More than 200 patients with prurigo pigmentosa, a disease described first by Nagashima in 1971, have been reported on in Japan, but only 28 non-Japanese patients have come to notice as of today. In order to establish reliable, repeatable criteria for diagnosis of the disease, we studied 25 patients with prurigo pigmentosa and reviewed the literature pertaining to it as recorded in another 182 patients.Clinically, prurigo pigmentosa presents itself as pruritic urticarial papules, papulovesicles, and vesicles arranged in reticular pattern and distributed symmetrically on the back, neck, and chest. Lesions involute in a matter of days, leaving behind netlike pigmentation. Exacerbations and recurrences are the rule. Histopathologically, prurigo pigmentosa begins with a superficial perivascular infiltrate of neutrophils. Shortly thereafter, neutrophils are scattered in dermal papillae and then sweep rapidly through an epidermis in which spongiosis, ballooning, and necrotic keratocytes are accompaniments. En route, abscesses may form in the surface epithelium. Very soon, eosinophils and lymphocytes come to predominate over neutrophils in a dermal infiltrate that assumes a patchy lichenoid pattern. Intraepidermal vesiculation follows on spongiosis and ballooning and, sometimes, subepidermal vesiculation on vacuolar alteration at the dermo-epidermal junction. As the epidermis becomes hyperplastic, parakeratotic, and slightly hyperpigmented, melanophages begin to appear in the dermis. Studies by immunofluorescence are negative invariably. Dapsone or minocyclin are effective treatments; both of those agents inhibit migration and/or function of neutrophils. The cause and pathogenesis have yet to be determined. Prurigo pigmentosa is unique among inflammatory diseases of the skin and the singularity of it is manifest both clinically and histopathologically.

Adult↗

PubMed2003

Melanomas in prepubescent children: review comprehensively, critique historically, criteria diagnostically, and course biologically.

Our series was comprised of 11 children age 10 years or younger (6 were younger than age 5) with primary cutaneous melanoma. All of the melanomas occurred de novo and all metastasized; one child died. In no instance was melanoma a clinical consideration, and in none did the histopathologist who first "signed out" the case make a diagnosis of melanoma. Despite the inability of clinicians and pathologists to diagnose correctly, with repeatability, melanomas that develop in children yet to be pubescent, those neoplasms, nonetheless, are melanomas and, therefore, criteria employed currently for diagnosis of melanoma, especially clinically, must be refined in order that they be applicable equally to melanomas in pre- and postpubescents. The vaunted ABCDs (Asymmetry, Border irregular, Color variability, Diameter >6.0mm) surely do not work for melanomas that appear in children who are prepubescent. Additionally, melanomas that occur in these children have distinctly different architectural and cytopathological features from those that arise in postpubescents, often being confused as they are by conventional microscopy with a Spitz's nevus. As a rule, melanomas in prepubescent children grow much more rapidly then those in adults but, like them, have the capability to disseminate widely and cause death.

Child↗

PubMed2003

Degos' disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se.

Degos' disease, known confusingly as malignant strophic papularis, is an uncommon condition of unknown cause characterized by distinctive infarctive lesions in the skin, gastrointestinal tract, and central nervous system; the lesions at the two latter sites often result in death. We deem Degos' disease to be analogous to lupus erythematosus in the sense that each is fundamentally a systemic pathologic process involving several organs, among them the skin, but, moreover, we regard Degos' disease, in most instances, to be an actual manifestation of lupus erythematosus. Histopathologically, the findings in sections of tissue of skin lesions of Degos' disease are indistinguishable from those of one expression of cutaneous lupus erythematosus; immunopathologically, some patients with morphologic findings stereotypical of Degos' disease display signs characteristic of lupus erythematosus. For these reasons, we consider Degos' disease to be a distinctive pattern of disease, rather than a specific disease per se, just as are erythema multiforme, erythema nodosum, leukocytoclastic vasculitis, Sweet's syndrome, and pyoderma gangrenosum, to name but five of scores of them. The singular pattern that is designated Degos' disease usually is an expression of lupus erythematosus, but, episodically, of conditions like dermatomyositis and rheumatoid arthritis.

Adolescent↗

PubMed2003