The following case report details a 53-year-old man with a 6-year history of 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 characteristic of
Degos' disease, but others exhibited different clinical morphologies 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 correlated with their clinical morphologies. Early papules were skin-colored and demonstrated a superficial and deep perivascular, periadnexal, and perineural chronic inflammatory cell infiltrate associated with interstitial
mucin deposition. The overlying epidermis showed a mild vacuolar interface reaction and the histologic appearances at this early stage resembled tumid lupus erythematosus. Fully developed papules were raised with umbilicated
porcelain-white centers and a surrounding erythematous rim. Histologically these exhibited a prominent interface reaction with squamatization of the dermo-epidermal junction,
melanin incontinence, epidermal
atrophy, and a developing zone of papillary dermal
sclerosis that resembled the early stages of
lichen sclerosus et atrophicus in miniature. These interface reactions were invariably confined to the central portion of the punch biopsy specimen, corresponding to the central
porcelain-white area seen clinically. Additional features of fully developed papules included a prominent lymphocytic
vasculitis affecting venules, a mild periadnexal infiltrate of neutrophils and/or eosinophils, and interstitial
mucin deposition. In late-stage papules, the
porcelain-white areas were better developed and the lesion flattened. Histologically, the degree of
inflammation was generally sparse and the overall picture mirrored the classic histologic description of
Degos' disease with a central roughly wedge-shaped zone of
sclerosis surmounted by an atrophic epidermis and hyperkeratotic compact stratum corneum. These late-stage papules closely resembled a miniaturized version of fully developed
lichen sclerosus et atrophicus confined to the center of the punch biopsy specimen.