LETTER TO THE EDITOR Year : 2022  |  Volume : 42  |  Issue : 1  |  Page : 77-79

Antiphospholipid antibody syndrome presenting as pyoderma gangrenosum

Mansoor C Abdulla
Department of General Medicine, M.E.S. Medical College, Perinthalmanna, Malappuram, Kerala, India

Date of Submission07-Oct-2020Date of Acceptance09-Jan-2021Date of Web Publication18-Dec-2021

Correspondence Address:
MD Mansoor C Abdulla
Department of General Medicine. M.E.S. Medical College, Perinthalmanna, Malappuram, Kerala 679338
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ejdv.ejdv_12_21

How to cite this article:
Abdulla MC. Antiphospholipid antibody syndrome presenting as pyoderma gangrenosum. Egypt J Dermatol Venerol 2022;42:77-9

The antiphospholipid antibody syndrome (APS) is a prothrombotic condition that can be associated with various cutaneous manifestations. Cutaneous manifestations of APS are extremely diverse and can vary from livedo reticularis to cutaneous necrosis. Cutaneous ulcers presenting as pyoderma gangrenosum (PG) have rarely been reported in APS. We describe a young woman with a PG-like skin lesion with primary APS.

A 35-year-old woman presented with a painful nonhealing ulcer over the left leg for 3 months. It started as a small nodulovesicular lesion over the lower aspect of left leg, which later ulcerated and increased in size. She denied history of fever, trauma, smoking, any drug intake, chronic disease, peripheral vascular disease, and abortions. Examination showed an ulcer over the left medial malleolus of 5×4 cm, necrotic floor, and indurated borders ([Figure 1]a). Peripheral pulses and blood pressure were normal.

Figure 1 Clinical photograph of the ulcer (5×4 cm) over the left medial malleolus (a). Wedge biopsy from the ulcer showing ulcerated skin with granulation tissue and dense neutrophilic crusting suggestive of pyoderma gangrenosum (hematoxylin and eosin staining with ×40 magnification) (b, c, and d).

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Hemoglobin was 10.1 g/dl (microcytic and hypochromic), total leukocyte count was 6000/ml (72% neutrophils, 26% lymphocytes, and 2% monocytes), platelet count was 260,000/μl, and erythrocyte sedimentation rate was 68 mm in 1 h. C-reactive protein was high. Peripheral smear showed microcytic and hypochromic anemia. Iron studies were suggestive of anemia of chronic disease. Urinalysis was normal. Blood chemistries were normal. Prothrombin time was normal, and partial thromboplastin time was prolonged. Blood cultures were negative. Wedge biopsy from the ulcer showed ulcerated skin with granulation tissue and dense neutrophilic crusting, suggestive of PG ([Figure 1]b–d). The history, examination, and routine investigations did not show any evidence to suspect the usual associations of PG-like inflammatory bowel disease, arthropathies, blood dyscrasias, and monoclonal gammopathy. Immunoglobulin G and immunoglobulin M antiphospholipid antibodies and lupus anticoagulant test findings were positive. Antinuclear antibodies, rheumatoid factor, cryoglobulins, and P and C antineutrophil cytoplasmic antibodies were negative. C3 and C4 levels were normal. A diagnosis of primary APS presenting as pyoderma gangrenosum was made based on the histopathology and antibody testing. She was treated with oral warfarin, and the dosage was adjusted to keep the international normalized ratio between 2 and 3. She was also treated with steroids (prednisone 50 mg initially) and was tapered over weeks. The ulcer healed completely after 3 months. Anticardiolipin antibodies repeated after 12 weeks remained elevated.

Several skin manifestations have been described in patients with APS. Livedo reticularis is the most common skin manifestation in patients with APS followed by skin ulcers. The other manifestations are Sneddon’s syndrome, necrotizing vasculitis, livedoid vasculitis, cutaneous gangrene, superficial thrombophlebitis, pseudovasculitic lesions, subungual bleeding and anetoderma [1]. PG is a rare ulcerating lesion of unknown etiology characterized by a necrotic or gangrenous base and a raised violaceous undermined edge. PG does not have any unique or specific histological features; however, it is characterized by epidermal and dermal necrosis and neutrophilic infiltrates, which can help support the diagnosis.

PG has rarely been reported in association with APS. Schmid et al. [2] described a 64-year-old woman with systemic lupus erythematosus who presented with two large nonhealing ulcers. PG associated with secondary APS was diagnosed. Chacek et al. [3] reported a 28-year-old man with PG and APS. Schlesinger et al. [4] reported two cases with PG-like skin lesions and primary APS. A 22-year-old male patient with PG, thrombosis of both popliteal arteries, ischemic stroke, and seropositivity for anticardiolipin antibody was reported by De Godoy et al. [5]. PG as the initial presentation of APS was also reported previously in a 31-year-old female [6].

This case is important for the following reasons: PG as the initial presentation of APS is extremely rare. Treating physicians should have awareness of such atypical presentations of APS and should consider APS also in all patients presenting with PG-like skin lesions.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 

  References 
1.Francès C, Niang S, Laffitte E, Pelletier FL, Costedoat N, Piette JC. Dermatologic manifestations of the antiphospholipid syndrome: two hundred consecutive cases. Arthritis Rheum 2005; 52:1785–1793.  
    2.Schmid MH, Hary C, Marstaller B, Konz B, Wendtner CM. Pyoderma gangrenosum associated with the secondary antiphospholipid syndrome. Eur J Dermatol 1998; 8:45–47.  
    3.Chacek S, MacGregor-Gooch J, Halabe-Cherem J, Nellen-Hummel H, Quinones-Galvan A. Pyoderma gangrenosum and extensive caval thrombosis associated with the antiphospholipid syndrome − a case report. Angiology 1998; 49:157–160.  
    4.Schlesinger IH, Farber GA. Cutaneous ulceration resembling pyoderma gangrenosum in the primary antiphospholipid syndrome: a report of two additional cases and review of the literature. J La State Med Soc 1995; 147:357–361.  
    5.De Godoy JP, Batigalia F, Braile DM. Pyoderma gangrenosum and anticardiolipin antibody. Indian J Dermatol 2006; 51:123–124.  
  [Full text]  6.D’Souza D, Dandakeri S, Bhat MR, Srinath MK. An atypical presentation of antiphospholipid antibody syndrome. Indian Dermatol Online J 2015; 6:263–265.  
    
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