A 35 years old man was hospitalised for multiple, painful and debilitating ulcers over both lower limb and scrotum. It initially started as a small wound over his right shin which gradually grew in size. Subsequently multiple similar lesions appeared extensively on both lower limb and scrotum over a period of 5 months. He was treated with multiple courses of antibiotics to no avail.
Additionally, he had 6 months history of polyarthralgia involving the inter-phalangeal joints of both hands, knees and elbows. He reported early morning stiffness of both hands with discolouration and numbness of his fingers upon exposure to cold temperatures. He also complained of bilateral lower limb numbness. He had significant impairment to his activities of daily living as a consequence of these symptoms. Besides that, he also complained of dysphagia, unintentional weight loss of 10 kg and fatigue.
There was no fever, recurrent oral ulcers, photosensitivity rash and history of gritty or red eyes. He denied chronic cough or night sweats. He had no abdominal pain or alteration in bowel habits. Review of other systems were unremarkable. Family and past medical history were insignificant. He denied alcohol intake, smoking, or use of recreational drugs. He was not a vegetarian and consumed meat regularly. There was no known food intolerance, medication allergies and surgical history.
On examination, the patient was afebrile and normotensive with a pulse rate of 110 bpm. There was clear evidence of alopecia, generalised hyper-pigmented fragile skin, depapillation of tongue and bilateral parotid swelling. Raynaud’s phenomenon was noted over both hands. Multiple tiny lymph nodes were palpable over bilateral cervical and supraclavicular areas which were soft and mobile. There were multiple ulcers on his scrotum and both legs of varying ages indicating chronicity with the largest measuring 10 × 5 cm. The ulcers were irregular in shape, had erythematous-purplish borders with a yellowish necrotic base. However, the legs were warm and well perfused, with distinctly palpable peripheral pulses. There was no nodular lesions or thrombophlebitis over the lower extremities. Neurological examination of his lower limbs revealed symmetrical paraesthesia distally with loss of vibratory sensation and diminished proprioception. Respiratory, cardiovascular and abdominal examinations were normal (Fig. 1).
Laboratory investigations revealed haemoglobin of 8 g/dl, mean corpuscular volume (MCV) of 84 fL, white blood cells of 6.27 × 10^3/μL with a lymphocyte count of 1.02 × 10^3 /μL. A peripheral blood film showed normochromic, normocytic anaemia with no evidence of haemolysis or abnormal blast cells. Anaemic work up showed low serum iron of 3.7 μmol/L, total iron binding capacity value of 25.3 μmol/L, low transferrin saturation of 14.6%, serum ferritin of 100 ng/ml, low cobalamin level of 176 pg/mL and normal folate level of 16.3 pg/mL. Serum cortisol level was low at 124.6 nmol/L whilst sodium and potassium levels were also low at 130 mmol/L and 3.0 mmol/L respectively. Liver function test showed low albumin level at 27 g/L with slightly raised AST at 63 U/L. Renal profile and coagulation screening were normal. Antinuclear antibody (ANA) was positive at a titre of 1:5120 which has a speckled pattern. Other autoimmune screening showed positive anti-SSA/Ro 60, anti-SSA/Ro 52, antiSmD1, and anti U1-snRNP. C-ANCA, p-ANCA, double stranded DNA, anti-SSB/La and rheumatoid factor were not detected. Complement 3 and 4 were below the reference range. Both inflammatory markers were elevated with CRP of 219 mg/L and ESR of 154 mm/hr. Serum protein electrophoresis displayed no paraprotein band or immunoparesis. Quantitative serum immunoglobulin test were normal. The serological tests for various infectious agents (HBsAg, anti HCV ab, anti HIV 1 + 2 ab and treponemal tests) were negative. Intrinsic factor antibodies and anti-tissue transglutaminase IgA were below detection limits.
Cultures and sensitivities from the leg ulcers were positive for Proteus mirabilis. He was treated with a two-week course of intravenous amoxicillin/clavulanic acid. Histopathological examination of the leg ulcers demonstrated dense inflammatory cells infiltrate with neutrophil predominance. There was no evidence of necrotising vasculitis, vascular thrombosis, squamous dysplasia or invasive malignancy. No atypical mycobacteria or yeast were detected. An oesophagogastroduodenoscopy and colonoscopy was done which revealed atrophic gastritis, duodenitis and normal colonic mucosa. Campylobacter-like organism (CLO) test was negative. Histologically, the biopsies of small bowel showed well-formed and irregularly spaced glands secondary to expansion of lamina propria by lymphoplasmacytic inflammatory cell infiltrates. Colonic biopsies were insignificant. Periodic acid-Schiff (PAS) staining was negative. The findings were inconsistent with inflammatory bowel disease, Whipple’s disease, coeliac disease or pernicious anaemia.
Based on clinical features, laboratory investigations, endoscopic and histological findings, he was diagnosed as SLE with pyoderma gangrenosum and cobalamin deficiency. He was treated with prednisolone, hydroxychloroquine, methotrexate, parenteral cyanocobalamin and iron supplements. Noticeable clinical improvement was observed after commencement of recommended treatment. Upon clinic review 1 month later, he reported weight gain, increased level of well-being, great improvement of skin lesions along with significant control of symptoms.