PAPULAR MUCINOSIS

Most patients previously reported to have papular mucinosis had the generalized lichenoid papular form (scleromyxedema) with abnormal gamma globulin. Microscopic examination of affected skin showed increased acid mucopolysaccharides in the dermis and proliferation of fibroblasts. Our patient had the discrete form of papular mucinosis without abnormal serum gamma globulin but with increased amounts of acid mucopolysaccharides in the dermis and no proliferation of fibroblasts.     

FOLLICULAR MUCINOSIS A STUDY OF 47 PATIENTS

SUMMARY. .
Forty-seven patients suffering from follicular mucinosis, collected over a period of 16 years from 1952 to 1968, have been studied in detail. Certain clinical and histological features of interest have been noted, and the natural history of the condition has been assessed in 40 patients.
In one group the lesions cleared spontaneously within a few months; in a second group the condition pursued a chronic relapsing but benign course over several years, and in a third group the lesions appeared in association with an overt reticulosis. This association was observed in 8 patients, representing 17% of the total group, and in each case the reticulosis was recognizable clinically and histologically early in the illness. No instance of transition from follicular mucinosis to reticulosis was observed. It is concluded that neither the chronicity of the follicular mucinosis or the age of onset of the condition are of importance in determining the association with a reticulosis, which appears in all cases to run an independent course.
Reticulosis was observed in only a proportion of those patients with extensive lesions, and was not seen in those in whom the lesions were limited to the head and neck. Recognition of those patients particularly at risk enables a more exact prognosis to be given to the individual case.     

GENERALISED ERUPTIVE SYRINGOMA-A PAPULAR DERMATOSIS

Generalized eruptive syringoma is a rare condition characterised by multiple papules over the anterior trunk, sometimes extending on to the flexural aspects of the arms and thighs. A 22 year old Ethiopian woman with this disorder is presented.     

NEUROPEPTIDERGIC ACRAL DYSESTHESIA: A NEW ENTITY

During a 4 year period, we observed three patients, aged 74, 47, and 55, with an average 12-year history of chronic itching and prickling skin discomfort. The dorsal and paimar surface of the hands and feet were involved without observable cutaneous lesions. We followed the patients for 1.5 years in our department. None of the subjects was dermographic, had personal or family history of atopy, or took drugs. The symptoms were not related to the degree of skin dryness, serum IgE levels, exercise, neoplasias, or high environmental temperature and low humidity caused by central heating or seasonal variations. No neurologic alterations were observed in a complete neurologic examination. Emotional upset did not induce symptoms. Upon psychiatric evaluation, the patients showed no alterations in their personality profile. Water exposure did not modify the symptoms. The wheals and pruritus induced by the intradermal injection of 1:10,000 histamine phosphate did not differ from those in three controls¹ Immersion of one hand and foot for 5 days per week for 2 weeks in water at different temperatures (0–45°C) for varying lengths of time and the administration of one minimum erythemal dose of ultraviolet light (280–340 nm) to the controlateral part of the body three times per week for 3 weeks both failed to reduce the severity of the discomfort. Biopsy specimens were taken from the symptomatic skin of the three subjects and from three controls. The specimens were routinely stained with hematoxylineosin for mast cells, elastic fibers, and glycosaminoglycans. There were no significant differences between the two groups. Cutaneous fibrinolytic activity, which is due to the release of cutaneous plasminogen activators, was similar in both groups.² Direct immuno-fluorescence staining (dif ) for neuropeptides substance P (sp) (Fig. 1), vasointestinal polypeptide (vip ), and calcitonin gene related peptide (CGRP) showed an increased number of pep-tidergic fibers in affected skin. After 2 weeks of three times daily application of 0.25% capsaicin in cold cream (8-methyl-N- vanillyl-6-nonenamide, known to interfere with the storage and release of neuropeptides), the symptoms disappeared completely, and neuropetidergic fibers were no longer de-tectable in the skin, as shown by dif . The application of cold cream alone on the contralateral part of the body did not modify DIF or the clinical symptoms. After suspension of capsaicin treatment, a relative to absolute refractory period of 10 to 18 days was observed, and the symptoms reappeared. Previous treatments with systemic Hi (with and without H²) antihistamines, antide-pressants, hypnotics, and topical corticosteroid prepara-tions did not achieve significant results. The increased number of neuropeptidergic fibers in the affected acral skin, the dramatic action of capsaicin in reducing the storage of neuropeptides in the same cutaneous fibers, and the com-plete disappearance of the clinical symptoms suggest that the cases reported here represent a distinct clinical entity, which could be called or described as neuropeptidergic acral dysesthesia.     

AN UNUSUAL EPITHELIAL-CONNECTIVE TISSUE NAEVUS WITH PERIFOLLICULAR MUCINOSIS*

Two pigmented lesions (one on each cheek of an adolescent boy) have been examined histologically on two occasions at an interval of eighteen months. The lesions had appeared following exposure to strong sunlight. Epithelial thickening with increased pigmentation, perifollicular mucin, absence of sebaceous glands and increased elastic tissue were found. Some comments are offered to explain these changes. It appears that the lesions are of naevoid origin.     

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.     

NECROLYTIC ACRAL ERYTHEMA: A CUTANEOUS MARKER OE VIRAL HEPATITIS C

Background. Necrolytic acral erythema (NAE) is a distinctive skin lesion that was found to affect the dorsa of the feet of seven patients having active viral hepatitis C. Necrolytic acral erythema occurs in the form of well circumscribed dusky erythematous areas that develop flaccid blisters in their early stages and a hyperkeratotic surface in their chronic form. Microscopically, lesions of NAE are similar to those of other necrolytic erythemas such as necrolytic migratory erythema, pellagra, and zinc deficiency. Method. Seven patients with NAE were included in this study. These patients underwent microscopic examination of punch biopsy specimens of the affected skin, abdominal sonography, ct scan of pancreas, and a liver biopsy. Blood samples were obtained for complete blood picture, serum glucose, zinc, amino acids, liver function tests, and markers of hepatitis. Results. All patients with nae were found to have hepatitis C by elisa and pcr . Conclusions. Necrolytic acral erythema is a distinctive type of necrolytic erythemas that was observed to occur almost exclusively with viral hepatitis C. Therefore, it should be considered an important cutaneous marker of hepatitis C, particularly in areas showing a high incidence of this form of hepatitis.