Dermatological Clue to Diagnosis of Degos Disease in a 2-Year-Old With Obscure Chronic Abdominal Pain

Abstract

months. No cause could be found despite multiple investigations. There was poor response to medication. The child was referred to dermatology for the evaluation of a rash that had developed over the past 1 month. According to her mother, the rash appeared as erythematous papules in crops and then resolved with scars. Examination revealed multiple painful erythematous papules scattered over the limbs in different stages of evolution (Figure 1). Some were raised whereas others were umblicated in the center with a necrotic base. Healed lesions appeared as whitish, porcelain-like macular scars (Figure 2). Systemic examination was normal. Her complete blood count, renal functions, liver functions, abdominal sonography and barium meal follow-through, and coagulation profile including the international normalized ratio and serum antinuclear antibody revealed no abnormality. Skin biopsy showed a thin epidermis with hyperkeratosis overlying a necrobiotic dermal collagen with sparse inflammatory infiltrate. The blood vessels were dilated with swelling of endothelial cells and luminal thrombosis. Scattered mucin deposits were also seen. Based on historical features, characteristic skin lesions, and a supportive histology, the patient was diagnosed with Degos disease. Aspirin (3 mg/kg) and dipyridamole (5 mg/kg) were prescribed. Within 48 hours, the patient improved. Irritability reduced significantly. Active lesions started healing. Abdominal pain improved but vomiting continued although frequency was reduced significantly. At the time of reporting, the patient is still admitted in our pediatric

Dermatologic manifestations of colonic disorders

“…Cutaneous lesions are believed to be the constant feature of the disease and are almost always the initial presenting feature, although there have been case reports of the gastrointestinal system becoming involved first [33,34 ]. The gastrointestinal system is involved in approximately 50% of cases (mostly small bowel), and intestinal necrosis, perforation and ultimately peritonitis is the most frequent (62%) cause of mortality [28]; the CNS is involved in approximately 20% of cases, but death can also be due to other affected systems, including cardiopulmonary [25,29 ].…”

Congenital Degos Disease: Case Report and Dermoscopic Findings

“…1A,B) may demonstrate interface dermatitis, intraluminal thrombi, dermal sclerosis, and epidermal atrophy. The classical wedge-shaped area of ischemia may be observed in developed and late-stage lesions, which usually lack perilesional erythema, but characteristic histologic features are not always consistently present, which may delay diagnosis (1,4,6,7,22). In our review, 13.8% (n = 5) of the children were found to have the typical wedgeshaped ischemia in the dermis (2,3,9,18,19) (Table 1).…”

“…Although DD most commonly affects middle-aged Caucasian adults, with a male preponderance, onset from birth to 70 years has been described (1,2). Pediatric cases are rare, and only a single case with presentation at birth has been reported (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(18)(19)(20)(21)(22)(23). Most cases appear to be sporadic, but some suggest the possibility of autosomal dominant inheritance (1,9,12).…”

“…There is no standard treatment for DD. In an attempt to address the presumed pathogenetic mechanism, antiplatelet or anticoagulant agents such as acetylsalicylic acid, dipyridamole, and heparin are often prescribed (2,4,6,(8)(9)(10)(11) (Table 1). Immunosuppressive regimens including cyclosporine, azathioprine, cyclophosphamide, and systemic corticosteroids have been tried and are ineffective (2,3,6,7,20,21) (Table 1).…”

Atrophic papulosis (Köhlmeier–Degos disease) in children and adolescents—A cross–sectional study and literature review

“…However, the number of case reports of paediatric atrophic papulosis (pAP) is constantly growing (Table 1). 3,6,9–41 Our study aimed to investigate the early occurrence of the disease, including congenital manifestations, and to compare this to adult atrophic papulosis (aAP).…”