We encountered a case of elastosis perforans serpiginosa in a patient with coexisting renal disease, the first such reported association of which we are aware. The perforating disorders tend to have atypical clinical features when associated with renal disease. The exact pathogenic mechanism by which renal disease predisposes to a perforating disease remains to be elucidated but is likely to be related to minor trauma, which occurs in renal disease.
The clinical manifestations of sporotrichosis can be classified into three disease patterns: (1) localized lymphatic sporotrichosis, (2) fixed cutaneous (or endemic) sporotrichosis, and (3) disseminated sporotrichosis. This presentation reflects a rare disseminated form of the disease with extensive cutaneous and systemic lesions, and further illustrates the difficulty in diagnosing systemic sporotrichosis infection.
A 17-year-old African woman presented with a 4-year history of palmoplantar keratoderma and a 2-year history of progressive constrictive lesions of the digits. There was no history of this disorder in her parents, grandparents, or two sisters. Apart from the cutaneous lesions, the rest of the clinical examination was normal. The skin involvement (onsisted of three distinct types of lesions.1. Both palms and both soles were extensively involved with a diffuse form of keratoderma ( Fig. 1). There was slight extension onto the lateral aspects of the hand and fingers where the keratoderma ended abruptly with coarse scaling. The thickened skin on the palms had a wavy, reticulated appearance due to variation in the thickness of the keraloderma (Fig. 2). This retirulafed appearance was not evident on the soles. The knuckles also had thickened skin, and the fingers were tapered.
2.Fibrous constricting bands were present on several digits (Fig. i). Both little toes had undergone aulo-amputation. Some fingers had more than one constricting band present. A total of 14 such lesions were counted on the fingers in varying slages of evolution. The small fingers were the most extensively involved: progressively fewer lesions occurring on the remaining fingers, and no lesions on the thumbs..!. Papular lesions (Fig. 4) were present on the dorsa of the wrists, on the ankles, and on the medial aspects of the knees. These were linear, hut. in some areas, coalesced to form plaques. The papules were slightly verruciform hut had a glistening surface. Some of the papules were depigmented.An x-ray of the hand showed hone thinning and osteoporotic changes distal to the constricting lesions.The nails, hair, and teeth were all normal and no hematologic or biochemical abnormality could be demonstrated on routine investigation.
HistopathologyBiopsy specimens were submitted from the byperkeratotic palm and from the papular lesions on the dorsum of the wrist. The palmar lesions showed hyperkeratotis without parakeratosis, a thickened granular layer, and a moderate degree of acanthosis. There was no cellular infiltrate in the dermis.The papular lesion ( Fig. 5} showed considerable hyperkeratosis, a marked increase in the thickness of the granular layer, and acanthosis. There was papilloma-FiG. 1. Right hand showing diffuse keratoderma and ainhum lesions of the digits. FIG. 2. Left hand showing reticulated hyperkeratosis with coarse scaling at the lateral margins.249
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.