Clinicohistological and immunopathological features of patients with cutaneous lupus erythematosus at tertiary dermatology centre in Malaysia.

BACKGROUND: Cutaneous lupus erythematosus (CLE) is a chronic, autoimmune skin disease with a wide spectrum of clinical presentations in different populations. AIM: To study the clinicohistological and immunological features of CLE in a multiethnic population and to identify the predictive factors of disease severity based on the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). METHODS: This was a cross-sectional study of CLE conducted from March 2019 to February 2020. RESULTS: In total, 111 patients were recruited with a female/male ratio of 4.9 : 1. Acute CLE contributed 47.7%, followed by chronic CLE at 46.9% and subacute CLE at 5.4%. A large majority (84%) of patients had systemic lupus erythematosus. Of patients with chronic CLE, about 67.3% developed systemic involvement. Antinuclear antibody (ANA) was detected in 90.0%. Skin biopsy was taken from 42 patients and showed perivascular lymphocytic infiltration (95.2%), epidermal atrophy (47.6%) and hydropic degeneration of the basal layer (47.6%). Immunoglobulin deposition at the dermoepidermal junction was seen in > 40% of patients, predominantly in a granular pattern. Mean CLASI Total was 6.44 ± 7.70, while CLASI Activity (CLASI-A) was 2.75 ± 4.10 and CLASI Damage (CLASI-D) was 3.71 ± 4.76. Involved body surface area (BSA) was found to be an independent predictive factor for CLASI-A (OR = 1.34, P < 0.02). For CLASI-D, positive predictive factors were involved BSA (OR = 4.14, P < 0.001), discoid lupus erythematosus subtype (OR = 13.10, P = 0.001), cutaneous vascular disease (OR = 26.59; P = 0.014), scalp involvement (OR = 8.7, P < 0.01) and hypocomplementaemia (OR = 5.71, P < 0.5). Mean Dermatology Life Quality Index was 5.91 ± 5.34 and correlated significantly with disease severity. CONCLUSIONS: We observed a high percentage of patients with CLE with systemic manifestations and positive ANA result. More aggressive treatment of patients with positive predictive factors for severe disease combined with significant clinical activity may be warranted.

The mystery of 'saturation gap': a case of dapsone-induced methaemoglobinemia in a pregnant mother with leprosy.

Limited data regarding methemoglobinemia in pregnancy, particularly secondary to dapsone is available up to date. We report a case of dapsone-induced methemoglobinemia in a pregnant mother with multibacillary leprosy who presented with fever, productive cough and cyanosis of 2 days duration 2 weeks after multidrug therapy was commenced. On examination, she had central cyanosis with low oxygen saturation (SpO2 = 84-88%). Arterial blood gas analysis showed PO2 of 111 mmHg and SO2 of 98 mmHg. Patient was administered 100% oxygen inhalation, but there was no improvement in cyanosis. Vitamin C (1000 mg/day) was prescribed. Dapsone was replaced by ofloxacin 200 mg twice daily. There was a gradual increase in SpO2 level. She delivered a healthy baby. In conclusion, clinicians should be aware of the side effects of dapsone and know how to promptly manage any undesirable events. Ofloxacin is a safe and feasible alternative in replacement of dapsone in pregnancy.

Disseminated AIDS-related Kaposi's sarcoma.

We present a case of disseminated Kaposi's sarcoma with both cutaneous and extracutaneous involvement in an HIV-infected patient with a relatively high CD4 count of 369 cell/mm3. He developed chronic diarrhea, constitutional symptoms, worsening bilateral pleural effusion with respiratory distress, and progression of skin lesions distributed over his chest and extremities. The temporal relationship between rapid clinical progression and initiation of HAART suggested the possibility of Kaposi's sarcoma-associated immune reconstitution inflammatory syndrome, which eventually resulted in the death of this patient.