A 32-year-old woman presented with a long history of abscesses and pustular lesions. She first noticed “cysts” over the face, armpits, and groin at the age of 13 years. Since then, she developed numerous painful, deep-seated abscesses predominantly in HS distribution, i.e., submammary, inguinal, and perianal areas (Fig. ), that required drainage for symptomatic relief. The axillae were less affected. Abscesses in unusual locations, such as the jawlines and neck, were also noted. Most lesions were in Hurley stage 1 because there was a sparsity of well-formed, typical sinus tracts (most lesions were “blind boils”), and scarring was subtle or absent. Hypertrophic scarring was not noted. Sparse comedones were observed, mostly in the inguinal/pubic area. Recalcitrant folliculitis was invariably noted over the breasts, jawlines, submandibular area/neck, and occasionally the pubic area (Fig. ). Abscesses were associated with symptomatic, reactive pre-auricular, submandibular, and inguinal lymphadenopathy. Occasional low-grade fever was noted. Skin lesions developed on a weekly basis, thus interfering with the patient's quality of life (QOL). The effect on QOL is also evidenced by the history of depression and anxiety in the patient. Her past medical history also included obesity, polycystic ovary syndrome controlled with a combined oral contraceptive, and acne.\nBiopsies of abscesses (Fig. ) and pustular lesions revealed no pathogenic bacteria or fungi with Brown and Brenn and periodic acid–Schiff stains. Numerous cultures from abscesses and pustules were performed over the years. Most cultures did not yield any pathogen, with only a few cultures showing HS microbial flora (e.g., Staphylococcus lugdunensis, Enterococcus faecalis, Peptostreptococcus). Laboratory workup revealed normal serum levels of C4 complement component and C1 inhibitor protein. The MBL level was exceptionally low at 27 and <0.5 ng/mL on two measurements (reference range: ≥100 ng/mL). Serum IgA and IgG were normal while IgM levels were slightly elevated (287 mg/dL; reference range: 48–271 mg/dL). CRP value was elevated at 18 mg/mL (reference range: <8 mg/mL). An extensive workup by Immunology including, among others, serum protein electrophoresis, lymphocyte subset analysis, serum free light chain ratio, and neutrophil oxidative burst testing did not show any other immunodeficiencies. Procalcitonin value was 0.07 ng/mL (values <0.50 ng/mL are noted in abacterial inflammatory disease) [].\nThe patient did not tolerate oral antibiotics, including tetracyclines, amoxicillin clavulanate, and clindamycin +/– rifampin, which, when tried, provided only mild temporary relief. Also, immunosuppressive agents including prednisone and cyclosporine, and hormonal treatment with spironolactone were ineffective. Furthermore, MBL replacement therapy was not an option because the twice-a-week infusion schedule was not possible for the patient []. The above made the patient dependent upon intralesional steroid (triamcinolone) injections and drainage of abscesses and tender pustules. Two recalcitrant abscesses required excision.
A 56-year-old Caucasian man complained of recurrent bilateral lower extremity cellulitis and ulcerations, requiring hospitalization and parenteral antibiotic administration. History included factor V Leiden mutation with recurrent lower extremity deep venous thromboses and recurrent MRSA urinary tract infections. He had undergone numerous abdominal procedures, with recurrent enterocutaneous fistulae and wall abscesses. Abdominal exam revealed wall ulceration with serosanguineous drainage and surrounding erythema. His lower extremities presented ochre dermatitis, bilateral +/4 edema below the knee; there were 2 ulcerations on the anterior portion of his left leg, measuring 1 × 1 cm and 2 × 3 cm (Figure ). A lower extremity ultrasound showed a left-sided nonocclusive chronic thrombus in the femoral and popliteal veins. Total levels of immunoglobulins were normal (except for a small decrease in IgG3), as were C2 and C4 (Table ). Anticardiolipin antibody was negative. The patient had a low level of MBL (<50 ng/ml), genotyping showing a LYPB/LYPB polymorphism for MBL and a wild-type MASP-2 (Table ). The patient's abdominal wall culture grew cephalosporin-sensitive Staphylococcus aureus; cultures from his lower extremity ulcerations were positive for Pseudomonas aeruginosa. The patient responded to antimicrobials, compression stockings and topical Tacrolimus©.