Benign cutaneous Degos' disease - A case report with emphasis on histopathology as papules chronologically evolve

The following case report details a 53-year-old man with a 6-year history o f the benign cutaneous or skin-limited form of Degos' disease. Clinically, the patient demonstrated a diffuse eruption of papules on the upper trunk; and arms. Many papules demonstrated the classic porcelain-white centers cha racteristic of Degos' disease, but others exhibited different clinical morp hologies that corresponded to the evolutionary stages of papules originally described by Degos. Over the course of several clinic visits, the patient underwent a total of 5 punch biopsies, the histologies of which were correl ated with their clinical morphologies. Early papules were skin-colored and demonstrated a superficial and deep perivascular; periadnexal, and perineur al chronic inflammatory cell infiltrate associated with interstitial mucin deposition. The overlying epidermis showed a mild vacuolar interface reacti on and the histologic appearances at this early stage resembled tumid lupus erythematosus. Fully developed papules were raised with umbilicated porcel ain-white centers and a surrounding erythematous rim. Histologically these exhibited a prominent interface reaction with squamatization of the dermo-e pidermal junction, melanin incontinence, epidermal atrophy, and a developin g zone of papillary dermal sclerosis that resembled the early stapes of lic hen sclerosus et atrophicus in miniature. These interface reactions were in variably confined to the central portion of the punch biopsy specimen, corr esponding to the central porcelain-whits area seen clinically. Additional f eatures of fully developed papules included a prominent lymphocytic vasculi tis affecting venules, a mild periadnexal infiltrate of neutrophils and/or eosinophils, and interstitial mucin deposition In late-stage papules, the p orcelain-white areas were better developed and the lesion flattened. Histol ogically, the degree of inflammation was generally sparse and the overall p icture mirrored the classic histologic description of Degos' disease with a central roughly wedge-shaped zone of sclerosis surmounted by an atrophic e pidermis and hyperkeratotic compact stratum corneum. These late- stage papu les closely resembled a miniaturized version of fully developed lichen scle rosus et atrophicus confined to the center Of the punch biopsy specimen.