Benign Cutaneous Degos' Disease

Abstract

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.

Malignant and benign forms of atrophic papulosis (Köhlmeier-Degos disease): systemic involvement determines the prognosis

“…In conclusion, atrophic papulosis is a rare disease with a severe and potentially life-threatening systemic form. In contrast, the cutaneous form is remarkably benign, a fact that confirms the findings of other authors 10,[13][14][15] and indicates that the previously so-called 'malignant atrophic papulosis' should be renamed 'atrophic papulosis' and classified into a malignant systemic disease (malignant atrophic papulosis) and a benign cutaneous disease (benign atrophic papulosis). Patients should be worked up and followed in specialized centres following a standard plan ( Table 3).…”

Benign cutaneous Degos' disease

“…It seems that familial cases may have a better prognosis 2 . Nevertheless, approximately 15% of Degos' disease cases exhibit long‐term survival with disease often limited to the skin and with no history of systemic involvement 9 . To emphasize this clinical picture, some authors have advocated the term benign cutaneous Degos' disease 10 .…”

“…2 Nevertheless, approximately 15% of Degos' disease cases exhibit long-term survival with disease often limited to the skin and with no history of systemic involvement. 9 To emphasize this clinical picture, some authors have advocated the term benign cutaneous Degos' disease. 10 As yet, there are no means by which one can predict which patients will or will not develop visceral involvement.…”

Malignant atrophic papulosis (Köhlmeier-Degos disease) - A review

“…However, these characteristic features cannot be demonstrated in all cases [25]. Harvell et al [26] examined in a case report the histology of the lesions according to the duration of their existence. Early lesions have shown a superficial and deep perivascular lymphocytic infiltration, with distinct mucin deposition, which resembled lupus erythematosus.…”