A pheochromocytoma presenting as generalized pigmentation.
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Bacterial skin infections are a common reason for children to be examined by a pediatrician. Streptococci and staphylococci are responsible for the great majority of the infections. Because of the variety of lesions produced by these bacteria, there is support for dividing impetigo into "traditional" crusted and bullous forms. Two important forms of cellulitis--facial and periorbital--have potential for serious systemic consequences. The bacterial etiology and treatment of cellulitis, animal bites, and puncture wounds of the foot require special attention for successful outcome.
A total of 21 patients suffering from drug-induced rashes from practolol have been seen over the past two years. The clinical manifestations varied, with the morphological appearances of the rash resembling those of eczema, lupus erythematosus, lichen planus, and a highly characteristic toxic erythematous psoriasiform eruption. Persistent ocular damage was a feature in three cases.
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We report a peculiar case of hemorrhagic pellagra in an exhausted Albanian refugee who had walked for 3 days under sunny skies on his way from his country to Greece. The peculiarities of the case are the fulminant course of the disorder; the "terrifying" appearance of the patient (initially he was admitted to an emergency unit); the gangrenous appearance of the hemorrhagic lesions of the palms and fingernails; the disturbed hepatic function that gradually returned to normal; and the absence of a history of alcohol consumption, alcohol malabsorption, or drug intake.
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We report the case of a young woman whose son had X-linked chronic granulomatous disease (CGD) while she was gene transmitter and presented with erythematous-squamous dermatosis predominant in unprotected regions, photosensitivity and oral ulcerations. The diagnosis of discoid lupus erythematosus, suggested by the clinical complex, was not confirmed by paraclinical examinations. CGD is an X-linked or, less often, autosomal recessive disease underlain by a selective deficiency of intraleucocytic bactericidal ability. Its clinical manifestations are repeated and severe infections involving most of the body organs. A review of the literature yielded 20 cases resembling that of our patient; all concerned mothers or sisters of children with X-linked CGD. The skin disease usually begins during childhood. The authors describe cutaneous manifestations as a photosensitive infiltrating erythema, frequently suggestive of lupus erythematosus, and oral ulcerations. At light microscopy, these skin lesions show a lymphohistiocytic infiltrate which sometimes erodes the basement membrane or remains at a distance from it. Such images suggest lupus erythematosus or, for some authors, a Jessner-Kanoff syndrome or a lichenoid infiltrate. Direct immunofluorescence was negative in all but one case, and there was usually no laboratory evidence of autoimmunity. In women who transmit the X-linked form of the disease, the bactericidal activity of granulocytes is reduced by 50 p. 100, but infection is not a major problem. The pathogenesis of these disorders is not yet fully understood. However, we would like to draw attention to the studies by Roberts et al. who demonstrated a defective degradation of bacterial DNA by circulating monocytes in patients with systemic or discoid lupus erythematosus.(ABSTRACT TRUNCATED AT 250 WORDS)
The angiogenic capability of peripheral blood mononuclear cells (MNCs) from patients with psoriasis was tested by lymphocyte-induced angiogenesis assay. The study was performed in 36 patients with psoriasis vulgaris, six with generalized pustular psoriasis of the von Zumbusch type, and five with palmo-plantar pustular psoriasis. When patients with psoriasis vulgaris were considered as a whole, no significant differences in the angiogenic capability of their MNCs could be detected, as compared with controls. However, when cases of psoriasis were classified into various types, the differences in angiogenic capability of MNCs became evident. This capability was found to be markedly increased in the active form of psoriasis vulgaris and decreased in the stationary stage of the disease. It was increased in palmo-plantar pustular psoriasis but markedly decreased in generalized pustular psoriasis of the von Zumbusch type.
Nevoid basal cell carcinoma syndrome has as its hallmarks such diverse manifestations as numerous cutaneous basal cell cancers and epidermal cysts, palmar and plantar pits, keratocysts of the jaw, calcified dural folds, various skeletal anomalies, cleft lip and/or palate, and various other neoplasms or hamartomas. Inheritance is autosomal dominant. The etiology of all of the above findings appears to be a mutation in a tumor suppressor gene that also plays a role in normal embryonic development.
Two cases of pyogenic granuloma recurred despite what was originally thought to be a sufficient surgical excision. The granulomas presented clinically as polypoid structures, one on the finger and one on the chin. On histologic examination, all showed polypoid configuration with lobules of small vessels and endothelial cell proliferation. The recurrent lesions also showed similar lobules, but interspersed between the collagen bundles in the deeper dermis. Vascular tumors such as the lobular pyogenic granulomas, intravenous capillary hemangiomas, and acquired tufted angiomas were compared to our tumors.
Clinical reports of hereditary palmoplantar keratoderma are generally based on a limited number of patients. In 1967 the prevalence in the northernmost county of Sweden (Norrbotten) was shown to be 0.55%. In 1982 it was possible to trace half of the original propositi from that study. Among these families, a severe clinical form with a presumed recessive inheritance could be distinguished. The clinical pictures in relatives of the original propositi were described, and other diseases were listed together with those in patients from previously performed studies. The frequency of dermatophytosis was 36.2%, which was equal to a prevalence of 37.6%. T. mentagrophytes occurred significantly more often and immunological factors, such as increased presence of blood group A, specific dermatophyte IgG antibodies, precipitating antibodies and an immunological in vitro reaction to keratin, supported differences in the distribution of dermatophytes. However, the amount of keratin was considered the most important factor for the affinity of dermatophytes to the palms and soles. A vesicular eruption along the hyperkeratotic border and a mononuclear cell infiltrate were often reported. Such reactions were interpreted as immunological reactions to dermatophytosis. Scaling and fissuring were considered clinical signs of dermatophyte infections and not a part of the originally reported clinical picture. Results of the histopathological study corresponded to previously reported descriptions of the Unna-Thost variety. However, it has recently been reported that the histopathological picture of this variety was based on histopathological features of epidermolytic palmoplantar keratoderma. The existence on the Continent of the Unna-Thost variety was therefore questioned. Histopathological features of epidermolytic palmoplantar keratoderma were not found in the County of Norrbotten and the designation "Diffuse HPPK type Norrbotten" has therefore been proposed. The histopathological picture of the presumed recessive variety did not differ from that of the dominant variety but ultrastructural characteristics differentiated it from Mal de Meleda and the dominant variety. It was therefore concluded that a new variety with a presumed recessive inheritance was found.
A follow-up study of 47 patients who had suffered from atopic dermatitis in infancy (< 2 years aged old) was conducted by means of a questionnaire and personal interview/examination at the mean age of 23 years. It was found that 72.3% of them were still suffering from atopic dermatitis. The atopic eczema was mostly localized on the fingers (67.6%), on the head, e.g. forehead, eyelids and scalp (32% each), neck (35%) and chest (32%). Different localizations from the juvenile and adolescence phase were observed. In 73.5% the lichenoid type of atopic eczema was seen, in 67.6% the eczematic form of reaction, and in 28.4% the follicular form, the latter having decreased significantly in frequency since the adolescent phase. The pruriginous form with prurigo papules was observed only in the 8.8% of the patients who had been suffering from a chronic form of the disease since childhood. Nummular reactions were not observed. In 66% of the patients micromanifestations were present, most frequently perlèches (40.4%), retroauricular intertrigo (34%), atopic eyelid eczema (21%) and 21.3% "pulpite sèche" (tylotic, rhagadiform fingerpad eczema) (21.3%). In 14.9% of all patients these minimal forms of atopic dermatitis were present exclusively.
BACKGROUND: Several years ago, a surgical technique for treating depigmentation resulting from burn injuries was developed. The treatment of this problem in the patient with burns was consistently successful. OBJECTIVE: The purpose of this study was to investigate the usefulness of the surgical technique developed for patients with burn injuries to determine whether the same technique would be useful in treating patients with vitiligo whose skin did not repigment with conventional medical treatments. METHODS: Five patients with stable vitiligo were treated. The epithelium of the vitiliginous areas was removed by dermabrasion. The dermabraded area was then reepithelialized with epithelial sheet grafts sufficient to cover the entirety of the vitiliginous dermabraded region. RESULTS: Each operation was successful. Scarring did not develop in the repigmented or donor site regions. The final color match has been good to excellent in all patients. CONCLUSION: Good to excellent repigmentation was observed in each patient. We believe our results, to date, are more successful than the results of other procedures reported previously in the medical literature.
Wall and Gebauer (Contact Dermatitis 1991: 24: 241-243) first described persistent post-occupational dermatitis (PPOD) as ongoing dermatitis for which there is no obvious present cause, precipitated by prior occupational contact dermatitis (OCD). We propose that individuals exhibiting PPOD lose the capacity for resolution of their condition upon removal from exposure to causative agents and subsequently develop persistent dermatitis, which can be continual or intermittent. Accordingly, we suggest modification of criterion 6 of the OCD criteria developed by Mathias (J Am Acad Dermatol 1989: 20: 842-848): 'Removal from exposure initially leads to improvement of dermatitis, however, over time there may be incomplete or no improvement, despite removal from exposures at work'. To satisfy the definition of PPOD, individuals must meet at least 4 of the 7 criteria, including the altered criterion 6. We present 6 cases of PPOD exemplifying these scenarios, which met the altered Mathias criteria. In some cases, subsequent failure to recognize the initial work relatedness of their skin conditions resulted in the termination of workers' compensation benefits. This situation is particularly relevant in the Australian context. The diagnosis of PPOD needs to be considered in all individuals with work-initiated dermatitis who present with ongoing endogenous-like eczema.
Reactive perforating collagenosis is characterised by trans-epidermal elimination of collagen and is hypothesized to be both autosomally dominant and recessive. We report a family in which two brothers and a sister had lesions of reactive perforating collagenosis.
Multinucleate cell angiohistiocytoma mainly affects middle-aged women, and usually presents as grouped reddish-brown papules. Histopathological features include the presence of multinucleate cells in the reticular dermis, and numerous dilated dermal vessels. The lesions are benign, and may persist for years. Treatment is needed for cosmetic reasons. We report the successful use of the argon laser to treat two patients who had multinucleate cell angiohistiocytoma.
Low-dose low molecular heparin inhibits T lymphocyte-mediated autoimmune disease and allograft reactions in mice in vivo and in vitro. High doses of heparin are not effective. The purpose of this preliminary study was to analyze the effect of low-dose, low molecular weight heparin (Enoxaparin, Clexane) on the expression of patch testing in patients with contact dermatitis. Eleven patients with allergic contact dermatitis, and positive patch tests reactions, were given a single subcutaneous injection of Clexane 3 mg (0.03 ml) and were reevaluated for positive reactions after the injection. Eight out of 21 positive reactions (38%) became negative after the injection and 4 out of 21 reactions (19%) changed from ++ before the injection to + after. An impressive improvement was observed in 3 patients with chronic allergic contact dermatitis. Low-dose low molecular weight heparin inhibits the elicitation of allergic contact dermatitis.