Multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs. We present a case report of this disease and an extensive review of the literature. We reviewed the data between 1991 and 2014 and extracted 52 individual cases. Only articles in English were chosen after checking for relevance. The articles were studies and data was extracted into excel spread sheets and later used to compute such variables like frequency, mean and percentage of distribution of various clinical manifestations. The treatments used in these articles were critically analyzed and graded for their relative efficacy for skin and joint manifestations. The grades were 0 = worse, 1 = no benefit/condition remained same, 2 = improvement without resolution, and 3 = resolution. This article also reports the demographic, clinical, laboratory and pathological data from the reviewed articles. Authors attempted to discuss the findings of this review in depth to help manage this condition and proposed a treatment algorithm to help clinicians approach this rare and challenging disease.

SEM is a possible adjuvant tool in the diagnosis of ED syndromes. There are significant differences, with high specificity, between the hairs of individuals with ED and those of control subjects and between subtypes.

An epithelial sheath neuroma (ESN) is a rare benign process of unknown pathogenesis, which is characterized histologically by multiple enlarged peripheral nerve fibers ensheathed by mature squamous epithelium. The histologic features can elicit concern for carcinomatous perineural invasion. The process is limited to the superficial dermis and is surrounded by a loose myxoid stroma, lymphocytic infiltrate and sometimes prominent infundibular cysts. The etiology of this peculiar entity has been debated and theories include a benign neoplasm, a hamartoma or an unusual reactive hyperplasia. There are only seven prior cases reported of ESN in the literature. Our case presented here is the first report to show connection of the ESN to the overlying epidermis and reactive epidermal hyperplasia. This suggests that ESN is indeed an unusual form of benign reactive hyperplasia. In addition, the clinical setting in this case was of pruritus and scratching in a background of papular urticaria, supporting the previous notion that ESN is probably a response to an external stimulus such as rubbing.

No abstract