A Case of Benign Atrophic Papulosis in a Young Male.

Dear Editor:

Atrophic papulosis (Degos disease) is a rare, thrombo-occlusive, multisystem disease. However, some patients have been described with a benign clinical course of sole skin involvement1. Herein, we present a 32-year-old male patient with classic skin lesions without systemic involvement during 7 years’ follow-up.

A 32-year-old male was admitted to our department for a one-year history of multiple erythematous papules with atrophic porcelain-white center on the trunk and extremities. He denied a history of headaches, arthritis, visual symptoms, gastrointestinal discomfort or any constitutional symptoms. Physical examination revealed numerous 3~10 mm papules with a porcelain-white atrophic center and an erythematous halo, alongside scattered atrophic white scars (Fig. 1A, B). The lesions were distributed across his trunk and limbs.

Fig. 1

(A, B) Clinical photographs before treatment. (A) Papules on the chest with white atrophic center and an erythematous rim. (B) Lesions on the back similar to those of the chest, alongside atrophic white scars. (C, D) Histopathological examination (C: H&E, original magnification ×40; D: H&E, original magnification ×200): back biopsy shows hyperkeratosis and epidermis atrophy, perivascular lymphocytic infiltration, melanin incontinence, homogeneous degeneration of collagen and wedge-shaped necrosis in dermis. There is also striking mucin deposition within the dermis.

Results of routine laboratory tests were all normal. His neurological and mental state examination had unremarkable results. Electrocardiography, echocardiography, and abdominal ultrasonography showed no abnormalities. Histopathological examination showed hyperkeratosis and epidermis atrophy, perivascular lymphocytic infiltration, melanin incontinence, homogeneous degeneration of collagen and wedge-shaped necrosis in dermis. There was also striking mucin deposition within the dermis (Fig. 1C, D). The diagnosis of Degos disease (benign atrophic papulosis [BAP]) was made.

Treatment was started with dipyridamole 25 mg twice a day. The color of rash became lighter after 5 months. Treatment discontinued after two years. After five years, the lesions continued to spread on his trunk and extremities. He reported no systemic symptoms to date. A Longer follow-up is still needed.

In 2014, Zouboulis et al. proposed that the previously socalled malignant atrophic papulosis (MAP) should be renamed as atrophic papulosis which could be classified into two variants: MAP and BAP12. It usually occurs between the 20th~50th year of life1. Pediatric cases have also been reported3. The differences between MAP and BAP is shown in the Table 11234. MAP and BAP can be distinguished only by the presence of systemic disease in MAP, which has a median onset of 1 year (0.03~0.97 quantiles: 0~7 years) after cutaneous presentation and portends a poor prognosis1. The probability of a benign course increased with time, from 71% at onset to 97% after 7 years of monosymptomatic cutaneous disease1. The etiology of atrophic papulosis remains unknown but coagulopathy, vasculitis and primary endothelial dysfunction are the most commonly suggested hypotheses1. Recently, anomalies in complement activation C5b-9 and dysregulation of interferon-α have been implicated5. Histopathologic findings include epidermal atrophy, dermal perivascular lymphocytic infiltrate, interstitial mucin deposition, and wedge-shaped dermal necrosis1, which are coincident with ours. Treatment options for atrophic papulosis are limited. First line treatments include aspirin, pentoxifylline, dipyridamole, ticlopidine. Recently, treatment with eculizumab and treprostinil have demonstrated partial improvement4. When systemic involvements such as acute abdomen or cerebral artery thrombosis occurs, surgical operation and thrombolytic therapy are feasible.

Table 1

Different characteristics between malignant atrophic papulosis (MAP) and benign atrophic papulosis (BAP)

Characteristic	MAP	BAP
Sites involved	Gastrointestinal tract, pericardial cavity, pleural cavity, muscles, diaphragm, central nervous system, myocardium, lungs, eyes, skin	Only skin
Cutaneous lesions	A depressed atrophic plaque with a white hue and a peripheral violaceous rim
Vulnerable population	In the third to fifth decade of life
	Male	Female
Histopathologic features	A superficial and deep perivascular and periadnexal lymphocytic infiltrate that is accompanied by interstitial mucin deposition. Inverted, wedge-shaped dermal necrosis
Treatment	Heparin and antiaggregant (or platelet aggregation-inhibiting) drugs, eculizumab and treprostinil
	When bowel perforation or cerebral artery thrombosis occurs, surgical operation and thrombolytic therapy are feasible	-
Prognosis	Cumulative 5-year survival rate of MAP is approximately 55.4%	No patient had a lethal outcome

In summary, we present a benign variant of atrophic papulosis without systemic involvement during 7-years’ follow-up, emphasizing the different course between clinical spectrum of malignant variant and benign form. Because systemic involvement may develop years after the onset of cutaneous lesions, it is crucial to perform regular follow-up of the patients.