIMILVESICULAR ACRODERMATITIS (GIANOTTI SYNDROME)

Six cases diagnosed as Gianotti-Crosti syndrome were divided into 2 groups, a group with liver dysfunction and a group with normal liver function, and the other signs and symptoms of the 2 groups were compared with special attention to differences in the cutaneous findings. The eruption in the group with liver dysfunction corresponds to that of Gianotti disease and in the group with normal liver function to that of Gianotti syndrome, which led to the conclusion that the two should be differentiated. The important points of differentiation are summarized as follows: In Gianotti disease there is acute hepatitis and the eruption is monomorphous with multiple disseminated 3–4 mm in diameter, copper red, flat-topped papules. Gianotti syndrome is without hepatitis, and the eruption consists of irregularly distributed 2–4 mm in diameter, faintly erythematous, hemispherical papules, but is frequently polymorphous, with intermingled vesicle-like, hemorrhagic, and molluscum contagiosum-like, papules.     

PAPULAR ACRODERMATITIS OF CHILDHOOD (GIANOTTI'S DISEASE)

Two cases of papular acrodermatitis of childhood (Gianotti's disease) are reported. Characteristic skin lesions and acute anicteric hepatitis associated with hepatitis B surface antigenemia were observed in each case. Subtype analysis of hepatitis B surface antigen (HBs-Ag) disclosed “adr” and “adw” respectively, which prevail among Japanese asymptomatic carriers. Intrafamilial father-to-child transmission of HB virus was strongly suspected in case 2. HBs-Ag disappeared from the serum after three months in case 1, but persisted in case 2, who also developed chronic persistent hepatitis.     

ACRODERMATITIS ASSOCIATED WITH ZINC DEFICIENCY: FEATURES AND POSTULATED MECHANISM

It is currently unexplained why zinc deficiency should result in a cutaneous eruption of such a distinctive and characteristic distribution (acrodermatitis), affecting the groin, periorificial surfaces and periungual areas of fingers and toes. In this study, we provide ultrastructural evidence suggestive of excessive damage of the epidermis by neutrophils, with other features resembling tissue damage from free radicals. We propose that these changes are consistent with a deficiency of superoxide dismutase, a zinc metalloenzyme, which is mainly responsible for neutralizing the destructive effects of the reactive oxygen species released by neutrophils. The various zymosans on many yeast cells, including Candida, activate complement, resulting in the generation of C5a, a potent chemattractant for neutrophils, and the location of the eruption in the characteristic sites in zinc deficiency may be explained by the natural occurrence of Candida albicans in these areas.     

NECROBIOTIC XANTHOGRANULOMA*

Eight patients who developed unusual xanthomatous plaques with a tendency to ulceration and scarring and subcutaneous extension are described. Six patients had an IgG monoclonal serum paraprotein. Two patients had multiple myeloma and one patient had a plasmacytic lymphoma. Neutropenia, hypocomplementaemia and cryoglobulinaemia were variable features. Sixteen skin biopsies showed a consistent pattern of xanthogranulomatous nodules separated by zones of necrobiosis and an admixture of prominent giant cells. Lymphoid or plasma cell nodules may occur in the biopsies. Necrobiotic xanthogranuloma belongs to a range of xanthomas which has been associated with dysproteinaemia and lymphoreticular malignancy.     

CONGENITAL LEUKAEMIA*

Congenital leukaemia is a rare condition hut is often characterized by multiple cutaneous skin infiltrates. A case is discussed and the literature is reviewed with particular reference to pathogenesis, clinical presentation, investigations and differential diagnosis. The prognosis for survival may improve with early diagnosis and treatment.     

Genital candidosis*

Vaginal thrush is a condition that has been known to medicine for well over a century. Over the years it has received considerable clinical and laboratory attention, Candida albicans, by far the predominant cause of thrush, has been scrutinized in fine detail by biochemists, microbiologists and immunologists. A plethora of antifungals formulated for intravaginal use has been made available to combat the infection. Yet vaginal candidosis remains one of the most common gynaecological complaints in Europe and North America. Its diagnosis is usually simple, but its treatment can be extremely difficult. There is still doubt as to the major epidemiological and pathological factors involved in the aetiology of the infection, so the reasons for failure by some patients to respond to treatment are uncertain. Meanwhile, candidosis of the penis, which appears to have about one-tenth the prevalence of vaginal candidosis, has attracted very little research interest at all. This article will describe aspects of genital candidosis that have been the subject of research in recent years.     

Bloom's Syndrome*

The clinical features, follow up, and laboratory investigations of a case of Bloom's Syndrome, originally known as congenital telangiectatic erythema resembling lupus erythematosus in dwarfs and congenital telangiectatic erythema and stunted growth, is presented in a six-year-old girl, as well as a review of relevant literature on this rare condition.